Rheumatology Rheumatology Rheumatology is Rheumatology is concerned with diseases of joints & concerned with diseases of joints & C.T., they are diseases of C.T., they are diseases of musculoskeletal system.musculoskeletal system.

Rheumatological conditions are Rheumatological conditions are characterized by , chronic pain, characterized by , chronic pain, stiffness &progressive impairment of stiffness &progressive impairment of joints & soft tisssue.joints & soft tisssue.

Arthritis and other rheumatic Arthritis and other rheumatic conditions are the leading cause of conditions are the leading cause of disability in US, is a major public disability in US, is a major public health problem and is the most health problem and is the most common chronic disease in USA.common chronic disease in USA.

Economically they are costy , the cost Economically they are costy , the cost comes from the direct spent of comes from the direct spent of treatment ( direct cost) & indirect cost treatment ( direct cost) & indirect cost from the lost of productivity.( these from the lost of productivity.( these costs are millions of dollars).costs are millions of dollars).

Rheumatolgical conditions are one of Rheumatolgical conditions are one of the major causes of disabilities in the major causes of disabilities in

community. community.

They may lead to various types of They may lead to various types of disabilities which could be ;disabilities which could be ;

1-Physical diability1-Physical diability ( walking, ( walking, bending,lifting & grasping).bending,lifting & grasping).

2-Social disability2-Social disability ( refers to higher ( refers to higher tasks , i.e.eating, dressing, tasks , i.e.eating, dressing, shopping& interacting with other shopping& interacting with other people.)people.)

Arthritis first causes of physical Arthritis first causes of physical dysfunction which lead to social dysfunction which lead to social

dysfunction.dysfunction.

Social dysfunction includes :Social dysfunction includes : 1-1-Basic personal care tasks ( ADL) Basic personal care tasks ( ADL)

such as eating , grooming& such as eating , grooming& toileting.toileting.

2-2-Instrumental activities of daily Instrumental activities of daily living(IADL) (house task) like living(IADL) (house task) like shopping and paying bills.shopping and paying bills.

Rheumatological historyRheumatological history

1.1. Is the problem is regional or Is the problem is regional or generalised, symmetric or generalised, symmetric or

asymmetric, peripephral or central.asymmetric, peripephral or central.

2.2. Is it an acute ,subacute or chronic Is it an acute ,subacute or chronic problem?Is it progressive.problem?Is it progressive.

3.3. Do symptoms suggest inflammation Do symptoms suggest inflammation or damage to musccloskeletal or damage to musccloskeletal

structures.structures.

4.Is there evidence of systemic 4.Is there evidence of systemic process? Are there associated process? Are there associated

extraarticular features. 5. Ask for extraarticular features. 5. Ask for underlying medical disorder which underlying medical disorder which may predispose,Family history and may predispose,Family history and

functional loss and disability.functional loss and disability.

JOINTS.JOINTS. Joint is articulation between two or Joint is articulation between two or more bones.more bones.

The activities of human body depends The activities of human body depends on effective interaction between on effective interaction between normal joints & neuromuscular units normal joints & neuromuscular units that drive them.that drive them.

Types of joints are:Types of joints are:

1. 1. Synarthrosis ( skull joints)Synarthrosis ( skull joints)

2. 2. Amphiarthrosis( Cartilaginous Amphiarthrosis( Cartilaginous joints) have joints) have

modest motion ( Intervertebral modest motion ( Intervertebral disc joints).disc joints).

3. 3. Diarthrosis are mobile joints have Diarthrosis are mobile joints have synovialsynovial

membrane (synovial joints ).membrane (synovial joints ).

Anatomy of synovial joints.Anatomy of synovial joints.

Diarthroidal joint consist of :Diarthroidal joint consist of :

1. 1. Opposing bony surfaces.Opposing bony surfaces.

2. 2. Hyaline cartilage covering the Hyaline cartilage covering the articulating surfaces ofarticulating surfaces of

bones.It is firmly attached to the bones.It is firmly attached to the underlying bone.underlying bone.

It is aneural & avascular( no blood or It is aneural & avascular( no blood or lymph). It gets the nourishment lymph). It gets the nourishment

from synovial fluid , s. membrane from synovial fluid , s. membrane &underlying bone.&underlying bone.

The cartilage is shock absorber.The cartilage is shock absorber.

3. 3. Capsule which is surrounding the Capsule which is surrounding the articular tissue.articular tissue.

4. 4. Synovial membrane which lines Synovial membrane which lines the capsule. the capsule.

Has A cells are macrophage like Has A cells are macrophage like cells havecells have

phagocytic function & B cells are phagocytic function & B cells are fibroblast fibroblast

like cells ( produce hyaluronate like cells ( produce hyaluronate part of synovialpart of synovial

fluid.fluid.

5.5.Joint cavity.Joint cavity.

6.6. Synovial fluid. Is viscous , pale Synovial fluid. Is viscous , pale yellow & clear yellow & clear

fluid , present in small amount fluid , present in small amount ranging fromranging from

( 0.1 _ 4). Hyaluronic acid making ( 0.1 _ 4). Hyaluronic acid making the fluid the fluid

viscous.The fluid functios are viscous.The fluid functios are lubrication,nutrient distribution and lubrication,nutrient distribution and shock absorption.shock absorption.

Rheumtology ; Rheumtology ; is concerned with the is concerned with the diseases of diseases of

joints & C.Ts which could be damaged joints & C.Ts which could be damaged by avariety by avariety

of pathological processes of pathological processes ( infection ,inflammation ( infection ,inflammation

, degeneration, metabolic , degeneration, metabolic disturbances &systemic disturbances &systemic

diseases like leukaemia or diseases like leukaemia or Haemochromatosis).Haemochromatosis).

Rheumatological Rheumatological Terminology.Terminology.

Arthritis:Arthritis: is inflammation of the is inflammation of the joint.joint.

Polyarthritis :Polyarthritis : is inflammation of is inflammation of five or more five or more

joints simultaneously.joints simultaneously.

Oligoarthritis ( Pauciarticular) :Oligoarthritis ( Pauciarticular) : 2_ 4 joint are 2_ 4 joint are

involved.involved.

Monoarthritis :Monoarthritis : is inflammation of is inflammation of one joint.one joint.

Arthralgia :Arthralgia : is a joint pain without is a joint pain without swelling .swelling .

Inflammation of joint is suggested by :Inflammation of joint is suggested by :

Pain , Swelling, Warmth & reddening Pain , Swelling, Warmth & reddening of overlying of overlying

skin.skin.

Joint stiffness which occur after a Joint stiffness which occur after a period of period of

immobility & lasting for over a immobility & lasting for over a period ( hour) is period ( hour) is

more reliable indication of more reliable indication of inflammation.inflammation.

Reduction of joint motion & loss of Reduction of joint motion & loss of function may function may

occur in noninflammatory condition occur in noninflammatory condition also.also.

Features of Chronic autoimmune Features of Chronic autoimmune inflammatory polyarthritis.inflammatory polyarthritis.

1. Significant morning stiffness.1. Significant morning stiffness.

2. Spontaneous day to day flactuation.2. Spontaneous day to day flactuation.

3. Improvement of symptoms with gentle 3. Improvement of symptoms with gentle use.use.

4. Symptoms worse at night and in the 4. Symptoms worse at night and in the morning.morning.

5. Constitutional symptoms:fatgue,night 5. Constitutional symptoms:fatgue,night sweats,fever,loss of weight and appetite. sweats,fever,loss of weight and appetite.

ClassificationClassification Inflammatory arthritis.Inflammatory arthritis. 1.1. R.A. R.A.

2.2. Seronegative arthropathies. Seronegative arthropathies.

3.3.C.T diseases : SLE.C.T diseases : SLE.

Scleoderma.Scleoderma.

Dermatomyositis.Dermatomyositis.

4.4.Crystal induced arthropathies.Crystal induced arthropathies.

5.5.Degenerative joint disease : primary Degenerative joint disease : primary or secondary.or secondary.

6.6. Arthritis associated with infection : Arthritis associated with infection :

( septic &nonseptic arthritis).( septic &nonseptic arthritis).

7.7. Arthritis associated with systemic Arthritis associated with systemic diseasesdiseases

(bacterial endocarditis, acromegaly, (bacterial endocarditis, acromegaly, thyroid thyroid

diseases,respiratory diseases & diseases,respiratory diseases & miscellaneous- miscellaneous-

sarcoidosis,amyloidosis etc.sarcoidosis,amyloidosis etc.

8.8. Nonarticular rheumatism& Nonarticular rheumatism& localized painlocalized pain

(tenosynovitis, (tenosynovitis, bursitis,fibrositis&enthesopathies)bursitis,fibrositis&enthesopathies)

CLINICAL FEATURES CLINICAL FEATURES Features help in the diagnosis of Features help in the diagnosis of arthritis in general are :arthritis in general are :

Age:Age: as in OA. as in OA.

Gender:Gender: RA & SLE are more common RA & SLE are more common in females.in females.

Race:Race: As arthritis associated with As arthritis associated with sickle cell sickle cell

anemia.anemia.

Occupation:Occupation: As in soft tissue As in soft tissue rheumatism.rheumatism.

Joint pain:Joint pain: the following points are the following points are of some value of some value

to in the diagnosis of arthritic to in the diagnosis of arthritic problems regarding problems regarding

pain :pain :

Duration of painDuration of pain ( in Gout is acute ( in Gout is acute while in RA is while in RA is

usually chronic).usually chronic).

Onset:Onset: ( abrupt in gout). ( abrupt in gout).

Precipitating factors:Precipitating factors: as trauma, use as trauma, use of diuretics of diuretics

etc.etc.

CharacterisiticsCharacterisitics Site of pain : This usually indicates Site of pain : This usually indicates

the site of the pathology.the site of the pathology. Radiation.Radiation. Severety: i.e. Gout.Severety: i.e. Gout. Aggravating & relieving factors.Aggravating & relieving factors. Diurnal variation.Diurnal variation.

( i.e.Inflammation).( i.e.Inflammation). Episodic arthritisEpisodic arthritis

Morning Stiffness.Morning Stiffness. Duration is important.Duration is important.

Pattern of joint involvement.Pattern of joint involvement. Reccurent attack, small joints Reccurent attack, small joints

involvement.involvement. Disability.Disability. ( depends on joints ( depends on joints

affected).affected).

Objective singes of arthritis.Objective singes of arthritis. Swelling , Tenderness, Limitation of Swelling , Tenderness, Limitation of joint joint

movement , deformity, Crepitas & movement , deformity, Crepitas & Heat & Heat &

redness. redness.

Joint pathology is indicated by pain & Joint pathology is indicated by pain & one or of one or of

other signes.other signes.

Five cardinal singes of inflammation Five cardinal singes of inflammation are :are : swelling, swelling,

warmth, erythema, tenderness & loss warmth, erythema, tenderness & loss of function.of function.

Joint examination: Joint examination:

Includes three stages.( Look at it , feel Includes three stages.( Look at it , feel it & move it) it & move it)

Lab. Tests:Lab. Tests:

1.ESR1.ESR

2.CRP(c-reactive protein)2.CRP(c-reactive protein)

3.Hb3.Hb

4.WBCs count4.WBCs count

Rheumatoid factors.Rheumatoid factors. Are autoantibodies Are autoantibodies directed against antigenic determinants on directed against antigenic determinants on the FC the FC fragment of immunoglbuline G. RF may be fragment of immunoglbuline G. RF may be of any of any isotype IgG, IgM, IgA or IgE.IgM is the isotype IgG, IgM, IgA or IgE.IgM is the one one routinely measured by clinical routinely measured by clinical laboratories.laboratories. RF is detected by agglutination of either RF is detected by agglutination of either latex latex particles or sheep red cells.particles or sheep red cells. Also by ELISA Also by ELISA & Nephelometry. & Nephelometry. Anti-CCP( anticyclic citriullinated peptide Anti-CCP( anticyclic citriullinated peptide antibodies) are autoantibodies directed against antibodies) are autoantibodies directed against the amino acids , it’s believed that have a role in the amino acids , it’s believed that have a role in the pathogenisis of RA.the pathogenisis of RA.Antinuclear antibodies( ANA).Antinuclear antibodies( ANA). Are Are antibodies antibodies which react to constituents of nucleous which react to constituents of nucleous ( SLE , ( SLE , Scleroderma, Juvenile RA. Etc.).Scleroderma, Juvenile RA. Etc.). Serum uric acid ; ( GOUT).Serum uric acid ; ( GOUT).

Imagings :Imagings :Plain x-ray is important in OA, RA & Plain x-ray is important in OA, RA & other other

rheumatological conditions. i.e rheumatological conditions. i.e ( osteophyte , ( osteophyte ,

erosion….).erosion….).

C.C.T scan & MRI are now more valuable T scan & MRI are now more valuable than x-ray than x-ray

in diagnosis. in diagnosis.

Synovial fluid analysis:Synovial fluid analysis: Is helpful in distinguishing between Is helpful in distinguishing between

inflammatory & noninflammatory inflammatory & noninflammatory arthritis. It is diagnostic in crystal arthritis. It is diagnostic in crystal induced arthropathies ( gout) & septic induced arthropathies ( gout) & septic arthritis.( look at Color, arthritis.( look at Color, Viscosity,turbidity, WBC,Crystal Viscosity,turbidity, WBC,Crystal etc……).etc……).

No absolut contraindication for joint No absolut contraindication for joint aspiration. aspiration.

Synovial Synovial biopsy:biopsy:done through needle biopsy or by done through needle biopsy or by

arthroscopy (which by itself is a arthroscopy (which by itself is a valuable investigation).valuable investigation).

Ultrasound of joint & soft tissues.Ultrasound of joint & soft tissues. Radioisotope bone scan: Radioisotope bone scan: ( tumors, ( tumors, infection, trauma, lead to increased infection, trauma, lead to increased uptake.).uptake.).

Histocompatability antigens: Histocompatability antigens: i.ei.e HLAB27 in AS 95% , Reiter’s disease HLAB27 in AS 95% , Reiter’s disease 60% positive.60% positive.

Some other investigations are helpfulSome other investigations are helpful::

serum alkaline phophatase serum alkaline phophatase

ASO ASO

low serum complements ( in SLE).low serum complements ( in SLE).

Osteoarthritis.Osteoarthritis. OA.Is a disease of diarthroidal joints , OA.Is a disease of diarthroidal joints , is a commonest musculoskeletal is a commonest musculoskeletal disease.disease.

Charecterised by Charecterised by

1.1. pain & limitation of function, pain & limitation of function,

2.2. osteophyte & joint space osteophyte & joint space narrowing.narrowing.

