dr. m jokardoctorjokar.com/attachment/doc/rheumatoidarthritis.pdf · ra -definition chronic...

Dr. M Jokar

By: Dr. Mohammad Hasan Jokar

RA - Definition

chronic systemic inflammatory disorderunknown etiologydiarthroidal jointssynovium affectedbone, cartilage, ligamentsdeformityextra-articular manifestations


RA - Epidemiology

worldwide distributionall races female > male 3:11% adults in U.SThe most common Inflammatory

disorder of jointAll ages(peak 35-55)


Causes

The cause of rheumatoid arthritis is unknown

Several factors have been identified that may lead to its causeGenetic factorsEnvironmental factorsHormonal factors


Pathophysiology

Ag Genetic Background APC

Ab B cell T Cell

IC Complement PMN

Inflammation


AgBacterialViralFoodsHSP 65 KDCollagen type IIProteoglycans


GENETIC BACKGROUND

First-degree relatives 3%Monozygotic twins 30%HLA-DR4 80%shared epitope (amino acids 70 to 74 of

DR beta chains)


Ag presentation


B Cell RF


Rheumatoid factor series of antibodies that recognize the Fc

portion of an IgG molecule any serotypemost IgMmany conditions associated with RF positivity -

chronic inflammation 70% RA positive at onset, overall 85% in first

two years associated with more severe disease, extra-

articular manifestations, mortalityBy: Dr. Mohammad Hasan Jokar

Anti-Cyclic Citrullinated Peptide Antibodies (AntiCCP)

Sensitivity 80%Specificity 95%


Ag + Ab

Synovium

InflammationBy: Dr. Mohammad Hasan Jokar

Inflammation

Cellular PMN Lysosome Arachidonic acid

HumoralHageman factorKinin system complement


Neuroendocr ine Axis

HypothalamusIL1

Hypophysis

Estrogen Androgen Cortisol

Immune response InflammationBy: Dr. Mohammad Hasan Jokar

Cytokines

I L1

I L6

TNF


IL1T Cell ActivationCartilage destructionBone destructionNeuroendocrne system activationFeverConstitutional symptomsAcute phase reactants


Clinical manifestations


OnsetIn two thirds the onset will begin with aprodromal illness lasting for weeks tomonths. The features of this illness arefatigue, anorexia, generalized weaknessand vague musculo-skeletal symptoms.


Onset

In 10% of people, the onset will be moreacute, with rapid development ofpolyarthritis, often accompanied byconstitutional symptoms including fever,lymphadenopathy and splenomegaly.


Clinical manifestations

Articular

Nonarticular


Articular Features

Pain Swelling TendernessWarmth (large joints) Stiffness (“gel phenomenon”) Deformity Redness is rare Symmetrical polyarthritis


Normal versus Ivoveded joint


Rheumatoid Arthritis


Joints most commonly affected are:

PIPMetacarpophalangeal (MCP)CarpalElbowsMetatarsophalangealFeetKneesUpper cervical spine


Joints spared are:

DIPLumbar spineSacro-iliac


HandsZ deformitySwan neckBoutonniereCarpal tunnel syndrome


“Z deformity”; radial deviation at the wrist, ulnar deviation at MCP joints


Swan neck”; PIP extension, with DIP flexion.


“Boutonniere”; PIP flexion, with DIP extension.


Extra-articular manifestations

Generalfever, lymphadenopathy, weight loss, fatigue

Dermatologicpalmar erythema, nodules, vasculitis

Ocularepiscleritis/scleritis, scleromalacia perforans,

choroid and retinal nodules


Rheumatoid nodule


Rheumatoid nodule

FrequencySize ConsistencyLocationsSymptomsPathologyRF+



Pulmonarypleuritis, nodules, interstitial lung disease

OthersSjogren’s syndrome, amyloidosis


Caplan's syndrome



Cardiacpericarditis, myocarditis, coronary vasculitis,

nodules on valvesNeuromuscularentrapment neuropathy, peripheral neuropathy,

mononeuritis multiplexHematologicAnemia, Felty’s syndrome, lymphomas


Backer’s cyst


Rheumatoid vasculitis


Laboratory abnormalities

anemia of chronic diseaseLeukocytosis thrombocytosis in active disease low white cell count in Felty’sESRCRPRf


Unusual patterns of RA

Adult-onset Still’s diseasePalindromic rheumatismMonoarticular


DDX of a positive RF

normal - 1-4%, 10-25% over age 70systemic autoimmune diseases infectionsmalignancychronic liver diseasepulmonary diseases


RA - differential diagnosis

spondyloarthropathiesCTDspolyarticular goutCPPDviral infectionsfibromyalgia


RA - differential diagnosis

UncommonhypothyroidismSBEhemochromatosishypertrophic pulmonary osteoarthropathyhyperlipoproteinemiashemoglobinopathiesrelapsing polychondritis


X - Ray

Soft tissue swellingJuxtaarticular osteoporosisErosionsAnkylosis


RA: Erosion Progression


Pathology

Synovial lining cell proliferationSubsynovial infiltrationsPannus formation


Synovial thickening


Pannus


Felty’s syndrome classic triad

RA, splenomegaly, leukopenia generally a neutropenia (<2000/mm3) thrombocytopenia may occur complications

infections, non-healing leg ulcersmost require no additional treatment for

cytopenias splenectomy?


RA - ManagementNonpharmacologicPatient educationrest splinting

physical therapyheat, cold, ultrasound, paraffin,

massageoccupational therapy


RA - Management

PharmacologicanalgesicsNSAIDs - full dosecorticosteroids

prednisone at low dose intra-articular steroids


Disease modifying agents

every patient should be considered for at least one modifying agent

MethotrexateAntimalariaSulfasalasineCytotoxic agents


RA - long term prognosis

RA shortens survival and produces disability

1/3 leave work force in five yearsaggressive DMARD TX can reduce

disability by 30% in 10-20 years


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