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Dr. M Jokar

By: Dr. Mohammad Hasan Jokar

RA - Definition

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

By: Dr. Mohammad Hasan Jokar

RA - Epidemiology

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

disorder of jointAll ages(peak 35-55)

By: Dr. Mohammad Hasan Jokar

Causes

The cause of rheumatoid arthritis is unknown

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

By: Dr. Mohammad Hasan Jokar

Pathophysiology

Ag Genetic Background APC

Ab B cell T Cell

IC Complement PMN

Inflammation

By: Dr. Mohammad Hasan Jokar

AgBacterialViralFoodsHSP 65 KDCollagen type IIProteoglycans

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GENETIC BACKGROUND

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

DR beta chains)

By: Dr. Mohammad Hasan Jokar

Ag presentation

By: Dr. Mohammad Hasan Jokar

B Cell RF

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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%

By: Dr. Mohammad Hasan Jokar

Ag + Ab

Synovium

InflammationBy: Dr. Mohammad Hasan Jokar

Inflammation

Cellular PMN Lysosome Arachidonic acid

HumoralHageman factorKinin system complement

By: Dr. Mohammad Hasan Jokar

Neuroendocr ine Axis

HypothalamusIL1

Hypophysis

Estrogen Androgen Cortisol

Immune response InflammationBy: Dr. Mohammad Hasan Jokar

Cytokines

I L1

I L6

TNF

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IL1T Cell ActivationCartilage destructionBone destructionNeuroendocrne system activationFeverConstitutional symptomsAcute phase reactants

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Clinical manifestations

By: Dr. Mohammad Hasan Jokar

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.

By: Dr. Mohammad Hasan Jokar

Onset

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

By: Dr. Mohammad Hasan Jokar

Clinical manifestations

Articular

Nonarticular

By: Dr. Mohammad Hasan Jokar

Articular Features

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

By: Dr. Mohammad Hasan Jokar

Normal versus Ivoveded joint

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Rheumatoid Arthritis

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Joints most commonly affected are:

PIPMetacarpophalangeal (MCP)CarpalElbowsMetatarsophalangealFeetKneesUpper cervical spine

By: Dr. Mohammad Hasan Jokar

Joints spared are:

DIPLumbar spineSacro-iliac

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HandsZ deformitySwan neckBoutonniereCarpal tunnel syndrome

By: Dr. Mohammad Hasan Jokar

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

By: Dr. Mohammad Hasan Jokar

Swan neck”; PIP extension, with DIP flexion.

By: Dr. Mohammad Hasan Jokar

“Boutonniere”; PIP flexion, with DIP extension.

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Extra-articular manifestations

Generalfever, lymphadenopathy, weight loss, fatigue

Dermatologicpalmar erythema, nodules, vasculitis

Ocularepiscleritis/scleritis, scleromalacia perforans,

choroid and retinal nodules

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Rheumatoid nodule

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Rheumatoid nodule

FrequencySize ConsistencyLocationsSymptomsPathologyRF+

By: Dr. Mohammad Hasan Jokar

Extra-articular manifestations

Pulmonarypleuritis, nodules, interstitial lung disease

OthersSjogren’s syndrome, amyloidosis

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Caplan's syndrome

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Extra-articular manifestations

Cardiacpericarditis, myocarditis, coronary vasculitis,

nodules on valvesNeuromuscularentrapment neuropathy, peripheral neuropathy,

mononeuritis multiplexHematologicAnemia, Felty’s syndrome, lymphomas

By: Dr. Mohammad Hasan Jokar

Backer’s cyst

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Rheumatoid vasculitis

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Laboratory abnormalities

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

By: Dr. Mohammad Hasan Jokar

Unusual patterns of RA

Adult-onset Still’s diseasePalindromic rheumatismMonoarticular

By: Dr. Mohammad Hasan Jokar

DDX of a positive RF

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

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

RA - differential diagnosis

spondyloarthropathiesCTDspolyarticular goutCPPDviral infectionsfibromyalgia

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RA - differential diagnosis

UncommonhypothyroidismSBEhemochromatosishypertrophic pulmonary osteoarthropathyhyperlipoproteinemiashemoglobinopathiesrelapsing polychondritis

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X - Ray

Soft tissue swellingJuxtaarticular osteoporosisErosionsAnkylosis

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RA: Erosion Progression

By: Dr. Mohammad Hasan Jokar

By: Dr. Mohammad Hasan Jokar

Pathology

Synovial lining cell proliferationSubsynovial infiltrationsPannus formation

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Synovial thickening

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Pannus

By: Dr. Mohammad Hasan Jokar

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?

By: Dr. Mohammad Hasan Jokar

RA - ManagementNonpharmacologicPatient educationrest splinting

physical therapyheat, cold, ultrasound, paraffin,

massageoccupational therapy

By: Dr. Mohammad Hasan Jokar

RA - Management

PharmacologicanalgesicsNSAIDs - full dosecorticosteroids

prednisone at low dose intra-articular steroids

By: Dr. Mohammad Hasan Jokar

Disease modifying agents

every patient should be considered for at least one modifying agent

MethotrexateAntimalariaSulfasalasineCytotoxic agents

By: Dr. Mohammad Hasan Jokar

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

By: Dr. Mohammad Hasan Jokar