3. 3. Alteration in cartilage integrity.Alteration in cartilage integrity.

4.4. NO systemic manifestations. NO systemic manifestations.

The disease occurs through the The disease occurs through the world & through the history, it is more world & through the history, it is more common in females.common in females.

Pathogenesis & pathologyPathogenesis & pathology..

It is a disease of articular cartilage.It is a disease of articular cartilage.

Chondrocytes are maintaining Chondrocytes are maintaining homeostasis of cartilage , it synthesize homeostasis of cartilage , it synthesize collagens, proteoglycans & collagens, proteoglycans & proteinases. proteinases.

OA results from failure of OA results from failure of chondrocytes to synthesize a good chondrocytes to synthesize a good quality of matrix in terms of elasticity quality of matrix in terms of elasticity & resistance & to maintain the balance & resistance & to maintain the balance between synthesis & degredation.between synthesis & degredation.

The degeneration process might be The degeneration process might be initiated by some stimuli initiated by some stimuli ( Mechanical insult or Biochemical ( Mechanical insult or Biochemical abnormalities of cartilage).abnormalities of cartilage).

The chodrocyte is believed to start The chodrocyte is believed to start releasing releasing

enzymes which degrade collagen & enzymes which degrade collagen & proteoglycan, proteoglycan,

breaks in the cartilage allow the breaks in the cartilage allow the uptake of water, uptake of water,

cartilage swells & splits leads to cartilage swells & splits leads to breaking of the breaking of the

cartilage( fibrillation of the surface) , cartilage( fibrillation of the surface) , clefts down clefts down

toward the bone surface, gradual toward the bone surface, gradual lyses of cartilage lyses of cartilage

by synovial enzymes & inflammation by synovial enzymes & inflammation of the of the

synovial membrane ( might be synovial membrane ( might be effusion).effusion).

The underneath bone become smooth The underneath bone become smooth & hard called ( eburnation) & & hard called ( eburnation) & osteophytes form from boney edges osteophytes form from boney edges & subchondral bone cyst might form.& subchondral bone cyst might form.

Joint insability may develop as a Joint insability may develop as a result of capsulare laxity , collapse result of capsulare laxity , collapse of subchondral bone cyst & muscular of subchondral bone cyst & muscular atrophy.atrophy.

CLASSIFICATION OF OA. SCLASSIFICATION OF OA. S A. Primary OA.A. Primary OA.

1.1. Localised ( Heberden s & Localised ( Heberden s & Bouchard s nodes)Bouchard s nodes)

2.2.Primary generlised OA.Primary generlised OA.( involvement of 3 or mor joints or ( involvement of 3 or mor joints or

joint groups).joint groups).

B. Secondary OA.B. Secondary OA. Any malalignment , damage or Any malalignment , damage or alteration to the constituents of the alteration to the constituents of the joint ( the bone, cartilage, capsule, joint ( the bone, cartilage, capsule, ligamemts or synovium may result in ligamemts or synovium may result in accelerated wear& the development of accelerated wear& the development of OA.OA.

1. 1. Trauma.( Intraarticular fracture, Trauma.( Intraarticular fracture, maalignment of maalignment of

fratcture, unequal leg length, fratcture, unequal leg length, occupation ).occupation ).

2. 2. Genetic, congenital & Genetic, congenital & developmental developmental

abnormalities: Hypermobility, abnormalities: Hypermobility, DDH, Perth’s DDH, Perth’s

disease etc.disease etc.

3. 3. Post inflammatory ( RA, Septic Post inflammatory ( RA, Septic arthritis etc).arthritis etc).

4. 4. Haemorrhage to the Haemorrhage to the joint( Haemophilia).joint( Haemophilia).

5. 5. Metabolic & endocrine diseases.Metabolic & endocrine diseases.

6. 6. Bone disorder( Paget s disease)Bone disorder( Paget s disease)

7. 7. Neuropathic joints.Neuropathic joints.

Risk factors for OA are:Risk factors for OA are: Obesity, Obesity, Heridity( in DIP joints), Heridity( in DIP joints), Age, Age, Previous joint disease ( trauma), Previous joint disease ( trauma), Abnormal joint mechanics( varus or Abnormal joint mechanics( varus or valgus) , valgus) , Smoking( in disc disease).Smoking( in disc disease).

Aetiology Aetiology OA is probably is multifactorial .OA is probably is multifactorial .

1.1. Age & gender. OA is more in aged & Age & gender. OA is more in aged & females.females.

2.2. Trauma & obesity. Trauma & obesity.

3.3. Hormonal & metabolic causes. Hormonal & metabolic causes.

4.4. Genetic factor. Genetic factor.

5.5. Diatery causes. Diatery causes.

Clinical Features.Clinical Features. Commonly affected joints are spine , knees, Commonly affected joints are spine , knees, DIPs, DIPs,

PIPs, thumb joint, & first MTP joints PIPs, thumb joint, & first MTP joints

SymptpmsSymptpms

Pain.Pain.

Is deep dull aching pain, worse by use ( in Is deep dull aching pain, worse by use ( in evening) may evening) may

be present at rest. Trauma precipitate it , be present at rest. Trauma precipitate it , related to related to

weather ( more in cold & damp weather), it weather ( more in cold & damp weather), it may radiate to may radiate to

the surrounding structures.It may be the surrounding structures.It may be persistent interfering persistent interfering

with normal function & sleep.with normal function & sleep.

Pain arises from structures possessing Pain arises from structures possessing nerve endings & may result from nerve endings & may result from

microfructures in subchondral bone.microfructures in subchondral bone.

Stiffness.Stiffness. It is less sever than in inflammatory It is less sever than in inflammatory arthritis & arthritis &

lasts for a shorter period ( few lasts for a shorter period ( few minutes).minutes).

Disability.Disability. It depends on a number & severity of It depends on a number & severity of joint joint

affection.affection.

Signs of OASigns of OA 1.1. Swelling. May be due to soft tissue, Swelling. May be due to soft tissue,

fluid or boney overgrowth.fluid or boney overgrowth.

Heberdens & Bouchards nodes. Are Heberdens & Bouchards nodes. Are boney articular nodes. ( H.N are boney articular nodes. ( H.N are seen on DIPs & B.N on PIPs).seen on DIPs & B.N on PIPs).

2.2. Creptus. Creptus.

3.3. Signs of inflammation. Signs of inflammation.

4.4. Limitation of joint movement. Limitation of joint movement.

5.5. Joint deformity.( valgus, varus , Joint deformity.( valgus, varus , flexion deformities or joint flexion deformities or joint instability).instability).

6.6. Loss of function. Loss of function.

Investigations. Investigations. There are no diagnostic makers in OA.There are no diagnostic makers in OA.

X- Ray changes.X- Ray changes.

1.1. Narrowing of the joint space Narrowing of the joint space

2.2. Oteophyte. Oteophyte.

3.3. Sclerosis of subchondral bone Sclerosis of subchondral bone ( eburnation).( eburnation).

4.4. Subchondral bone cyst. Subchondral bone cyst.

5.5.Deformity resulting from sublaxation.Deformity resulting from sublaxation.

6.6.Presence of loose bodies.Presence of loose bodies.

7.7.Irregularity of bone surfaces. Irregularity of bone surfaces.

MRI is superior to X-Ray ( showing cartilage MRI is superior to X-Ray ( showing cartilage changeschanges).).

MANAGEMENTMANAGEMENT Aims are.Aims are.

1.1. Relieving pain. Relieving pain.

2.2. Maintaining & improving joint Maintaining & improving joint function.function.

3.3. Preventing or minimizing disability Preventing or minimizing disability improving functioning.improving functioning.

General measures.General measures.1. 1. Education of the patient.Education of the patient.

2. 2. Maintain & improve joint function.Maintain & improve joint function.

3. 3. Short lower limb should be Short lower limb should be correted.correted.

4. 4. Reduce weight .Reduce weight .

5. 5. Avoid trauma.Avoid trauma.

6.6. Use aid . Use aid .

7. 7. Change jobe.Change jobe.

Phamcological therapy. Phamcological therapy. 1.1. Simple analgesia( paracetamol). Simple analgesia( paracetamol). 2.2. NSAIDs. NSAIDs. 3.3. Intraarticular injections( steroid , Intraarticular injections( steroid ,

Hyaluronic acid).Hyaluronic acid). 4.4. New agents ( might reduce cartilage New agents ( might reduce cartilage

degredation & stimulate cartilage degredation & stimulate cartilage synthesis)( Glucosamininglycan & synthesis)( Glucosamininglycan & chondroitoin ).chondroitoin ).

5. Fibroblast growth factor 18(FGF-18) 5. Fibroblast growth factor 18(FGF-18) stimulate cartilage formation.(research).stimulate cartilage formation.(research).

Physiotherapy. Physiotherapy. To maintain joint mobility &function & To maintain joint mobility &function &

improve muscular condition. improve muscular condition.

Surgery.Surgery. ( i.e joint replacement).( i.e joint replacement).

Rheumatoid Arthritis.Rheumatoid Arthritis. Is a chronic systemic inflammatory Is a chronic systemic inflammatory

disease of unknown aetiology disease of unknown aetiology characterized by symmetrical characterized by symmetrical arthritis of hands , feet & other arthritis of hands , feet & other joints ( PIP & MCPs) with bone joints ( PIP & MCPs) with bone erosion.erosion.

RA is a commonest inflammatory RA is a commonest inflammatory arthritis.arthritis.

RA is characterized by:RA is characterized by:

1.1. A symmetrical inflammatory A symmetrical inflammatory polyarthritis.polyarthritis.

2.2. It has extraarticular features. It has extraarticular features.

3.3. Progressive joint damage causing Progressive joint damage causing sever disability in young people.sever disability in young people.

Epidemiology.Epidemiology. RA is a world wide disease affects all RA is a world wide disease affects all racial & racial &

ethinic groups.It affects 1—3% of ethinic groups.It affects 1—3% of population , population ,

female/ male is 3/1. female/ male is 3/1.

Age range is 10 – 70y. ( starts 30& Age range is 10 – 70y. ( starts 30& 40ys.). 40ys.).

5—10% having family history & 70% 5—10% having family history & 70% have have

HLADR4, and HLADR1 found in HLADR4, and HLADR1 found in majority of HLADR4 negative patients.majority of HLADR4 negative patients.

Aetiology & Pathogenesis.Aetiology & Pathogenesis. The cause of RA is unknown. Several The cause of RA is unknown. Several factors may factors may

possibly operate to produce the possibly operate to produce the disease ( i.e. disease ( i.e.

Genetic, toxic substances and Genetic, toxic substances and environmental factors..). RA is said to environmental factors..). RA is said to be an be an

autoimmune disease.( there are autoimmune disease.( there are many many

immunological disturbances in RA. immunological disturbances in RA. As As

autoantibodies).( Cytochins , autoantibodies).( Cytochins , growth factors , growth factors ,

tumor necrosis factors& tumor necrosis factors& metalloproteases have metalloproteases have

role in the production of the role in the production of the disease.).disease.).

Pathology.Pathology. RA is a disease of synovial membrane.RA is a disease of synovial membrane. There is Inflammation & proliferation.There is Inflammation & proliferation.( synovitis occur ( synovitis occur with chronic inflammatory cells with chronic inflammatory cells infiltration, lymphocyte, infiltration, lymphocyte, plasma cells & macrophages followed by plasma cells & macrophages followed by gaint gaint cells infiltration, fibrinoid degeneration& cells infiltration, fibrinoid degeneration& hyperplasia of hyperplasia of lining cells followed by granulation tissue lining cells followed by granulation tissue ( Pannus) ( Pannus) formation which is a tumor like mass. This formation which is a tumor like mass. This leads to leads to destruction of the cartilage & bone & destruction of the cartilage & bone & might be effusion.might be effusion. Synovitis occurs in tendon sheath & Synovitis occurs in tendon sheath & bursae. Nodules bursae. Nodules might occur over pressure areas ( more might occur over pressure areas ( more on extensor on extensor surfaces).surfaces). Vasculitis( panarteritis) may occur.Vasculitis( panarteritis) may occur. Chest could be involved. Chest could be involved. Eyes might be affected.Eyes might be affected.

Clinical Features.Clinical Features. The onset is variable ( 70% insidious The onset is variable ( 70% insidious 15- 20% is 15- 20% is

subacute & 5—10% acute while few subacute & 5—10% acute while few having having

episodic symptoms that progress to episodic symptoms that progress to persistant persistant

diseases ).diseases ).

Commonly affected joints are MCPs, Commonly affected joints are MCPs, PIPs, wrists, PIPs, wrists,

MTPs & larger joints.MTPs & larger joints.

RA might starts with few joints then RA might starts with few joints then progress& progress&

become symmetric.become symmetric.

Thoracolumbare , sacroiliac & DIP Thoracolumbare , sacroiliac & DIP joints are very rarely involved.joints are very rarely involved.

RA is generally symmetrical, RA is generally symmetrical, destructive, disabling & deforming destructive, disabling & deforming polyarthritis , affecting small & large polyarthritis , affecting small & large joints.joints.

It has systemic manifestations, It has systemic manifestations, extraaricular features& circulating extraaricular features& circulating antiglobulin antibodies antiglobulin antibodies

(RA factor).(RA factor).

Symptoms. Symptoms.

1.1. Joint painJoint pain.( More in the morning & .( More in the morning & might might

disturb the sleep).disturb the sleep).

2.2. Morning stiffness.Morning stiffness. Is a prominent Is a prominent feature , feature ,

present after a peroid of rest often lasts present after a peroid of rest often lasts for several hours.for several hours.

3.3. General symptpms.General symptpms. Malaise, fever,loss of Malaise, fever,loss of weight & weight &

strength & diffuse muscle wasting .strength & diffuse muscle wasting .

4.4. Disability.Disability. This depends on joints This depends on joints affected & affected &

destructions occurred.destructions occurred.

5.5. Non articular symptoms:eNon articular symptoms:e .g CTS. .g CTS.

Signs.Signs. Swelling, Swelling, Warmth,Warmth, Limitation of movements, Limitation of movements, Deformities .Deformities . Nodules. Nodules.

Pettern of joint involvement.Pettern of joint involvement.

RA is usually starts from small joints RA is usually starts from small joints of hands & feet but in most patients of hands & feet but in most patients eventually many joints are involved eventually many joints are involved ( wrists, knees, ankls , shoulders ( wrists, knees, ankls , shoulders etc…).etc…).

Hands & feet.Hands & feet.

MCPs &PIPs are early involved MCPs &PIPs are early involved ( spindle shape ( spindle shape

swelling of PIPs). , Swelling of swelling of PIPs). , Swelling of wrists. wrists.

