SPONDYLOARTHROPATHY(SPONDYLOARTHRITIS)

DEFINITION

Group of multisystem inflammatory disorders affecting various joints, Psoriatic arthritis Enteropathic/Inflammatory bowel disease –

associated spondyloparthropathy Ankylosing spondylitis Reactive arthritis (including Reiter’s

syndrome) Juvenile-onset spondyloarthritis Undifferentiated spondyloarthropathy

COMMON FEATURES Axial spondyloarthritis

low back pain (sacroilitis, spondylitis) Peripheral arthritis

Pain and swelling in legs>arms Asymmetric, oligoarticular

Seronegative rheumatoid factor Enthesitis (inflmmation at osseus insertion sites

of tendons and ligaments) Extra-articular features

Mucocutaneous, uveitis Male predominance <40, familial clustering HLA-B27 association(>90%), absence autoAb

I. AXIAL SPONDYLOARTHRITIS

2. PERIPHERAL ARTHRITIS

3. ENTHESITIS

Dactylitis (‘sausage-like' digit) Tendonitis Fasciitis Spondylitis

Enthesitis is primary lesion in spondyloathropathy; synovitis is main lesion in RA

4. EXTRA ARTICULAR FEATURE

Uveitis

ANKYLOSING SPONDYLITIS

I. ANKYLOSING SPONDYLITIS (AS) Also known as Marie-Strumpell disease or Bechterew’s

disease Chronic inflammatory disorder of axial skeleton affecting the

SI joint and the spine Hallmark : bilateral sacroilitis Most common spondyloarthropathy •Onset at 40 years or younger.

•Insidious in onset and present for at least 3 months.•Morning stiffness is present and improves with exercise.•Pain occurs at night.•No improvement with rest.

ETIOPATHOGENESIS

SYMPTOMS

PE: SPINAL MOBILITY TEST

Modified Schober test

PE: SPINAL MOBILITY TEST

occiput-to-wall distance (tragus to wall test) Stand with heels against

wall, ask patient to touch the back of his head to the wall

Normal distance <10cm, no gap in between

If >10cm or +ve gap, indicates thoracic or cervical disease or both

PE: MEASUREMENT OF CHEST EXPANSION

useful in determining the degree of costovertebral joint involvement.

Ask the patient to put shoulders above the head and perform full expiration and full inspiration. At 4th interspace in men/below the breast in women, measure the expansion value.

N >5cm, abN <5cm / 2.5cm less than N value

PE: LUMBAR SPINE MOBILITY

Lumbar Spine Side Flexion test Heels against wall, hip width

apart, back against the wall, fingers extending down on the side. Measure distance from fingers to floor. Then ask patient to flex to the side with back against the wall, re-measure and note the difference.

PE: HIP MOBILITY

Hip Abduction test Knees straight, toes

pointing up, spread out the legs as far as they can and measure btw both malleoli

Normal = 120cm, ,120cm indicates restriction

PE: CERVICAL MOBILITY

Cervical Spine Rotation Test Inclinometer is placed on forehead (to

measure degree of inclination) and ask the patient to turn the head to left and right as far as they can without moving the upper body.

Normal is approximately 90degree rotation.

LABORATORY FINDINGS

Mild anemia (15%) ↑ ESR,CRP - often without a definite correlation with disease

activity (75%) ↑ALP ↑serum IgA -ve rheumatoid factor, anti CCP, ANA +ve HLA-B27

Screening for HLA-B27 in all patients with back pain is not useful because more than 95% of these patients do not have a spondyloarthropathy. In patients with inflammatory back pain or sacroiliitis on plain radiographs, the addition of a positive HLA-B27 result increases the likelihood of a diagnosis of an axial spondyloarthropathy. The diagnostic specificity of the HLA-B27 test increases with an associated decrease of prevalence in the target population

XRAY – (I) SACROILITISSacroilitis grading can be achieved using plain films according to the New York criteria 4. grade 0 - normal grade I -  some blurring of the joint margins - suspicious grade II - minimal sclerosis with some erosion grade III -

definite sclerosis on both sides of joint 5 severe erosions with widening of joint space (pseudowidening) with or without ankylosis

grade IV - complete ankylosis

X-RAY – (II) SPONDYLITIS

Radiographic changes in the spine occur initially in the lumbar spine and gradually ascend the spine, generally in a continuous fashion. squaring of vertebral

bodies progression to erosions and

sclerosis of the anterior corners of the vertebral bodies (“shiny corners”)

ossification of the anulus fibrosus (syndesmophyte formation)

over many years, spinal fusion (“bamboo spine)

(I) SQUARING OF VERTERBRAL BODIESIn lumbar spine, progression of disease leads to Straightening (caused by loss of lordosis) Reactive sclerosis (caused by osteitis of anterior corners of the

vertebral bodies ) With subsequent erosion, leading to “squaring” of vertebral

bodies (loss of normal concavity of anterior border)

(II) SHINY CORNERS (ROMANUS LESIONS) Inflammation at the site of insertion of the annulus fibrosus

resulted in osteitis (progression to erosions and sclerosis of the anterior corners of the vertebral bodies)

(III)SYNDESMOPHYTE FORMATION Progressive ossification leads to formation of marginal

syndesmophyte, visible as bony bridges connecting successive vertebral bodies anteriorly and laterally

(III) SYNDESMOPHYTE FORMATION

(IV) BAMBOO SPINE Complete fusion results in a complete rigidity of

the spine, a condition known as bamboo spine.