Progression of the disease will lead Progression of the disease will lead wasting & atrophy of small muscles , wasting & atrophy of small muscles , weakening of capsule & other weakening of capsule & other supportive tissue & joint destruction supportive tissue & joint destruction result in limitation of joint motion , result in limitation of joint motion , instability, sublaxation & deformities.instability, sublaxation & deformities.

Hand deformities.Hand deformities.

Radial deviation at wrist.Radial deviation at wrist.

Ulnar deviation at MCP joints.Ulnar deviation at MCP joints.

Swan neck deformity ( hperextension Swan neck deformity ( hperextension at PIPs).at PIPs).

Boutonnier deformity( flexion at Boutonnier deformity( flexion at PIPS)PIPS)

Feet deformities are more or less are Feet deformities are more or less are like those occur in hands. like those occur in hands.

The kneesThe knees.. 1.1.Synovial effusion Synovial effusion

2.2.Deformities( valgus., varus &/ or Deformities( valgus., varus &/ or flexion ). flexion ). 3.3.Bakers cyst in Bakers cyst in popliteal fossa which may repture.popliteal fossa which may repture.( sudden onset of pain & swelling in ( sudden onset of pain & swelling in calf and ankle) diagnosed by US. & calf and ankle) diagnosed by US. & arthrogram.arthrogram.

PresentationsPresentations.. RA is commonly affecting small joints of RA is commonly affecting small joints of hands & feet.hands & feet.

It could be presented as acute polyathritis It could be presented as acute polyathritis ( 15% ).( 15% ).

Involvement of large joints.Involvement of large joints.

Monoarticular is not uncommon.Monoarticular is not uncommon.

Palindromic( episodic).Palindromic( episodic).

Soft tissue involvement.Soft tissue involvement.

Prodromal systemic symptoms.Prodromal systemic symptoms.

With the progression of the disease With the progression of the disease get( destructive get( destructive

changes, deformities, symptoms of changes, deformities, symptoms of mechanical effects as mechanical effects as

pressure on the nerves( CTS) ).pressure on the nerves( CTS) ).

Criteria for the diagnosis of Criteria for the diagnosis of RA.RA.

1.1. Morning stiffness for> one hour. Morning stiffness for> one hour.

2.2. Swelling of 3 or more Swelling of 3 or more joints( arthritis).joints( arthritis).

3.3. Swelling of hand joints ( PIPs , Swelling of hand joints ( PIPs , MCPs & wrist ).MCPs & wrist ).

4.4. Symmetrical polyarthritis. Symmetrical polyarthritis.

5.5. Subcutaneous nodules. Subcutaneous nodules.

6.6. Serum rheumatoid factor. Serum rheumatoid factor.

7.7. Radiological changes ( erosion ). Radiological changes ( erosion ).

Duration of (1,2,3,4) for 6 weeks or Duration of (1,2,3,4) for 6 weeks or more.more.

Extra-articular manifestations. Extra-articular manifestations.

A.A. In soft tissue surrounding joints.In soft tissue surrounding joints.

1. 1. Rheumatoid nodules.( found in 20—Rheumatoid nodules.( found in 20—30% ) , mostly in seropositsve . Are 30% ) , mostly in seropositsve . Are subcutaneous, not attached to skin subcutaneous, not attached to skin or underlying tissue, mobile , not or underlying tissue, mobile , not tender & located on extensor tender & located on extensor surface of elbow , forearm, wrist , surface of elbow , forearm, wrist , occiput& achills tendon. Might be occiput& achills tendon. Might be found in chest , heart or eye.found in chest , heart or eye.

2. 2. Bursitis. Olrcranon or other bursae.Bursitis. Olrcranon or other bursae.

3. 3. Tenosynovitis. ( flexor tendons in Tenosynovitis. ( flexor tendons in palm—Trigger finger).palm—Trigger finger).

4. 4. Muscle wasting. As in small Muscle wasting. As in small muscles of hands.muscles of hands.

5. 5. Cyst & repture joints.( baker s Cyst & repture joints.( baker s cyst).cyst).

B.B. The eyes.The eyes. 1.1. Insuffecient secretion ( dry eye – Insuffecient secretion ( dry eye –

Keratoconjunctivitis sicca – Keratoconjunctivitis sicca – Sjogrens syndrome) Sjogrens syndrome)

2.2. Scleritis, Scleritis,

3.3. Episcleritis. Episcleritis.

C.C. The nervous system.The nervous system. 1. 1. Peripheral neuropathy.Peripheral neuropathy.

A.A. Polyneuropathy( rare causing glove & Polyneuropathy( rare causing glove & stocking)stocking)

B.B. Entrapment neuropathy ( CTS ). Entrapment neuropathy ( CTS ).

2. 2. Atlanto-axial sublaxation.Atlanto-axial sublaxation.

D.D. The spleen, lymph nodes & The spleen, lymph nodes & blood.blood. 1.1. Palpable lymph nodes are Palpable lymph nodes are common.common.

2.2. Spleen may be enlarged. Spleen may be enlarged.

3.3. Felty syndrome{ arthritis(RA) , Felty syndrome{ arthritis(RA) , splenomegaly & splenomegaly &

neutropenia.}neutropenia.}

4.4. Aanaemia is almost universal Aanaemia is almost universal ( nomochromic ( nomochromic

nomocytic type).nomocytic type).

NSAIDs might cause it.NSAIDs might cause it.

E.E. Blood vessels & heart.Blood vessels & heart. Vasculitis might occur in RA. ( tinny Vasculitis might occur in RA. ( tinny spots spots

around finger nails,around finger nails, Raynud’s phenomenon but gangrene Raynud’s phenomenon but gangrene could occur)could occur) PericarditisPericarditis MyocarditisMyocarditis Conduction defectsConduction defects EndocarditisEndocarditis

F. Respiratory system.F. Respiratory system. RA in pleura or lung.RA in pleura or lung. Pleural effusion.Pleural effusion. Fibrosing alveolitis.Fibrosing alveolitis. Caplan’s lung.Caplan’s lung. Lung nodule.Lung nodule.

G. The kidneys.G. The kidneys. Effecct of NSAIDs.Effecct of NSAIDs. Amyloidosis.( Nephrotic syndrome).Amyloidosis.( Nephrotic syndrome). RA predispose to infection.RA predispose to infection.

Investigations.Investigations. A.A. Laboratory testsLaboratory tests

1.1. Anaemia. ( of chronic diseasea), Anaemia. ( of chronic diseasea), elevation of elevation of

ESR , leucocytosis ( may be) , ESR , leucocytosis ( may be) , thrombocytosis.thrombocytosis.

2.2. Rheumatoid factor is positive in Rheumatoid factor is positive in 75- 80% of 75- 80% of

cases.cases.

3.3. ANA is positive in 20- 40 % . ANA is positive in 20- 40 % .

4.4. Synovial fluid is of inflammatory Synovial fluid is of inflammatory type.( WBC type.( WBC

30.000/ cc).30.000/ cc).

5.Anti CCP. 5.Anti CCP.

B.B. Radiology.Radiology.

1. 1. Soft tissue swelling.Soft tissue swelling.

2. 2. Periarticular osteoporosis.Periarticular osteoporosis.

3. 3. Marginal erosion.Marginal erosion.

Later.Later.

4. 4. Decrease joint space.Decrease joint space.

5.5. More extensive erosion. More extensive erosion.

6. 6. Joint sublaxation & dislocation.Joint sublaxation & dislocation.

7.7. Ankylosis. Ankylosis.

Course & prognosis & causes Course & prognosis & causes of death.of death.

1.1. 20% might improve spontaneously 20% might improve spontaneously (1(1stst. Year). Year). .

2. 2. A small group develop very sever A small group develop very sever form form

( malignant or fulminating RA)( malignant or fulminating RA)

3. 3. Infection ( pyoarthrosis or Infection ( pyoarthrosis or septicaemia).septicaemia).

4.4. Cricoaretenoid arthritis. Cricoaretenoid arthritis.

5. 5. Atlantoaxial sublaxation.Atlantoaxial sublaxation.

6. 6. From hazards of drug therapy.From hazards of drug therapy.

7.7. Amyloidosis. Amyloidosis.

8. the commonest cause of death is 8. the commonest cause of death is cardiac involvement. cardiac involvement.

TreatmentTreatment Treatment is comprehensive ( Medical Treatment is comprehensive ( Medical

, Rehabilitative & Surgical )., Rehabilitative & Surgical ). Aims are :Aims are : 1.1. Education & motivation of the Education & motivation of the

patient .patient . 2.2. Relief of pain. Relief of pain. 3.3. Induction of remission. Induction of remission. 4.4. Maintenance of joint function. Maintenance of joint function. 5.5. Avoid & recognize side effects early. Avoid & recognize side effects early. 6.6. Preserve or restore function. Preserve or restore function. 7.7. Maintain life style. Maintain life style.

Drug therapy.Drug therapy. 1.1.AnalgesiaAnalgesia. .

2.2.NSAIDs.NSAIDs.

3.3. Disease modifying antirheumatic Disease modifying antirheumatic drugs.drugs.

a.a. Gold salts( injectable—sodium Gold salts( injectable—sodium aurothiomalate or oral one auranofine ).aurothiomalate or oral one auranofine ).

( ( b.b. D-penecillamine.) D-penecillamine.)

c.c. Antimalarials ( Chloroquine & Antimalarials ( Chloroquine & Hydroxychloroquine).Hydroxychloroquine).

d.d.Methotrexate.Methotrexate.

e.e. Sulfasalazine. Sulfasalazine.

f.f.Leflunomide.Leflunomide.

Some other cytotoxic drugs like Some other cytotoxic drugs like Azathioprine or Chlorambucile ---etc—Azathioprine or Chlorambucile ---etc—

could be used.could be used.

4. 4. Biologic agents ( Tumor necrosis Biologic agents ( Tumor necrosis factor inhibitors –Infleximab & factor inhibitors –Infleximab & Etanercept ).Etanercept ).

5. 5. Steroids ( systemic or local).Steroids ( systemic or local).

6. 6. Combination therapy.Combination therapy.

Course & prognosis.Course & prognosis. The course is variable; The course is variable;

1.1. 25% remains fit for all activities. 25% remains fit for all activities.

2.2. 40% develop moderate disability. 40% develop moderate disability.

3.3. 25% are quit badly disabled. 25% are quit badly disabled.

4.4. 10% become wheelchair bound. 10% become wheelchair bound.

Poor prognosis is indicated by:Poor prognosis is indicated by: 1.1. High titer rheumatoid factor. High titer rheumatoid factor.

2.2. Early appearance of erosion. Early appearance of erosion.

3.3. Rheumatoid nodules. Rheumatoid nodules.

4.4. Systemic manifestations. Systemic manifestations.

5.5. Presence of HLADR4. Presence of HLADR4.

6.6. Extraarticular manifestations. Extraarticular manifestations.

7.7. Sever functional impairment. Sever functional impairment.

8.8. Continues active disease. Continues active disease.( Remission is better ). ( Remission is better ).

Juvenile Rheumatoid Juvenile Rheumatoid ArthritisArthritis

This is a term used to describe a group of chronic arthritidies that affect children( under age of 16 years) .

Classification.Classification. 1.1. Systemic JCA ( Jevenile chronic Systemic JCA ( Jevenile chronic

arthritis).arthritis).

2.2. Pauciarticular JCA. Pauciarticular JCA.A- A- Young girls .Young girls .

B- B- Older boys ( HLA B27).Older boys ( HLA B27).

3.3. Polyarticular onset ( sero_ Polyarticular onset ( sero_ Negative & sero + ) .Negative & sero + ) .

Characters.Characters. 1.1. Starts befor age of 16 y. Starts befor age of 16 y.

2.2. Lasts at least 3 months. Lasts at least 3 months.

3.3. 1/ 2000 of population are at risk. 1/ 2000 of population are at risk.

4.4. Most are sero- negative for IgM. Most are sero- negative for IgM.

5.5. Peak onset is at 2_ 4 years of age Peak onset is at 2_ 4 years of age with another smaller peak at with another smaller peak at puberty.puberty.

Systemic onset Systemic onset This type makes about 20% of This type makes about 20% of JCA .JCA .

1.1.It occurs under 5 y. of age.It occurs under 5 y. of age.

2.2. Fever & rash are main Fever & rash are main features.features.

3.3. Has prominent extrarticular Has prominent extrarticular features.features.

( lymphadenopathy, splenomegaly, ( lymphadenopathy, splenomegaly, hepatomegaly , pericarditis, pleurisy & hepatomegaly , pericarditis, pleurisy &

abdominal pain).abdominal pain).

Arthritis are of knees,wrists and Arthritis are of knees,wrists and ankles.Cervical spine might be ankles.Cervical spine might be

involved, and tenosynovitis could involved, and tenosynovitis could occurs.occurs.

Systemic JRASystemic JRA

Pauciarticular .Pauciarticular . This account for about 40% of JCA. This account for about 40% of JCA. Arthritis is frequently asymmetric(<5 Arthritis is frequently asymmetric(<5 joints).joints).

1.1.Female predominance ( Early onset Female predominance ( Early onset and usually positive for ANA but not and usually positive for ANA but not RA factor).( there is a risk of RA factor).( there is a risk of iridocyclitis & blindness).Have iridocyclitis & blindness).Have HLADR5 , HLADR8 .HLADR5 , HLADR8 .

Knees , ankles & wrists are Knees , ankles & wrists are frequently affected.frequently affected.

2.2. 2nd type has strong male 2nd type has strong male predominance , later age onset & predominance , later age onset & majority has HLAB27 & gradually majority has HLAB27 & gradually evolves to AS.evolves to AS.

Pauci articular JRAPauci articular JRA

Polyarticular onset. Polyarticular onset. Account for 40% of Account for 40% of cases.Polyarthritic onset. cases.Polyarthritic onset.

Females are more affected . Females are more affected .

About 10% of these are sero + About 10% of these are sero + which has worst prognosiwhich has worst prognosi

s.Arthritis is s.Arthritis is

Symmetrical(Symmetrical(

Knees, anklesKnees, ankles

Small joints…Small joints…

Etc.Etc.

Investigations.Investigations. There is no an absolut diagnostic There is no an absolut diagnostic test. ESR is elevated in the test. ESR is elevated in the active phase, WBC active phase, WBC count might be high, count might be high, ANF is present in 30% of cases & ANF is present in 30% of cases & RA factor in adult type . RA factor in adult type .

X_ ray changes are mainly X_ ray changes are mainly periarticular osteoporosis & periarticular osteoporosis & periosteitis around joints. periosteitis around joints.