DYNAMIC MRI WITH FAT SATURATION

> Short-tau inversion recovery (STIR) sequence > T1-weighted images with contrast enhancement Can detect abN at earlier stages Highly sensitive and specific for identifying early intra-articular inflammation,

cartilage changes and u/l bone edema in sacroilitis The Berlin MRI spine score14 15 is a modification of the ankylosing spondylitis

spine MRI-activity (ASspiMRI-a) scoring system for active inflammatory lesions of the spine

MODIFIED NEW YORK CRITERIA 1984

ASSESSMENT OF SPONDYLOARTHRITIS INTERNATIONAL SOCIETY (ASAS)

BATH ANKYLOSING SPONDYLITIS DISEASE ACTIVITY INDEX (BASDAI)

Scores ≥4 : suboptimal control of disease (good candidates for either a change in their medical therapy or for enrollment in clinical trials evaluating new drug therapies directed at Ankylosing Spondylitis.

ANKYLOSING SPONDYLITIS DISEASE ACTIVITY SCORE (ASDAS)

TREATMENT

EXERCISE PROGRAM

NSAID

Effective for reducing symptoms Have disease-controlling property

ANTI-TNF

Anti-TNFα – if pt refratory to physical therapy and other interventions infliximab (Remicade), IV infusion every 2-6-

8 weeks at a dose of 5 mg/kg; etanercept (Enbrel), SC injection of 50 mg

once weekly adalimumab (Humira), SC injection of 40 mg

biweekly golimumab (Simponi), SC injection of 50 mg

once a month

SIDE EFFECTS OF ANTI-TNF

Serious infection (Disseminated TB) Hematologic disorder (pancytopenia) Demyelinating disorders Exacerbation of congestive heart failure SLE-related autoAb and C/M Hypersensitivity infusion or injection site

reactions Severe liver diseaseUse is restricted for patients with definitive diagnosis and active dx (BASDAI ≥4) that is inadequately responsive to 2 different NSAID

CONVENTIONAL DMARD

Conventional DMARD which plays a dominant role in RA tx has no proven efficary for axial AS, but a limited efficacy for peripheral manifestation

- sulfasalazine: 2g/d for 4mths (for peripheral manifestation)

- methotrexate: trials reveal no superiority over placebo

PSORIATIC ARTHRITIS

PSORIATIC ARTHRITIS

Inflammatory arthritis that occurs in individuals with psoriasis

Affects male and female equally Peak age at diagnosis: 20-40 Dermatological features of

psoriasis precede arthritis in 90% 

There is a strong association with nail involvement, particularly for DIP joint arthritis.

I. SKIN LESIONS

II. NAIL LESIONS Pitting nail Horizontal ridging Onycholysis Yellowish discoloration of nail margins Dystrophic hyperkeratosis

III. ARTHRITIS

The patterns are not fixed and the patterns that persist chronically often differs from that of initial presentation.

Recent use of simpler scheme: 1) oligoarthritis 2) polyarthritis 3) axial athritis

WRIGHT AND MOLL CLASSIFICATION ASYMMETRIC OLIGOARTHRITIS

(70-80%) Generally mild and most

common Involves a knee or a large joint

with few small joints SYMMETRIC POLYARTHRITIS (5-

20%) Ddx with RA, is typically milder,

less tender and with less cases of deformity

DIP PREDOMINANT (10%) Ddx with OA, this type usually

affects distal interphalangeal joint

SPONDYLITIS (5-20%) Ddx with AS, more neck

involvement, less thoracolumbar, nail changes

ARTHRITIS MUTILANS (<5%) Widespread shortening of digits

“telescoping”, with ankylosis and contractures in other digits

SYMMETRIC POLYARTHRITS

DIP PREDOMINANT

ARTHRITIS MUTILANS

XRAY Peripheral joint features:

Large eccentric erosions Tuft resorption

Destructive changes “Pencil-in-cup” deformities in DIP

(arthritis mutilans) Proliferative changes

Fluffy periostitis,“wkiskering”(periosteal bone formation)

Ivory phalanx (endosteal bone formation)

Bony ankylosis and joint fusion Spinal changes (ddx AS)

“chunky syndesmophyte” (paravertebral ossification)

marginal bone erosions with adjacent irregular bone proliferation

PENCIL-IN-CUP DEFORMITY

The deformity occurs in arthritis mutilans due to marked osteolysis “pencilling”(distal head of a bone

becomes pointed) adjacent joint surface becomes cup-

like

FLUFFY PERIOSTITIS, “WHISKERING”

IVORY PHALANX Increased density throughout the osseous structures

due to sclerosis of an entire phalanx, typically the great toe, is likely the result of bony proliferation as an exaggerated healing response to injured bone