In younger children premature In younger children premature appearance of epiphysis & in older appearance of epiphysis & in older ones premature fusion of epiphysis are ones premature fusion of epiphysis are features. Erosion is mainly seen in features. Erosion is mainly seen in sero+ group & comes late in other sero+ group & comes late in other types.types.

Management.Management. Aims.Aims.1.1.Suppress & control the disease activity.Suppress & control the disease activity.2.2.Prevent deformities and correct it.Prevent deformities and correct it.3.3. Help the child to live a normal life. Help the child to live a normal life. Rest & proper positioning in acute Rest & proper positioning in acute phase.phase. Start physiotherapy program as soon Start physiotherapy program as soon

as the child is ready. Splint(s) could be as the child is ready. Splint(s) could be used. used. Suppression of Suppression of

activity. activity. Drugs are more or less are those used in Drugs are more or less are those used in adult RA.adult RA. NSAID are used. NSAID are used. DMARD are used ( Gold , methotrexate ) . DMARD are used ( Gold , methotrexate ) . Steroids are sometimes used ( systemic & Steroids are sometimes used ( systemic & local ). Surgery is restricted to local ). Surgery is restricted to synovectomy, correction of deformities synovectomy, correction of deformities and joint replacement ( hip).and joint replacement ( hip).

Seronegative Seronegative spondarthritides.spondarthritides.

Are a group of hetrogenous Are a group of hetrogenous

disorders charecterised by :disorders charecterised by : 1.1. Constant absence of IgM Constant absence of IgM

rheumatoid serum factor.rheumatoid serum factor. 2.2. Involvment of axial skeleton. Involvment of axial skeleton. 3.3. Peripheral joint involvement. Peripheral joint involvement. 4.4. Clinical evidence of overlap exist Clinical evidence of overlap exist

between them.between them. 5.5. Basic pathological changes is Basic pathological changes is

ENTHESOPATHY.ENTHESOPATHY. 6.6. Tendency for familial aggregation Tendency for familial aggregation

( HLA B27).( HLA B27).

EnthesopathyEnthesopathy

This group consist of:This group consist of:

1.1. AS. AS.

2.2. Psoriatc arthritis. Psoriatc arthritis.

3.3. Enteropathic arthritis. Enteropathic arthritis.

4.4. Reiter’s syndrome(reactive Reiter’s syndrome(reactive arthritis).arthritis).

5.5. Juvenile onset Juvenile onset spondylarthropathyspondylarthropathy

6.6. Undifferentiated Undifferentiated spondylarthropathy.spondylarthropathy.

7.7.Behcet’s disease ?]Behcet’s disease ?]

The common features are:The common features are: 1.1. Negative test for IgM Negative test for IgM

rheumatoid factor.rheumatoid factor.

2.2. Absence of rheumatoid Absence of rheumatoid nodules.nodules.

3.3. Asymmetrical peripheral Asymmetrical peripheral arthritis.arthritis.

4.4. Radiological sacroilitis. Radiological sacroilitis.

5.5. Evidence of clinical overlap Evidence of clinical overlap between them.between them.

6.6. Tendency to familial Tendency to familial aggregation.aggregation.

Diagnostic criteria for diagnosis Diagnostic criteria for diagnosis of spondylarthropathies by Amor of spondylarthropathies by Amor

( 1993).( 1993). 1. Lumbar pain at night or morning stiffness( 1 ). 2. Asymmetrical oligoarthritis ( 2 ). 3. Buttock pain ( 2 ). 4. Alternating buttock pain ( 2 ). 5. Susage like toe or digits ( 2 ).

6. Heel pain or enthesis ( 2 ).

7. Iritis ( 2 ).

8. 8. Nongonococcal urethritis , Nongonococcal urethritis , cervicitis within one cervicitis within one month of onset (1).month of onset (1). 9. 9. Acute diarrhea within one month Acute diarrhea within one month of arthritic of arthritic onset (1).onset (1). 10. 10. Psoriasis , balanitis or Psoriasis , balanitis or inflammatory bowel inflammatory bowel disease (1).disease (1). 11. 11. Sacroiliitis (2).Sacroiliitis (2). 12. 12. Positive test for HLA B27 or Positive test for HLA B27 or positive family positive family history for spodylarthropathy.(2)history for spodylarthropathy.(2) 13. 13. Rapid response to NSAIDs Rapid response to NSAIDs ( within 48 hours ) ( within 48 hours ) (2).(2). Diagnosis require score more than Diagnosis require score more than

( 6 ) scors.( 6 ) scors.

Ankylosing Spondylitis.Ankylosing Spondylitis.

AS is an inflammatory disorder of AS is an inflammatory disorder of unknown aetiology that primarily unknown aetiology that primarily affecting axial skeleton & large affecting axial skeleton & large

proximal joints.proximal joints.

Epidemiology & Aetiology. Epidemiology & Aetiology. AS shows a striking correlation with HLA B27. It s a world wide disease.

HLA B27 is found in 10 to 20% among first degree relatives of of AS patients While it is found in 50% of identical twins of AS patients.

An abnormal response to Klebsiela which carry an antigen mimics B27 has been suggested as underlying cause of AS.These findings indicate that both genetic &environmental factors play role in pathogenesis of AS.

Pathology.Pathology. 1.1. The basic pathological lesion is The basic pathological lesion is ENTHESOPATHY.ENTHESOPATHY.

2. 2. The process usually starts from The process usually starts from Sacroiliac joints.( lower Sacroiliac joints.( lower

part).part).

3. 3. the early lesion is subchondral the early lesion is subchondral granulation tissue granulation tissue

( lymphocyte, plasma cells, mast cells ( lymphocyte, plasma cells, mast cells macrophage & macrophage &

chondrocytes). Usually starts from the chondrocytes). Usually starts from the lower 2/3lower 2/3

synovial part of sacroiliac joint & synovial part of sacroiliac joint & thinner iliac cartilagethinner iliac cartilage

is first affected.is first affected.

4.4. The inflamed joint margin become The inflamed joint margin become widened , irregular widened , irregular

later on calcified( ossified) ultimately later on calcified( ossified) ultimately the joint may bethe joint may be

totally obliterated.totally obliterated.

5.5. In the spine ( Squering of vertebrae In the spine ( Squering of vertebrae ossification of outer most fibers of ossification of outer most fibers of annulas fibrosus, syndesmophytes & annulas fibrosus, syndesmophytes & eventually Bamboo spine).eventually Bamboo spine).

Diffuse osteoporosis of the spine could Diffuse osteoporosis of the spine could occur, erosion and sclrerosis of occur, erosion and sclrerosis of verteberal bodies at disk margine , verteberal bodies at disk margine , squaring of vertebrae & inflammation & squaring of vertebrae & inflammation & destruction of disk_ bone border . destruction of disk_ bone border . Inflammatory arthritis of the apophysial Inflammatory arthritis of the apophysial joints is common. joints is common.

6.6. Inflammatory arthritis might occur in Inflammatory arthritis might occur in peripheral jointsperipheral joints

( hip is commonest ).( hip is commonest ).

7. 7. Enthesopathy ( plantar fasciitis, tennis Enthesopathy ( plantar fasciitis, tennis elbow etc….).elbow etc….).

8.8.Other cartiligenous joints i.e Other cartiligenous joints i.e Sternomnibural & pubic symphysis Sternomnibural & pubic symphysis could be affected.could be affected.

9.9.Eyes ( anterior uveitis ( iritis) Eyes ( anterior uveitis ( iritis) occurs in 20% of cases.occurs in 20% of cases.

10.10.Aortic insufficiency occurs in small Aortic insufficiency occurs in small number of cases.number of cases.

11.11.Microscopical inflammatory lesions Microscopical inflammatory lesions of colon & ileocecal valve have been of colon & ileocecal valve have been found in 25__ 50% of patients even found in 25__ 50% of patients even without clinical evidence of without clinical evidence of inflammatory bowel disease. inflammatory bowel disease.

EnthesopathyEnthesopathy

Clinical Manifestations.Clinical Manifestations. 0.1 – 1% of population affected.0.1 – 1% of population affected.

Age onset is 16—40 y.Age onset is 16—40 y.

Male / female is 2—3 / 1.Male / female is 2—3 / 1.

Onset is insidious.Onset is insidious.

Low back pain & stiffness are the usual Low back pain & stiffness are the usual presenting presenting

symptoms.symptoms.

1.1.The pain is insidious & dull ache in The pain is insidious & dull ache in nature , felt nature , felt

deep in the lower lumber & gluteal deep in the lower lumber & gluteal region ( sacroiliacregion ( sacroiliac

region). region).

2.2.Morning stiffness up to few hours.Morning stiffness up to few hours.

3.3.Might have constitutional Might have constitutional symptoms.symptoms.

4.4. Symtoms are initially usually Symtoms are initially usually episodic.episodic.

5.5. Nocturnal exacerbation that make Nocturnal exacerbation that make the patient to the patient to

walk around. walk around.

6.6. Tender lower back. Tender lower back.

7.7.Decrease spinal movement in all Decrease spinal movement in all directionsdirections

which spreads up to thoracic & which spreads up to thoracic & cervical regions.cervical regions.

8.8. Enthesopathy ( plantar fasciitis , Enthesopathy ( plantar fasciitis , tennis elbow tennis elbow

etc….).etc….).

9.9. Occasionally boney chest pain is a Occasionally boney chest pain is a presentingpresenting

symptom.symptom.

10.10. Involvement of costovertebral joint Involvement of costovertebral joint results in results in

the reduction of chest expansion.the reduction of chest expansion.

11.11.Peripheral asymmetrical arthritis Peripheral asymmetrical arthritis might occurmight occur

( hip is commonest ).( hip is commonest ).

12.12. Eye ivolvement occur in 40% of Eye ivolvement occur in 40% of cases (scarring of uveal tract with cases (scarring of uveal tract with secondary glaucoma).secondary glaucoma).

13.13. Aortic insufficiency might produce Aortic insufficiency might produce heart heart

failure.failure.

14.14.In lung apical fibrosis & cavitation In lung apical fibrosis & cavitation mightmight

occur.occur.

Physical signs:Physical signs:

1.1. Decrease spinal movments ( schober Decrease spinal movments ( schober test) test)

2.2. Tender SI joints . Tender SI joints . 3.3. Decrease chest expansion. Decrease chest expansion. 4.4. Decrease hip movments. Decrease hip movments. 5.5. Kyphosis in thoracic spine & Kyphosis in thoracic spine &

reduced lumbar lordosis.reduced lumbar lordosis. 6.6. Atrophy of buttocks muscles. Atrophy of buttocks muscles.

Investigations.Investigations.

ESR is elevated in acute phase & so ESR is elevated in acute phase & so C- reactive C- reactive

protein protein

Normochoromic normocytic anemia .Normochoromic normocytic anemia .

Alkaline phosphates & IgG might be Alkaline phosphates & IgG might be high.high.

HLAB27 is present in above 90% of HLAB27 is present in above 90% of cases.cases.

Radiology ( imagingRadiology ( imaging ). ).

MRI & CT scan are showing MRI & CT scan are showing changes before conventional changes before conventional

x—ray. x—ray.A. Radiological changes in S.I joints A. Radiological changes in S.I joints

are :are :

1.1. Blurring of subcortical Blurring of subcortical margine of subchondral bone.margine of subchondral bone.

2.2. Erosions & sclerosis. Erosions & sclerosis. 3.3. Pseudowidnening of the joint. Pseudowidnening of the joint. 4.4. Boney ankylosis. Boney ankylosis.

Normal SI joint Sacro Normal SI joint Sacro ilitisilitis

B.B. In the spine.In the spine. 1.1. Squaring of vertebra. Squaring of vertebra.

2.2. Ossification of outer layer of Ossification of outer layer of annulas firosus.annulas firosus.

3.3. Marginal syndesmophyte. Marginal syndesmophyte.

4.4. Bamboo spine. Bamboo spine.

Bamboo spineBamboo spine

Treatment.Treatment.

The principles are:The principles are:

1.1. Relief pain. Relief pain.

2.2. Proper positioning. Proper positioning.

3.3. Exercise program to maintain Exercise program to maintain mobility & preserve range of mobility & preserve range of motion.motion.

Drug therapy:Drug therapy: 1.1. NSAIDs.( Indomthacine, NSAIDs.( Indomthacine,

phenylbutazone can be used ).phenylbutazone can be used ). 2.2. Sulfasalzine & MTX are used when Sulfasalzine & MTX are used when

there is peripheral joint there is peripheral joint involvement.( used in spinal involvement.( used in spinal involvement).involvement).

3.3. Tumor necrosis factor inhibitors Tumor necrosis factor inhibitors are effective (Infleximab or are effective (Infleximab or Etanercept).Etanercept).

4.4. Systemic steroid might be used Systemic steroid might be used ( intractable uveitis )( intractable uveitis )

5.5. Local steroid to affected joints Local steroid to affected joints could be used (CT guided for SI could be used (CT guided for SI joints). joints).

Psoriatic ArthritisPsoriatic Arthritis

PsA is a chronic disease sero-PsA is a chronic disease sero-negative inflammatory arthritis that negative inflammatory arthritis that affects 5_7% of people with cutaneous affects 5_7% of people with cutaneous psoriasis.psoriasis. In adults it mostly occurs in patients In adults it mostly occurs in patients with active cutaneous disease while in with active cutaneous disease while in children the joint manifestations children the joint manifestations might antedate articular features. might antedate articular features. The extend of psoriatic skin disease The extend of psoriatic skin disease correlates poorly with the onset of correlates poorly with the onset of arthritis , the risk of PsA increases arthritis , the risk of PsA increases with family history of with family history of spondylarthropathy or extensive nail spondylarthropathy or extensive nail pitting.pitting.

Aetiology & Pathogenesis. Aetiology & Pathogenesis.

The cause & pathogenesis of PsA is The cause & pathogenesis of PsA is unknown . There is a strong evidence unknown . There is a strong evidence of genetic influence in the disease over of genetic influence in the disease over 30% having family history of the 30% having family history of the disorder there is an increased disorder there is an increased frequency of ( HLA_B17 , CW6, DQ2 frequency of ( HLA_B17 , CW6, DQ2 &/ or HLA_B27) in patients with &/ or HLA_B27) in patients with psoriatic spondylitis while B27, BW62 psoriatic spondylitis while B27, BW62 , B38, B39 & DR7 have noted in , B38, B39 & DR7 have noted in association with peripheral arthritis . association with peripheral arthritis . Fulminating cases should make one Fulminating cases should make one suspect human immunodeficiency suspect human immunodeficiency virus disease ( HIV ).virus disease ( HIV ). Trauma & infection suggested to Trauma & infection suggested to increase cellular immunity ( i.e increase cellular immunity ( i.e streptococcus) may each play a role.streptococcus) may each play a role.

Clinical Features. Clinical Features. PsA has an insidious onset & PsA has an insidious onset & progressive course.progressive course.

The onset of arthritis is usually The onset of arthritis is usually preceded by skin disease but may preceded by skin disease but may accompany it or the arthritis may accompany it or the arthritis may preced the skin lesion ( as in preced the skin lesion ( as in children).children).

There are at least five clinical There are at least five clinical subsets.ssubsets.s

1.1. Asymmetric oligoarthritis : Asymmetric oligoarthritis : Characterised by asymmetric Characterised by asymmetric involvement of both large & involvement of both large & small joints & the appearance of small joints & the appearance of susage _ shaped digits is susage _ shaped digits is common. This subset is making common. This subset is making about 30__50 % of patients , about 30__50 % of patients , there is a little relationship there is a little relationship between the joint & skin between the joint & skin activity.activity.

2.2. Involvement of DIP joints. Is Involvement of DIP joints. Is seen in 10% of cases it is strongly seen in 10% of cases it is strongly associated with nail changes of associated with nail changes of pitting , onycolysis , subungal pitting , onycolysis , subungal keratosis &Ridging.keratosis &Ridging.

Psoriatic arthritis with distal joint Psoriatic arthritis with distal joint involvement in the third and fifth involvement in the third and fifth

digits (arrow). Onycholysis is also seen digits (arrow). Onycholysis is also seen in most of the fingernails. in most of the fingernails.

3.3. Symmetric polyarthritis : Symmetric polyarthritis : Resembling RA.Resembling RA.

4.4. Psoriatic spondylitis : is Psoriatic spondylitis : is making about 20% making about 20%

of PsA , 50% are having of PsA , 50% are having HLA_B27 , theyHLA_B27 , they

have sacroiliitis . have sacroiliitis .

There is malepredominance.There is malepredominance.

5.5. Arthritis mutilance : There is a Arthritis mutilance : There is a severe severe

destructive arthropathy destructive arthropathy resulting in resulting in

mutilation of joints & mutilation of joints & telescoping digits totelescoping digits to

produce the so called opera produce the so called opera glass hand.glass hand.

Skin & nail changes. Skin & nail changes. Psoriatic lesions are typically Psoriatic lesions are typically occur on the occur on the

scalp , elbow,behind ears & scalp , elbow,behind ears & knees.knees.

Nail changes include small pits , Nail changes include small pits , onycolysis, subungual keratosis , onycolysis, subungual keratosis , thickening & distortion of nail thickening & distortion of nail with ridging. Presence of 20 pits with ridging. Presence of 20 pits in total is suggestive of psoriasis in total is suggestive of psoriasis while presence of more than 60 while presence of more than 60 pits been diagnostic.pits been diagnostic.

This photograph of the hand of a patient This photograph of the hand of a patient with psoriatic arthritis shows characteristic with psoriatic arthritis shows characteristic early nail separation (onycholysis), swelling early nail separation (onycholysis), swelling and erythema of the index and little finger and erythema of the index and little finger

distal interphalangeal joints. distal interphalangeal joints.

Investigations.Investigations. 1.1. Elevated ESRin 1/3 of cases, C—reactive Elevated ESRin 1/3 of cases, C—reactive protein & protein &

complement levels reflect inflammation.complement levels reflect inflammation.

2.2. Rheumatoid factors are absent. Rheumatoid factors are absent.

3.3. Mild normochromic normocytic anemia Mild normochromic normocytic anemia is seen in is seen in

chronic cases.chronic cases.

4.4. Immunological levels esoecially IgA level Immunological levels esoecially IgA level may bemay be

elevated.elevated.

5.5. Synovial fluid is of inflammatory type. Synovial fluid is of inflammatory type.

6.6. ANA could be positive in low titre & anti ANA could be positive in low titre & anti dsDNA dsDNA

in 3% of cases.in 3% of cases.

7.7. AntiCCP could be positiove in 8—16% . AntiCCP could be positiove in 8—16% .

8.8. Radiology. Radiology.

Soft tissue swelling , loss of cartilage Soft tissue swelling , loss of cartilage space, erosion , boney ankylosis of space, erosion , boney ankylosis of fingers , sublaxation & subchondral fingers , sublaxation & subchondral bone cyst. There is less bone bone cyst. There is less bone demineralization. More unique & demineralization. More unique & suggestive of PsA arthritis are erosion suggestive of PsA arthritis are erosion of DIP joints , expansion of base of of DIP joints , expansion of base of terminal phalanx & terminal terminal phalanx & terminal phalangeal osteolysis ( reabsorption).phalangeal osteolysis ( reabsorption).

Diagnosis of of PsA depends on finding typical Diagnosis of of PsA depends on finding typical cutaneous or nail changes in association with one of cutaneous or nail changes in association with one of recognized articular variantsrecognized articular variants..

Psoriatic arthritis associated with pencil-in-cup Psoriatic arthritis associated with pencil-in-cup

abnormality in the distal interphalangeal (DIP) joints abnormality in the distal interphalangeal (DIP) joints of the first and second fingers (short arrows), plus of the first and second fingers (short arrows), plus early changes in the DIP joint of the fourth finger. early changes in the DIP joint of the fourth finger. Other changes include ankylosis in the DIP joint in Other changes include ankylosis in the DIP joint in the fifth finger (long arrow) and destruction of the the fifth finger (long arrow) and destruction of the wrist.wrist.

The axial skeleton shows asymmetric The axial skeleton shows asymmetric or unilateral sacroiliitis , often a or unilateral sacroiliitis , often a symptomtic paravertebral symptomtic paravertebral ossification including cervical spine ossification including cervical spine & large asymmetric & large asymmetric nonmarginal osteophytes.nonmarginal osteophytes.

TreatmentTreatment.. 1.1. NSAIDs. NSAIDs. 2.2. DMARD ( Sulfasalazine , gold , D_ DMARD ( Sulfasalazine , gold , D_

penicillamin & Methotrexate ) could be used penicillamin & Methotrexate ) could be used , while the use of antimaliarials is , while the use of antimaliarials is controversial . MTX is particularly effective controversial . MTX is particularly effective in managing the skin & joint disease.in managing the skin & joint disease.

3.3. Itra-articular steroid could be used in Itra-articular steroid could be used in absence of psoriatic lesions around the joint absence of psoriatic lesions around the joint injected.injected.

4.4. Systemic steroid is better to be avoided since Systemic steroid is better to be avoided since tapering tends to cause an exacerbation of tapering tends to cause an exacerbation of the skin lesion.the skin lesion.

5.5. Azathioprin may be used . Azathioprin may be used . 6.6. Spinal disease is treated as AS. Spinal disease is treated as AS. 7.7. Biological agents could be used. Biological agents could be used. In most cases the prognosis is good & joint In most cases the prognosis is good & joint

function is preserved or slightly impaired , function is preserved or slightly impaired , but deformity & disability occur in some but deformity & disability occur in some patients.patients.

Reactive Arthritis.Reactive Arthritis. Is refers to occurance of an acute Is refers to occurance of an acute

nonsuppurative , sterile inflammatory nonsuppurative , sterile inflammatory arthropathy arising after an infectious arthropathy arising after an infectious process but at a site remote from the process but at a site remote from the primary infection.primary infection.

The microbial pathogens commonly The microbial pathogens commonly associated with reactive arthritis are associated with reactive arthritis are shigella , salmonella , yersenia & shigella , salmonella , yersenia & Chlamydia.Chlamydia.

Many reactive arthritis occur after a Many reactive arthritis occur after a known infection & therefore have known infection & therefore have named post_ infection.named post_ infection.

Reactive arthritis begins as Reactive arthritis begins as asymmetrical oligoarthritis often asymmetrical oligoarthritis often precede by an identifiable infectious precede by an identifiable infectious event by one to four weeks.event by one to four weeks.

The common example of reactive The common example of reactive arthritis is Reiter’ s syndrome.arthritis is Reiter’ s syndrome.

COURSE AND PROGNOSISCOURSE AND PROGNOSIS In general, reactive arthritis follows one of In general, reactive arthritis follows one of

four courses after the initial acute attack . four courses after the initial acute attack .

1. 1. The arthritis attack is self-limiting and The arthritis attack is self-limiting and may never recur, with a duration usually may never recur, with a duration usually less than six months (35 percent). Pain at less than six months (35 percent). Pain at the metatarsophalangeal joints and the the metatarsophalangeal joints and the heels might remain painful for months. heels might remain painful for months. Approximately 75 percent of patients are in Approximately 75 percent of patients are in complete remission after two years. complete remission after two years.

2. 2. The disease goes into remission but The disease goes into remission but recurs intermittently (35 percent). The recurs intermittently (35 percent). The recurrence might only be an arthritis, an recurrence might only be an arthritis, an enthesopathy, or be found in an enthesopathy, or be found in an extraarticular location. extraarticular location.

3.3. The attack never subsides The attack never subsides completely but continues with a completely but continues with a waxing and waning course (25 waxing and waning course (25 percent). percent).

4.4. A few patients continue to have A few patients continue to have severe inflammatory disease for severe inflammatory disease for many years and may even develop many years and may even develop features of destructive arthritis in features of destructive arthritis in multiple joints or the diffuse spinal multiple joints or the diffuse spinal changes of ankylosing spondylitis (5 changes of ankylosing spondylitis (5 percent).percent).

Reiter’s SyndromeReiter’s Syndrome This syndrome consists of the triad of This syndrome consists of the triad of seronegative reactive arthritis seronegative reactive arthritis nonspecific uerethritis nonspecific uerethritis ConjunctivitisConjunctivitis ( this traid is only observed in 33% of ( this traid is only observed in 33% of

patients ), many patients will not have patients ), many patients will not have prodromal enteric or urethral prodromal enteric or urethral inflammation such patients are inflammation such patients are designated as incomplete Reiter’s. designated as incomplete Reiter’s.

When arthritis alone follows sexual When arthritis alone follows sexual exposure or enteric infection the term exposure or enteric infection the term reactive arthritis is frequently used.reactive arthritis is frequently used.

Clinical Features Clinical Features The earlest features of RS frequently appear The earlest features of RS frequently appear

within 1__4 weeks of Putative microbial within 1__4 weeks of Putative microbial exposure. Disease onset is usually heralded exposure. Disease onset is usually heralded by development of one or of the by development of one or of the extraarticular feature.extraarticular feature.

The diagnostic traid in RS is The diagnostic traid in RS is (urethritis ,conjunctivitis & arthritis ) one (urethritis ,conjunctivitis & arthritis ) one of this triad may of this triad may

be absent. be absent. Urethritis can occur in the postdysentric Urethritis can occur in the postdysentric

form of the disease & the reverse is also form of the disease & the reverse is also possible.possible.

Age range is 16__ 35 Y, male / female is Age range is 16__ 35 Y, male / female is 20 / 1 in post-urethritic infection , while it 20 / 1 in post-urethritic infection , while it has an equal sex distribution in post-has an equal sex distribution in post-dysenteric one.dysenteric one.

Arthritis Arthritis : : Is often the last feature to appear , it is Is often the last feature to appear , it is

sero- negative mono or oligoarthritis sero- negative mono or oligoarthritis affecting weight bearing joints ( knees, affecting weight bearing joints ( knees, metatarsophalangeal , ankles or metatarsophalangeal , ankles or interphalangeal joints ). In half of the interphalangeal joints ). In half of the cases the onset is acute ( acute arthritis cases the onset is acute ( acute arthritis with effusion ). The severity of arthritis with effusion ). The severity of arthritis is varies from mild transient to chronic is varies from mild transient to chronic destructive .destructive .

Enthesitis is might be a feature .Enthesitis is might be a feature .

Sacroiliitis & spondylitis occur in about Sacroiliitis & spondylitis occur in about 50% of cases & lead to backache( with 50% of cases & lead to backache( with radiological changes in chronic cases ). radiological changes in chronic cases ).

ACR criteria for diagnosis require the ACR criteria for diagnosis require the presence of peripheral arthritis of presence of peripheral arthritis of more than one month duration more than one month duration occurring in association with occurring in association with urethritis &/ or cervicitis.urethritis &/ or cervicitis.

Isolated involvement of lumbar & Isolated involvement of lumbar & thoracic spine could be an initial thoracic spine could be an initial radiographic findings & involvement radiographic findings & involvement of cervical spine is uncommon.of cervical spine is uncommon.

Urethritis :Urethritis : is nongonococcal , is usually the first is nongonococcal , is usually the first

feature , manifests as a clear sterile feature , manifests as a clear sterile discharge with minimal dysuria , it discharge with minimal dysuria , it might be asymptomatic . might be asymptomatic . Occasionally it is sever.Occasionally it is sever.

Occlar lesion :Occlar lesion :

Bilateral sterile conjunctivitis is the Bilateral sterile conjunctivitis is the most common form & is usually mild most common form & is usually mild but occasionally may be sever with but occasionally may be sever with almost all parts of the eye involved.almost all parts of the eye involved.

Mucocutaneous lesions :Mucocutaneous lesions : Features may affect the GU or GI tracts . In Features may affect the GU or GI tracts . In

GU are ( transient mucopurulent GU are ( transient mucopurulent discharge , urethritis, circnate balanitis discharge , urethritis, circnate balanitis appears as painless vesicls or large, appears as painless vesicls or large, shallow, serpiginous ulceration or plaques shallow, serpiginous ulceration or plaques on the glans or shaft of the penis . on the glans or shaft of the penis .

Painless lingual, palatal & oral ulceration Painless lingual, palatal & oral ulceration may be seen in up to 50% of patients.may be seen in up to 50% of patients.

Keratdermia blennorrhagica ( is painless Keratdermia blennorrhagica ( is painless papulosquematous eruption frequently papulosquematous eruption frequently found on the sole or palm, it heals found on the sole or palm, it heals without leaving scar ).without leaving scar ).

Patients with chronic disease might Patients with chronic disease might demonstrate nail changes (onycholysis demonstrate nail changes (onycholysis or subungual hyperkeratosis).or subungual hyperkeratosis).

Other features : As sever systemic Other features : As sever systemic upset with high fever , loss of upset with high fever , loss of weight . weight .

Pericarditis ,pleurisy & transient Pericarditis ,pleurisy & transient pulmonary infiltrate might occur. pulmonary infiltrate might occur.

Myocarditis , heart block & aortic Myocarditis , heart block & aortic incompetence are very rare.incompetence are very rare.