• Narrowed joint space (*)• Subchondral cyst (Arrowhead)• Marginal erosions (thin arrow)• New periosteal bone formation (thick arrow)• Endosteal bone formation (ivory

phalanx)

SPINAL CHANGES Sacroilitis

Asymmetric Spondylitis

> cervical spine, < lumbar spine “chunky syndesmophyte” Comma-shaped paravertebral

ossification

DDX

General imaging differential considerations include rheumatoid arthritis

there is a MCP joint predominance in rheumatoid arthritis(RA) vs interphalangeal predominant distribution in PsA

bone proliferation not a feature in RA Osteoporosis not a feature in PsA

erosive osteoarthritis “gull wing” central erosions are present in erosive OA vs

“mouse ears” peripheral bare area erosions in PsA Heberden nodes are usually not inflammatory

reactive arthritis (Reiter syndrome) tends to involve feet > hands

Gout Often involves other sites and is accompanied by tophi

1. DDX WITH RA

2. DDX WITH OA

A and B. Mouse Ears.(Pso) Note the combination of

erosions and fluffy periostitis produces the mouse ears appearance in psoriasis.

C and D. Gull Wings.(OA) Observe that the biconcave

articular contour produces the gull wings appearance of erosive osteoarthritis.

3. DDX AS (ANKYLOSING SPONDYLITIS)

ASSESMENT

TREATMENT

Mild joint dx- NSAIDS- Intra-articular

steroidsModerate-severe- Systemic oral

DMARDs- Biologics

REACTIVE ARTHRITIS

II. REACTIVE ARTHRITIS (RE-A)

Can’t SEE, can’t PEE, can’t climb TREES Reiter’s syndrome:

Conjunctivitis Urethritis Postinfectious arthritis

Predilection for the lower extremity Soft Tissue swelling Osteoporosis Uniform loss of joint space Marginal erosions Periostitis >> Deformity

ENTEROPATHIC ARTHRITIS

III. ENTEROPATHIC ARTHRITIS (EA)

Bilateral and symmetric bone erosions, bone sclerosis, and widening of sacroiliac joints

Hyperparathyroidism

Bilateral and symmetric bone sclerosis and irregularity of sacroiliac joints (arrow). Note marked widening of sacroiliac joints and renal dialysis catheter.

UNDIFFERENTIATED AND JUVENILE ONSET SPA

IV. JUVENILE ONSET SPONDYLOARTHROPATHY

Composite images of ankylosing tarsitis in a second patient, a 16-year-old boy with 9 years' disease duration and complete ankylosis of the tarsal bones and grade 2 bilateral sacroiliitis. (A) Flat foot and swelling around the

ankle. (B) Complete ankylosis of the tarsal bones

and enthesophytes at the plantar fascia attachment.

(C) T2-weighted fat-saturation MRI showing edema (white spots) in several tarsal bones, joint spaces, and surrounding fat.

(D) Posterior and (E) coronal views showing the same

changes in the bones and joint spaces seen in (C), but also demonstrating distorted architecture of the tarsus and, notably, edema around the tendons.

UNDIFFERENTIATED SPNDYLOARTHROPATHY

The term “undifferentiated spondyloarthropathy” is used to describe manifestations of a spondyloarthropathy in patients who do not meet criteria for any of the well-defined spondyloarthropathies.

Over time, a small proportion of these patients develop a well-defined spondyloarthropathy.

However, most patients have less specific symptoms, including inflammatory back pain, unilateral or alternating buttock pain, enthesitis, dactylitis and, occasionally, extra-articular manifestations.

Patients with undifferentiated spondyloarthropathy generally have a good prognosis and often respond well to NSAID therapy.

Treatment of patients with more severe disease is similar to that for ankylosing spondylitis.

SAPHO SYNDROME

SAPHO SYNDROME

Syndrome of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) is characterized by a variety of skin and musculoskeletal manifestations.

ESR is usually elevated, sometimes dramatically

Bacteria culture: >Proprionibacterium acnes Coexistence of IBD in 8% patients B27 only +ve in minority patients Diagnostic test: bone scan or CT scan High does NSAID relieves bone pain.

DERMATOLOGICAL MANIFESTATION

Palmoplantar pustulosis Acne conglobata Acene fulminans Hidradenitis suppurativa

MUSCULOSKELETAL MANIFESTATION

Sternoclavicular and spinal hyperostosis

Chronic recurrent foci of sterile osteomyelitis

Axial or peripheral arthritis

WHIPPLE’S DISEASE

WHIPPLE’S DISEASE

Clinical examination of rheumatic disease patient http://www.arthritis.co.za/the%20clinical%20examination%20technique.htmlAS objective tests http://www.youtube.com/watch?v=c-IeFZkPEoE&list=PLB376ABEAA66A984DXrays collection of SpA,RA, OAhttp://pubs.rsna.org/doi/full/10.1148/radiol.2482062110Can’t miss signs in rheumatologic dxhttp://reference.medscape.com/features/slideshow/diseases-plain-radiography#20