Investigations :Investigations : ESR is elevated in acute phase , WBC ESR is elevated in acute phase , WBC

may be high & mild normochromic may be high & mild normochromic normocytic anemia might present.normocytic anemia might present.

HLAB27 is present in about 80% HLAB27 is present in about 80% while serum rheumatoid factor & ANA while serum rheumatoid factor & ANA are absent.are absent.

Rdiological findings are : Periaticular Rdiological findings are : Periaticular osteoporosis, reduction of joint space osteoporosis, reduction of joint space & erosions can be seen in prolonged or & erosions can be seen in prolonged or recurrent arthritis. Periosteitis might recurrent arthritis. Periosteitis might be seen. Sacroiliitis also could occur.be seen. Sacroiliitis also could occur.

Management:Management: 1. 1. NSAIDs are useful for most of the NSAIDs are useful for most of the

patients.patients.

2 .2 . Sulfasalzine ( 2__ 3 gms ) . Sulfasalzine ( 2__ 3 gms ) .

3.3. MTX or Azathioprine could be MTX or Azathioprine could be used used

4.4. Uveitis is may be medical Uveitis is may be medical emergency requiring topical, emergency requiring topical, subconjunctival or systemic steroid.subconjunctival or systemic steroid.

5. Tetracycline is effective in 5. Tetracycline is effective in treatment of nonspecific urethritis. treatment of nonspecific urethritis.

6.The use of TNF inhibitor agents for 6.The use of TNF inhibitor agents for patients with reactive arthritis and patients with reactive arthritis and spondyloarthropathy is similar to spondyloarthropathy is similar to that in patients with rheumatoid that in patients with rheumatoid arthritis.arthritis.

Prognosis & course of RS is varied Prognosis & course of RS is varied &unpredictable..&unpredictable..

Patients with AIDS may develop an Patients with AIDS may develop an aggressive form of RS. The vast aggressive form of RS. The vast majority of AIDs patients with RS are majority of AIDs patients with RS are having HLAB27 antigen. having HLAB27 antigen.

Enteropathic Arthropathy.Enteropathic Arthropathy. Enteropathic arthritis refers to the Enteropathic arthritis refers to the

arthropathies associated with bowel arthropathies associated with bowel diseases ( usually Crhon’ s disease diseases ( usually Crhon’ s disease or Ulcerative colitis ).or Ulcerative colitis ).

These disorders are unified by clinical These disorders are unified by clinical & histological gut inflammation & histological gut inflammation

Alterd intestinal permeability & Alterd intestinal permeability & development of an inflammatory development of an inflammatory peripheral or axial arthritis . peripheral or axial arthritis .

Peripheral arthritis is observed in 20% Peripheral arthritis is observed in 20% & axial arthritis in 10__ 15% of & axial arthritis in 10__ 15% of patients .patients .

Peripheral arthritis occur more Peripheral arthritis occur more frequently in those with frequently in those with extraintestinal manifestations (-extraintestinal manifestations (-erythema nodosum )erythema nodosum )

Males & females are equally affected . Males & females are equally affected .

All age groups are affected .All age groups are affected .

Disease onset features are low grade Disease onset features are low grade fever , painful oral ulceration , ocular fever , painful oral ulceration , ocular manifestations, cutaneous manifestations, cutaneous manifestations ( erythema nodosum ) manifestations ( erythema nodosum ) or enthesis .or enthesis .

Peripheral arthritis manifests as Peripheral arthritis manifests as inflammatory , non-erosive . inflammatory , non-erosive . asymmetrical mono or oligoarthritis asymmetrical mono or oligoarthritis affecting large joints ( knees, ankles, affecting large joints ( knees, ankles, elbws etc….).elbws etc….).

Initially the arthritis may be migratory Initially the arthritis may be migratory & may resolve in weeks or months.& may resolve in weeks or months.

Controlling gut problem may prove Controlling gut problem may prove beneficial or managing peripheral beneficial or managing peripheral arthritis.arthritis.

Axial arthritis is clinically Axial arthritis is clinically indistinguishable from AS. 50% of indistinguishable from AS. 50% of patients with axial skeleton patients with axial skeleton involvement having HLAB27.involvement having HLAB27.

Treatment. Treatment. 1.1. Control the underlying gut disease. Control the underlying gut disease.

2.2. NSAIDs. NSAIDs.

3.3. Aspiration & local steroid injection. Aspiration & local steroid injection.

4.4. Sulfasalazine. Sulfasalazine.

5. Biologic agents( Infliximab)5. Biologic agents( Infliximab)

Behcet’ s SyndromBehcet’ s Syndrom This vasculitis of unknown aetiology This vasculitis of unknown aetiology

is characteristically targets venuls. It is characteristically targets venuls. It is common in silk route countries , is common in silk route countries , Mediterranean & Japan. It an Mediterranean & Japan. It an assoiation with HLAB5 & subtype 51.assoiation with HLAB5 & subtype 51.

Clinical featuresClinical features

Oral aphthous ulceration( 93___ Oral aphthous ulceration( 93___ 100%)100%)

Genital ulcers (69__ 100% ) Genital ulcers (69__ 100% )

Occular symptoms ( 50___ 79%)Occular symptoms ( 50___ 79%)

Arthritis 35___50%),Arthritis 35___50%),

Skin lesions ( 35__65%)Skin lesions ( 35__65%)

CNS disease(10__30%)CNS disease(10__30%)

Major vessels occlusion/ aneurysm Major vessels occlusion/ aneurysm (10__37%) & constitutional symptoms (10__37%) & constitutional symptoms 63%.63%.

Recurrent oral ulceration:Recurrent oral ulceration: Aphthous like stomatitis Aphthous like stomatitis is initial manifestation in 25__75% of is initial manifestation in 25__75% of patients. Preferential sites are patients. Preferential sites are mucous membrane of the lips, mucous membrane of the lips, gingiva, check (buccal mucosa) & gingiva, check (buccal mucosa) & tounge. Ulcers are usually deep, tounge. Ulcers are usually deep, multiple& painful & last for 10__30 multiple& painful & last for 10__30 days & heal without scarring .days & heal without scarring .

Genital ulcers :Genital ulcers : Are similar to Are similar to mouth ulcers occur on mouth ulcers occur on genitalia( scrotum, vulva, penis, genitalia( scrotum, vulva, penis, perianal& vaginal mucosa). Lesions perianal& vaginal mucosa). Lesions in male are more painful than in in male are more painful than in women , genital ulcers are usually women , genital ulcers are usually deeper than oral ones & may leave deeper than oral ones & may leave scar after healing, vulvar ulcers often scar after healing, vulvar ulcers often develop during the premenstrual develop during the premenstrual stage.stage.

Other features commonly occur Other features commonly occur include : thrombophlebitis , include : thrombophlebitis , erythema nodosum , pastules & erythema nodosum , pastules & folliculitis. folliculitis.

The pustular reaction of the skin to The pustular reaction of the skin to intradermal skin prick ( some times intradermal skin prick ( some times referred as pathergy test or called referred as pathergy test or called venepuncture) , this reaction occur venepuncture) , this reaction occur in 70% 0f cases , usually occur in in 70% 0f cases , usually occur in those with extensive disease, the test those with extensive disease, the test is nonspecific occuring in 7% of is nonspecific occuring in 7% of healthy control people.healthy control people.

Arhritis occur in about 60% of cases is Arhritis occur in about 60% of cases is mono or oligoarticular usually mono or oligoarticular usually intermittent , self-limited non intermittent , self-limited non erosive localized to knees & ankles erosive localized to knees & ankles & other lower limb joints may be & other lower limb joints may be affected.affected.

The CNS can be involved with The CNS can be involved with meningoencephalitis , other meningoencephalitis , other manifestations include seizure , manifestations include seizure , cranial nerve palsies, bulbar palsy, cranial nerve palsies, bulbar palsy, ataxia, transient ischaemic attacks & ataxia, transient ischaemic attacks & strokes.strokes.

Small & large ulcers in the gut Small & large ulcers in the gut produce symptoms of produce symptoms of inflammatory bowel disease & inflammatory bowel disease & perforation might occur.perforation might occur.

The disease follows a course of The disease follows a course of relaps & remissions.relaps & remissions.

Recurrent uveitis can be very Recurrent uveitis can be very sever & result in blindness& it is sever & result in blindness& it is often bilateral. often bilateral.

uveitis, iritis &hypopyonuveitis, iritis &hypopyon

Diagnostic criteria of Behcet s Diagnostic criteria of Behcet s disease : disease :

The International Crieteria for The International Crieteria for Behcet’s disease was created Behcet’s disease was created in 2006 is, the presence of in 2006 is, the presence of two of the following(genital two of the following(genital ulcer,skin lesions,eye lesions ulcer,skin lesions,eye lesions and pathergy test) will and pathergy test) will diagnose/classify the patient diagnose/classify the patient as Behcet(BD).as Behcet(BD).

Old Crietera:oral ulceration Old Crietera:oral ulceration plus 2 of the followings :plus 2 of the followings :

1. Recurrent genital 1. Recurrent genital ulceration.ulceration.

2. Eye lesions.2. Eye lesions.

3. Skin lesions.3. Skin lesions.

4. Pathergy test.4. Pathergy test.

Treatment:Treatment: 1.1. Oral & genital ulcers are treated Oral & genital ulcers are treated

symptomatically with local symptomatically with local unaesthetic & steroid cream.unaesthetic & steroid cream.

2.2. Thrombosis of major vessels needs Thrombosis of major vessels needs anticoagulant therapy.anticoagulant therapy.

3.3. CNS involvement is an indication CNS involvement is an indication for high dose of steroid.for high dose of steroid.

4.4. Colchicin or interferon—a can be Colchicin or interferon—a can be beneficial for arthritis & beneficial for arthritis & mucocutneous lesions.mucocutneous lesions.

5.5. Cyclosporin has shown promising in Cyclosporin has shown promising in reducing ocular attacks.reducing ocular attacks.

6.6. MTX is used. MTX is used.

7.7. TNF blckers are used. TNF blckers are used.

8.8. Arhritis tends to be the least Arhritis tends to be the least troublesome of the complications , it troublesome of the complications , it is self-limiting & responds well to is self-limiting & responds well to NSAIDs &/ or intraarticular steroid. NSAIDs &/ or intraarticular steroid.

Infliximab might be used.Infliximab might be used.

Crystal induced Crystal induced ArthropathyArthropathy

The crystal arthropathies include The crystal arthropathies include gout , calcium pyrophosphate gout , calcium pyrophosphate deposition disease (CPPD) or deposition disease (CPPD) or pseudogut , basic calcium phosphate pseudogut , basic calcium phosphate syndromes , & calcium oxalate syndromes , & calcium oxalate arthritis arthritis

GOUTGOUT Gout is a systemic disease resulting Gout is a systemic disease resulting

from abnormality of serum uric acid from abnormality of serum uric acid metabolism , characterized by metabolism , characterized by hyperuicemia & uric acid crystal hyperuicemia & uric acid crystal deposition in joints causing acute deposition in joints causing acute gouty arthritis, in soft tissue causing gouty arthritis, in soft tissue causing tophi & tenosynovitis & in the tophi & tenosynovitis & in the urinary tract causing urate stones.urinary tract causing urate stones.

Chronic hyperuricemia is necessary for Chronic hyperuricemia is necessary for the development of gout ( serum uric the development of gout ( serum uric acid more than 7.0 mg/dl in males & acid more than 7.0 mg/dl in males & 6.0 mg/dl in females) .6.0 mg/dl in females) .

Urat deposition in the synovial Urat deposition in the synovial membrane leads to an intense & membrane leads to an intense & often destructive arthritisoften destructive arthritis

Gout occurs mainly in developed Gout occurs mainly in developed countries in USA the prevalence is countries in USA the prevalence is 13.6/1000 in adult males & 6.4/1000 13.6/1000 in adult males & 6.4/1000 in females, while the prevalence of in females, while the prevalence of hyperuricemia is about 5% .hyperuricemia is about 5% .

At puberty male serum uric acid level At puberty male serum uric acid level rise & remain higher than those for rise & remain higher than those for female until menopause.female until menopause.

Gout is very uncommon before Gout is very uncommon before puberty.puberty.

Serum uric concentrations are related Serum uric concentrations are related to several demographic factors to several demographic factors ( age , gender , body bulk , life style, ( age , gender , body bulk , life style, economic state & genetic economic state & genetic constitution).constitution).

It is higher in the urban than in rural It is higher in the urban than in rural communities & correlates positively communities & correlates positively with social class, weight & high with social class, weight & high protein diet.protein diet.

Uric acid production & elimination. Uric acid production & elimination.

The serum urate level depends on the The serum urate level depends on the production & its disposal. The majority production & its disposal. The majority of urate is produced by hepatic xanthin of urate is produced by hepatic xanthin oxidase acting on hypoxanthin & oxidase acting on hypoxanthin & xanthin, derived by degredation of xanthin, derived by degredation of neucleic acid of senescent cells& from neucleic acid of senescent cells& from the metabolic turnover of cellular purin the metabolic turnover of cellular purin neucleotides . neucleotides .

Less than 10% of uric acid comes from Less than 10% of uric acid comes from performed dietary nucleotides & performed dietary nucleotides & nucleosides , about 60% of uric acid is nucleosides , about 60% of uric acid is replaced daily by constant turnover, the replaced daily by constant turnover, the quantity produced is proportional to the quantity produced is proportional to the body size.body size.

The uric acid is complety filtered by The uric acid is complety filtered by the glomerulus 100% is then the glomerulus 100% is then reabsorbed in the proximal tubules reabsorbed in the proximal tubules &then 75% is secreted by distal &then 75% is secreted by distal tubules some postreabsorption also tubules some postreabsorption also takes place.takes place.

The kidneys dispose 2/3 of uric acid The kidneys dispose 2/3 of uric acid and the remainder is filtered in the and the remainder is filtered in the gut luman, where part of it degraded gut luman, where part of it degraded by bacteria in the gut, this increases by bacteria in the gut, this increases substantially in renal insufficiency .substantially in renal insufficiency .

Pathogenesis. Pathogenesis. The biochemical abnormality is The biochemical abnormality is

hyperuricemia resulting from hyperuricemia resulting from overproduction or underexcretion of overproduction or underexcretion of uric acid . A high dietary intake of uric acid . A high dietary intake of purines can be an additional factor but purines can be an additional factor but doesnot in itself produce hyperuricemiadoesnot in itself produce hyperuricemia

Causes of hyperuricemia.Causes of hyperuricemia. Genetic, diet , diseases Genetic, diet , diseases

( Myeloproliferatiive disease, on ( Myeloproliferatiive disease, on chemotherapy,),& drugs decrease renal chemotherapy,),& drugs decrease renal elimination of uric acid i.e aspirin in low elimination of uric acid i.e aspirin in low doses.doses.

Pathology.Pathology. Prolonged hyperuricemia causes the Prolonged hyperuricemia causes the

formation of small crystal aggregates , formation of small crystal aggregates , these crystals accumulates in the these crystals accumulates in the synovium & at various external sites synovium & at various external sites such as the ear cartilage & olecranon such as the ear cartilage & olecranon sites , & may eventually expand & sites , & may eventually expand & become visible tophi.become visible tophi.

Crystal deposition may proceed over Crystal deposition may proceed over years without symptoms. Disturbance in years without symptoms. Disturbance in homoeostasis lead to shedding of homoeostasis lead to shedding of crystals in to the joint cavity from crystals in to the joint cavity from synovial microtophi , if enough free synovial microtophi , if enough free intraarticular crystals are present they intraarticular crystals are present they will produce an acute inflammatory will produce an acute inflammatory response.response.

Clinical features.Clinical features.

The peak age of onset is about 45 y.in The peak age of onset is about 45 y.in men while in predisposed women it men while in predisposed women it occurs some years after menopause.occurs some years after menopause.

Gout classically occurs in obese Gout classically occurs in obese middle or young men who drinks middle or young men who drinks more alcohol than average.more alcohol than average.

The patient wakes early hours in the The patient wakes early hours in the morning with sever agonizing pain morning with sever agonizing pain usually in theusually in the

metatarsophalangeal joint of the big metatarsophalangeal joint of the big toe ( the joint is usually red, swollen, toe ( the joint is usually red, swollen, warm & tender).The overlying skin is warm & tender).The overlying skin is shinny with periarticular edema, & shinny with periarticular edema, & there is often fever.there is often fever.

75% of the first attack is 75% of the first attack is monoarticular, and half involve monoarticular, and half involve metatarsphalageal joint of big toe. metatarsphalageal joint of big toe.

Attack lasts days or weeks befor Attack lasts days or weeks befor subsiding spontaneously . Patient subsiding spontaneously . Patient might have one attack or may have might have one attack or may have reccurences at monthly or yearly reccurences at monthly or yearly intervals.intervals.

Reccurent attacks may merge in to Reccurent attacks may merge in to each other. No synovial joint is each other. No synovial joint is immune from gout . Boney erosion & immune from gout . Boney erosion & joint destruction might occur & lead joint destruction might occur & lead to disability.to disability.

Acute gout could occur in Acute gout could occur in nonarticular sites i.e olecranon bursa nonarticular sites i.e olecranon bursa , achillis tendon & prepatellar bursa., achillis tendon & prepatellar bursa.

Tophi.Tophi. A tophus is a deposite of fine needle A tophus is a deposite of fine needle

( msu) surrounded by grnuloma & giant ( msu) surrounded by grnuloma & giant cells. They are found in the articular cells. They are found in the articular cartilage,ear cartilage, synovium, cartilage,ear cartilage, synovium, tendon sheath, bursae & other tendon sheath, bursae & other periarticular structure..Patient periarticular structure..Patient possessing tophi is said having chronic possessing tophi is said having chronic tophaceous gout either or not tophaceous gout either or not experiencing episodes of acute gouty experiencing episodes of acute gouty arthritis. Presence of tophi is an arthritis. Presence of tophi is an indication for long term treatment to indication for long term treatment to lower the serum uric acid.lower the serum uric acid.

Renal calculiRenal calculi is present in 10% of gout & renal is present in 10% of gout & renal

nephropathy might be found on autopsy.nephropathy might be found on autopsy.

Large tophus of the knee in patient Large tophus of the knee in patient with chronic uncontrolled gout.with chronic uncontrolled gout.

Three inflamed tophi over the proximal Three inflamed tophi over the proximal interphalangeal joints in a patient with chronic interphalangeal joints in a patient with chronic tophaceous gout. Several of the lesions tophaceous gout. Several of the lesions ruptured spontaneously over the next three ruptured spontaneously over the next three days, exuding a pasty material composed of days, exuding a pasty material composed of urate crystals and inflammatory cells but no urate crystals and inflammatory cells but no organisms. organisms.

Plain radiograph of the hand demonstrating Plain radiograph of the hand demonstrating soft tissue calcifications adjacent to soft tissue calcifications adjacent to interphalangeal joints and at the base of interphalangeal joints and at the base of the thumb (arrows). These findings the thumb (arrows). These findings represent calcification within gouty tophi. represent calcification within gouty tophi.

Plain radiograph of the foot Plain radiograph of the foot demonstrating features consistent demonstrating features consistent with gout. There is soft tissue swelling with gout. There is soft tissue swelling and extensive erosions involving the and extensive erosions involving the first metatarsophalangeal joint, as well first metatarsophalangeal joint, as well as calcifications within a tophus. as calcifications within a tophus.

Dfferential diagnosis.Dfferential diagnosis. PseudogoutPseudogout Acute rheumatic feverAcute rheumatic fever RARA Pyogenic arthritis Pyogenic arthritis Cellulites Cellulites Bursitis, Bursitis, TendonitisTendonitis Thrombophelibitis.Thrombophelibitis.

Investigation .Investigation . ESR is elevated in acuter attack . ESR is elevated in acuter attack . Serum uric acid could be high but it is Serum uric acid could be high but it is

not necessary to be so , & high serum not necessary to be so , & high serum uric acid alone doesn’t justify the uric acid alone doesn’t justify the diagnosis of gout.diagnosis of gout.

In acute gout the presence of long In acute gout the presence of long needle shaped negatively birefringent needle shaped negatively birefringent nmonosodium crystals in the synovial nmonosodium crystals in the synovial fluid is diagnostic.fluid is diagnostic.

X- ray shows punched out area , joint X- ray shows punched out area , joint destruction & might be ankylosis.]destruction & might be ankylosis.]

Management.Management. The aims are to reduce acute synovitis , The aims are to reduce acute synovitis ,

prevent further crystal formation & prevent further crystal formation & identify associated disease.identify associated disease.

In acute attack : (rest,elevation and In acute attack : (rest,elevation and ice pack application)advise intake of ice pack application)advise intake of cherry juice and diary product diet. cherry juice and diary product diet.

Alcohol intake should be stopped.Alcohol intake should be stopped.

1.1. NSAID ( often indomethacine ) NSAID ( often indomethacine )

2.2. Oral colchicine. Oral colchicine.

3.3. Intra-articular corticosteroids. Intra-articular corticosteroids.

4.4. Oral corticosteroid. Oral corticosteroid.

Azapropazone ( 600 twice a day ) is both anti-Azapropazone ( 600 twice a day ) is both anti-inflamatory & uricosuric agent.inflamatory & uricosuric agent.

When long term treatment is necessary When long term treatment is necessary xanthine oxidase inhibitor ( allopurinol ) or xanthine oxidase inhibitor ( allopurinol ) or uricosuric drugs are started after several uricosuric drugs are started after several weeks from controlling the acute attack.weeks from controlling the acute attack.

Indications for long term therapy Indications for long term therapy include :include :

1.1. Reccurent attacks Reccurent attacks

2.2. Tophaceous gout Tophaceous gout

3.3. Renal calculi. Renal calculi.

Uricosuric drugsUricosuric drugs inhibit renal tubular inhibit renal tubular reabsorption of uric acid, ( Probenecid, reabsorption of uric acid, ( Probenecid, Sulphinpyrazone & Salicylate in high doses). Sulphinpyrazone & Salicylate in high doses). They are ineffective in renal failure.They are ineffective in renal failure.

Allopurnol :Allopurnol : Is the most popular hypourecemic agent , it Is the most popular hypourecemic agent , it

is a xanthin oxidase inhibitor , reduces the is a xanthin oxidase inhibitor , reduces the oxidation of hypoxanthin to xanthin & oxidation of hypoxanthin to xanthin & xanthin to uric acid.xanthin to uric acid.

Indications are :Indications are : Extensive topaceous gout ,Extensive topaceous gout , Renal impairment & ston ?Renal impairment & ston ? Hyperuricemia due to antimitotic drug Hyperuricemia due to antimitotic drug

therapy therapy Intolerance or failure of uricosuric therapy.Intolerance or failure of uricosuric therapy.

Side effects include dyspepsia& skin rash. Side effects include dyspepsia& skin rash.

OxypurinolOxypurinol is also could be used ( having is also could be used ( having longer half life 28 hours ). longer half life 28 hours ).

Febuxostate.is a new xanthin oxidase Febuxostate.is a new xanthin oxidase inhibitor ,used for the treatment of inhibitor ,used for the treatment of hyperuricemia ,dose 80-120 mg/day , its hyperuricemia ,dose 80-120 mg/day , its efficacy demonstrated in pt in mild to efficacy demonstrated in pt in mild to moderate renal impairment and gout.moderate renal impairment and gout.

It can cause abnrmality in LFT(Liver It can cause abnrmality in LFT(Liver function tests) , so you should monitor function tests) , so you should monitor LFT ( shouldn’t be used with LFT ( shouldn’t be used with azathioprine and theophyline)azathioprine and theophyline)

Pegloticase: uricase is an enzyme that Pegloticase: uricase is an enzyme that convert poorly soluble uric acid to more convert poorly soluble uric acid to more soluble allantoin,which is excreted in the soluble allantoin,which is excreted in the urine . Uricase present in most mammels urine . Uricase present in most mammels , the human lacks uricase ., the human lacks uricase .

Pegloticase : is porcine uricase which is Pegloticase : is porcine uricase which is used for treatment of gout in pt who fail used for treatment of gout in pt who fail conventional therapy ,given IV 8 mg conventional therapy ,given IV 8 mg every 2 weeks and leads to decrease every 2 weeks and leads to decrease tophi.tophi.

The drug is :The drug is : 1. Peglated porcine uricase.1. Peglated porcine uricase. 2. useful in both pt with increase synthesis 2. useful in both pt with increase synthesis

and decreased clearance of uric acid.and decreased clearance of uric acid. 3. inceases solubility of uric acid.3. inceases solubility of uric acid. 4.pt should be watched for allergic 4.pt should be watched for allergic

reaction reaction 5.should be used with caution in pt with 5.should be used with caution in pt with

known cardiac disease.known cardiac disease.

Interlukin-1 antagonist could be used Interlukin-1 antagonist could be used in acute goutin acute gout

Etanercept could be used.Etanercept could be used.

Connective Tissue DiseasesConnective Tissue Diseases The term C.T diseases is used for three The term C.T diseases is used for three

conditions:conditions: 1.1. Systemic lupus erythematosus Systemic lupus erythematosus 2.2. Systemic sclerosis Systemic sclerosis 3.3. polymyositis & dermatomyositis. polymyositis & dermatomyositis.

They some sharing features:They some sharing features: Arthritis or arthralgia, multisystem Arthritis or arthralgia, multisystem

involvement, vasculitis & involvement, vasculitis & immunological abnormalities such as immunological abnormalities such as ciculating autoantibodies & immune ciculating autoantibodies & immune complex deposition.complex deposition.

Features of all three diseases appear in Features of all three diseases appear in mixed connective tissue disease.mixed connective tissue disease.

Systemic Lupus ErythematosusSystemic Lupus Erythematosus I s a disease of unknown cause, I s a disease of unknown cause,

characterized by the presence of , in serum, characterized by the presence of , in serum, antibodies against nuclear components . It antibodies against nuclear components . It is a multisystem disease with :is a multisystem disease with :

Arthralgia & rash the commonest clinical Arthralgia & rash the commonest clinical features while renal & cerebral involvement features while renal & cerebral involvement are the most serious problems.are the most serious problems.

The clinical course of the disease is The clinical course of the disease is characterized by periods of remission & characterized by periods of remission & acute or chronic relaps, mltisystem acute or chronic relaps, mltisystem pathologiese & presence of immune pathologiese & presence of immune abnormalitiese especially antibodies to a abnormalitiese especially antibodies to a number of nuclear & cellular antigens.number of nuclear & cellular antigens.

EpidemiologyEpidemiology : : It s a world wide disesease , affects It s a world wide disesease , affects

about 0.1% of the population, it is 20 about 0.1% of the population, it is 20 times less common than RA, it is times less common than RA, it is much more commoner in black much more commoner in black womens in USA. Fmales/ males is womens in USA. Fmales/ males is about 9 / 1. The peak age of onset is about 9 / 1. The peak age of onset is between 15 & 40 years. In children between 15 & 40 years. In children female to male ratio is 5/1. female to male ratio is 5/1.

Pathogensis & aetiology. Pathogensis & aetiology. The cells & tissue are damaged by pathogenic The cells & tissue are damaged by pathogenic

autantibodies & immune complexes , the classical autantibodies & immune complexes , the classical example is that of diffuse prolefrative example is that of diffuse prolefrative glomerulonephritis …. Immun complexes glomerulonephritis …. Immun complexes deposits in glomerular basement membrane. This deposits in glomerular basement membrane. This will lead to attraction & infiltration of leucocyte will lead to attraction & infiltration of leucocyte which then phagocyte immune complexes & which then phagocyte immune complexes & cause release of mediators ( such as activators of cause release of mediators ( such as activators of clotting system ) . With continuing immune clotting system ) . With continuing immune complex deposition , chronic inflammation may complex deposition , chronic inflammation may ensue( come) , ultimately leading to fibrinoid ensue( come) , ultimately leading to fibrinoid necrosis & scarring ( crescent) & loss of renal necrosis & scarring ( crescent) & loss of renal function.function.

The cause of SLE is unknown but is probably The cause of SLE is unknown but is probably multifactorial including a variable genetic multifactorial including a variable genetic predisposition & environmental factors that predisposition & environmental factors that trigger the disease.trigger the disease.

Predisposing factors include :Predisposing factors include : Heredity :Heredity : The identical twine of SLE The identical twine of SLE

patient has a 30% chance to develop the patient has a 30% chance to develop the disease, first degree relatives have 5% disease, first degree relatives have 5% chance, while certain races are especially chance, while certain races are especially prone to the disease i.e black Americans prone to the disease i.e black Americans & Africans.& Africans.

Sex hormone status :Sex hormone status : Females are much Females are much more affected than males.more affected than males.

Complement deficiencies of all Complement deficiencies of all types(C3,C4)types(C3,C4)..

Environmental triggers(sun light{U.V}).Environmental triggers(sun light{U.V}). Drugs:Drugs: such as hydralazine, & ultra such as hydralazine, & ultra

violate light.violate light. Infection : a virus infection is a popular Infection : a virus infection is a popular

theory for the cause of lupus.theory for the cause of lupus.

Immnological mechanism of the disease.Immnological mechanism of the disease.1.1. Polyclonal B-cell activation ; increased number Polyclonal B-cell activation ; increased number

of B-cells lead to hyperglobulinaemia.of B-cells lead to hyperglobulinaemia.

2.2. Antinuclear antibodies & other autoantibodies Antinuclear antibodies & other autoantibodies are produced.are produced.

3.3. Impaired T-cell regulation of the immune Impaired T-cell regulation of the immune response.response.

4.4. Failure to remove immune complxes from the Failure to remove immune complxes from the circulation, circulating immune complexes circulation, circulating immune complexes cause arthralgia, deposition of immune cause arthralgia, deposition of immune complexes in tissue cause vasculitis & other complexes in tissue cause vasculitis & other features of the disease including features of the disease including glomerulonephritis .glomerulonephritis .

SLE may coexist with other autoimmune SLE may coexist with other autoimmune diseases such as haemlytic anemia , diseases such as haemlytic anemia , thyroiditis, & idiopathic thrombocytopenic thyroiditis, & idiopathic thrombocytopenic purpura.purpura.

Pathology.Pathology. SLE is characterized by wide spread SLE is characterized by wide spread

vasculitis affecting capillaries , vasculitis affecting capillaries , arteriols ,& venules. Vascular arteriols ,& venules. Vascular changes include perivascular changes include perivascular mononuclear cells , luman mononuclear cells , luman obliteration , enlarged endothelial obliteration , enlarged endothelial cells & thrombi .cells & thrombi .

Pleura & pericardium are Pleura & pericardium are infiltrated by mononuclear cells . infiltrated by mononuclear cells . Coronary arteries often demonstrate Coronary arteries often demonstrate premature onset athresclerosis.premature onset athresclerosis.

Biopsy from malar erythema may Biopsy from malar erythema may reveal some minor basal layer reveal some minor basal layer abnormalities as well as immune abnormalities as well as immune complex deposits at the dermal – complex deposits at the dermal – epidermal junction.epidermal junction.

Fibrinoid ( an eosinophilic amorphous Fibrinoid ( an eosinophilic amorphous material ) is found along blood material ) is found along blood vessels & tissue fibers . The synovium vessels & tissue fibers . The synovium of joint may be oedematous & may of joint may be oedematous & may contain fibrinoid deposits.contain fibrinoid deposits.

Haemotoxyline bodies ( which are Haemotoxyline bodies ( which are rounded blue homogenous haemtoxyline rounded blue homogenous haemtoxyline stained deposits ) are seen in stained deposits ) are seen in inflammatory infiltrates & thought to inflammatory infiltrates & thought to result from the interaction of antinuclear result from the interaction of antinuclear antibodies and cell nuclei.antibodies and cell nuclei.

The onion skin lesions are found in the The onion skin lesions are found in the spleen & consist of concentric layers of spleen & consist of concentric layers of fibrosis surrounding the vessels ( arteries fibrosis surrounding the vessels ( arteries ).).

Necrotising vasculitis occurs in the small Necrotising vasculitis occurs in the small & medium sized arteries in the skin with & medium sized arteries in the skin with inflammation & degeneration in the inflammation & degeneration in the dermal—epidermal junction may also dermal—epidermal junction may also cause skin lesions. cause skin lesions.

Clinical FeaturesClinical Features..

Are variable. Most of the features are Are variable. Most of the features are due to VASCULITIS.due to VASCULITIS.

Mild cases may presents with only Mild cases may presents with only Arthralgia whilst in sever cases Arthralgia whilst in sever cases there might be Multisystem there might be Multisystem involvement.involvement.

General features :General features : fever is common in fever is common in exacerbations ( 80%), malaise, exacerbations ( 80%), malaise, tiredness& fatigue.tiredness& fatigue.

Joint involvement:Joint involvement: is a commonest is a commonest manifestation (90%) & is similar to manifestation (90%) & is similar to that of RA in distribution but erosion that of RA in distribution but erosion & deformities are rare ( could be & deformities are rare ( could be arthralgia).arthralgia).

Tenosynovitis could be a feature.Tenosynovitis could be a feature.

Subcutaneous nodules may occur.Subcutaneous nodules may occur.

Patient with long-standing lupus has developed Patient with long-standing lupus has developed subluxation at the MCP joints and swan neck subluxation at the MCP joints and swan neck deformities of her fingers. These deformities deformities of her fingers. These deformities are reducible (probably being due to lax are reducible (probably being due to lax tendons) and x-rays reveal no erosions or tendons) and x-rays reveal no erosions or cysts, both of which differentiate these cysts, both of which differentiate these findings from those in rheumatoid arthritis.findings from those in rheumatoid arthritis.

Skin features :Skin features : (80%). (80%).

Malar ( Butterfly) rash.Malar ( Butterfly) rash.

Butterfly Rash ( Malar Rash) .Butterfly Rash ( Malar Rash) .

Discoid rashDiscoid rash

Photosensivity. Photosensivity.

Raynaudes phenomenon.Raynaudes phenomenon.

Vsculitic lesionsVsculitic lesions on the finger tips & on the finger tips & around nails folds , purpura & around nails folds , purpura & urtecaria occur.urtecaria occur.

Livedo reticularis, palmar & plantar Livedo reticularis, palmar & plantar erythema.erythema.

Pigementation & alopecia which may Pigementation & alopecia which may be diffuse or patchy.be diffuse or patchy.

Band test in the skin ( seen by Band test in the skin ( seen by immunofluorescent, is deposition of immunofluorescent, is deposition of immunoglobuline & complements in immunoglobuline & complements in dermo—epidermal junction).dermo—epidermal junction).

In mucosmembrane:In mucosmembrane:( mouth ulcers, ( mouth ulcers, vaginal ulcers & nasal septal erosions.vaginal ulcers & nasal septal erosions.

Lungs ;Lungs ; Pleursy & exudates effusion.& Pleursy & exudates effusion.& pneumonitis.(70%).pneumonitis.(70%).

The heart :The heart : (40%) Pericarditis , small (40%) Pericarditis , small effusion, mild myocarditis, aortic valve effusion, mild myocarditis, aortic valve lersion & cardiomyopathy.lersion & cardiomyopathy.

The kidneys :The kidneys : means bad prognosis ( 30 means bad prognosis ( 30—40 %) protienuria less than 1 gm / 24 —40 %) protienuria less than 1 gm / 24 hours is common, various types of hours is common, various types of glomerulonephritis could occur. glomerulonephritis could occur. Hypertension duo to renal involvement Hypertension duo to renal involvement could occur.( Should do renal biopsy for could occur.( Should do renal biopsy for SLE patients). SLE patients).

The nervous system :The nervous system : (60%) (60%) depression, Epilepsy , cerebellar depression, Epilepsy , cerebellar ataxia, CVA, or peripheral ataxia, CVA, or peripheral neuropathymay be seen . These neuropathymay be seen . These lesions may be due to vasculitis or lesions may be due to vasculitis or immune complex deposition , CNS immune complex deposition , CNS involvement means bad prognosis.involvement means bad prognosis.

Eyes ; Eyes ; could be involved due to could be involved due to retinal vasculitis.retinal vasculitis.

Investigations Investigations Blood : Anemia usually normochromic Blood : Anemia usually normochromic

normocytic., normocytic., Neutropenia & Neutropenia & thrombocytopenia. thrombocytopenia. ESR is raised ESR is raised proportional to disease activity.proportional to disease activity.

While CRP is usually normalWhile CRP is usually normal..

Serum antinuclear antibodies ( ANAs) Serum antinuclear antibodies ( ANAs) are positive in almost all cases .are positive in almost all cases .

Anti-double strand antibodies ( anti Anti-double strand antibodies ( anti DNA) were found to more specific but DNA) were found to more specific but present in about 50—70% of cases present in about 50—70% of cases particularly those with sever systemic particularly those with sever systemic involvement i.e. renal disease.involvement i.e. renal disease.

Sreum rheumatoid factor is seen in Sreum rheumatoid factor is seen in half of cases.half of cases.

Serum complement levels are reduced Serum complement levels are reduced during active phase(C3,C4). during active phase(C3,C4). Immunoglobulins are usually raised Immunoglobulins are usually raised ( IgM & IgG)( IgM & IgG)

False positive tests for syphilis are False positive tests for syphilis are found in 1/3 of SLE patients.found in 1/3 of SLE patients.

Anti-sm is more specific but present in Anti-sm is more specific but present in 20% of patients. 20% of patients.

Anti Ro ant Anti La could be present.Anti Ro ant Anti La could be present.

Management . Management . Aims :Aims :

1.1. Relieving symptoms. Relieving symptoms.

2.2. Suppression of inflammation. Suppression of inflammation.

3.3. Preventing future pathology. Preventing future pathology.

The risk to benefit ratio of The risk to benefit ratio of potentially toxic drugs must be potentially toxic drugs must be tailored to the patient tailored to the patient

Should avoid sulfonamide , Should avoid sulfonamide , penicillin & high oestrogen pills penicillin & high oestrogen pills which exacerbate the disease.which exacerbate the disease.

Treatment.Treatment.1.1. Avoid over exposure to sunlight & Avoid over exposure to sunlight &

ultraviolate light & use sunblock .ultraviolate light & use sunblock .

2.2. Take low fat diet & add fish oil Take low fat diet & add fish oil derivatives(Omega3) . Give Calcium derivatives(Omega3) . Give Calcium & vit. D for healthy bones.& vit. D for healthy bones.

Drug therapy:Drug therapy:

This depends on the organ involvement This depends on the organ involvement but steroid is the mainstay in of the but steroid is the mainstay in of the treatment.treatment.

In mild disease ( artharlgia & fever) In mild disease ( artharlgia & fever) ----- NSAIDs.----- NSAIDs.

Artharlgia, myalgia& lethargy Artharlgia, myalgia& lethargy ------------Antimalarials +-- NSAIDs.------------Antimalarials +-- NSAIDs.

Skin without joint involvement Skin without joint involvement -----------Antimalarials + sunblock.-----------Antimalarials + sunblock.

Skin & joints-------------------- Skin & joints-------------------- NSAIDs + Antimalarials.NSAIDs + Antimalarials.

Serositis/ arthritis/ myositis--------Serositis/ arthritis/ myositis--------Steroid ( if mild serositis --- NSAIDs Steroid ( if mild serositis --- NSAIDs may be enough ).may be enough ).

Renal ------------ Steroid + Renal ------------ Steroid + azathioprine. (might need monthly azathioprine. (might need monthly i.v. steroid &Cyclophosphamide for i.v. steroid &Cyclophosphamide for six months then 2—3 monthly for 2 six months then 2—3 monthly for 2 years.(Mycophnolate mofetile could years.(Mycophnolate mofetile could be used when in renal involvement). be used when in renal involvement).

CNS ------------- Steroid CNS ------------- Steroid ( anticonvulsant).( anticonvulsant).

Pregnancy in SLE.Pregnancy in SLE.1.1. No major contraindication for No major contraindication for

pregnancy.pregnancy.2.2. Fertility is normal except when there is Fertility is normal except when there is

sever renal involvement.sever renal involvement.3.3. There is an increased rate of a fetal There is an increased rate of a fetal

loss, recurrent abortion can occur befor loss, recurrent abortion can occur befor or during the clinical course of SLE. or during the clinical course of SLE.

4.4. Exacerbation may occur at any time Exacerbation may occur at any time during the course of pregnancy. ( but during the course of pregnancy. ( but 30% go to remission in pregnancy).30% go to remission in pregnancy).

5.5. A postpartum deterioration is common . A postpartum deterioration is common .6.6. A patient with active renal disease have A patient with active renal disease have

much worse prognosis.much worse prognosis.

Course & prognosis.Course & prognosis.1.1. An episodic course is characteristic, An episodic course is characteristic,

with exacerbation & complete remission.with exacerbation & complete remission.

2.2. Chronic course is occasionally seen. Chronic course is occasionally seen.

3.3. Unless there is sever renal or CNS Unless there is sever renal or CNS involvement the outlook is now much involvement the outlook is now much improved. 5—10 y. survival is about 95%.improved. 5—10 y. survival is about 95%.

4.4. In most of the cases the pattern of the In most of the cases the pattern of the disease becomes established in the first disease becomes established in the first few years , if serious problems haven’t few years , if serious problems haven’t developed in this time they are unlikely developed in this time they are unlikely to do so. to do so.

5.5. The arthritis is usually not erosive or The arthritis is usually not erosive or destractive. destractive.

Revised criteria of the ARA for the Revised criteria of the ARA for the classification of SLE.classification of SLE.

1.1. Malar rash. Malar rash.

2.2. Discoid rash. Discoid rash.

3.3. Photosensitivity. Photosensitivity.

4.4. Oral ulcers usually painless. Oral ulcers usually painless.

5.5. Arthritis nonerosive , not Arthritis nonerosive , not deforming.deforming.

6.6. Serositis. Pleuritis or Serositis. Pleuritis or pericarditis.pericarditis.

7.7. Renal disorder persistent proteinuria Renal disorder persistent proteinuria of more than 0.5 gm/24 h or cellular cast.of more than 0.5 gm/24 h or cellular cast.

8.8. Neurological disorder seizure or Neurological disorder seizure or psychosis.psychosis.

9.9. Haematological disorders Haemolytic Haematological disorders Haemolytic anemia, leucopenia, lymphopenia & anemia, leucopenia, lymphopenia & thrombocytopenia.thrombocytopenia.

10.10. Immunological disorders Immunological disorders Raised antinative DNA antibody Raised antinative DNA antibody binding. binding. Anti—Sm Anti—Sm antibody,AntiRo,Anti La. antibody,AntiRo,Anti La. Positive antiphispholipid antibodies.Positive antiphispholipid antibodies.

11.11. Antinuclear antibody in raised titre. Antinuclear antibody in raised titre.

Systemic SclerosisSystemic Sclerosis

Is a chronic multisystem disorder of Is a chronic multisystem disorder of unknown aetiology characterised clinically unknown aetiology characterised clinically by thickening of skin caused by by thickening of skin caused by accumulation of CT and structural and accumulation of CT and structural and functional abnormalities of visceral organs functional abnormalities of visceral organs including GI tract, Lungs, heart, & kidneys.including GI tract, Lungs, heart, & kidneys.

Classification: Classification:

Localised types. (Morphea, Linear, En Localised types. (Morphea, Linear, En coupe de sabre).coupe de sabre).

Systemic Sclerosis.( Limited cutaneous Systemic Sclerosis.( Limited cutaneous disease,& diffuse cutaneous disease).disease,& diffuse cutaneous disease).