Juvenile Rheumatoid Arthritis

Dr Althaf Ahmed 2005 MBBS

Objectives

 recognize the characteristic symptoms and signs of the three clinical subgroups of JRA (JIA)  understand which laboratory and radiology studies are helpful when considering the diagnosis  be familiar with the differential diagnosis including infections, malignancies, and non inflammatory conditions  understand the general approaches to treatment

JUVENILE RHEUMATOID ARTHRITIS  most frequent connective tissue disease of childhood  one of the more common chronic illnesses of childhood and an important cause of disability  currently defined in the U.S. by criteria established by ACR

JUVENILE RHEUMATOID ARTHRITIS American College of Rheumatology Revised Criteria  age of onset < 16 years  arthritis of one or more joints  duration of disease > 6 weeks  other conditions which present with arthritis in childhood must be excluded

JUVENILE RHEUMATOID ARTHRITIS Epidemiology  described in all races and geographic areas  incidence: 6 – 19.6 cases/100,000 children  prevalence: 16-150/100,000  females predominate 2:1

JUVENILE RHEUMATOID ARTHRITIS Etiology and Pathogenesis  unknown  combination of factors  environment (infection, trauma, stress)  autoimmunity  immunogenetic

JUVENILE RHEUMATOID ARTHRITIS Onset Types pauciarticular (< 4 joints) polyarticular (> 5 joints) systemic (arthritis with fever and rash)

ILAR Proposed Classification Criteria

Juvenile Idiopathic Arthritis (JIA) systemic polyarticular RF+ polyarticular RF oligoarticular persistent extended psoriatic arthritis enthesitis-related arthritis other arthritis

JUVENILE RHEUMATOID ARTHRITIS Clinical features: pauciarticular disease  40-60% of patients with JRA  insidious onset  morning irritability/stiffness subtle  systemic symptoms: usually absent  large joints (rarely hip), asymmetric involvement  uveitis 20%  subtypes

JUVENILE RHEUMATOID ARTHRITIS Laboratory Studies: Pauciarticular Disease  CBC: normal  ESR: usually normal  ANA: frequently positive  RF: usually negative  synovial fluid: class II (inflammatory)  x-ray findings: soft tissue swelling, periarticular osteoporosis, growth disturbance, loss of joint space

JUVENILE RHEUMATOID ARTHRITIS Clinical features: polyarticular disease  30-40% of patients with JRA  morning irritability/stiffness more prominent  systemic symptoms: mild to moderate  large and small joints including cervical spine, symmetric involvement  uveitis 5%  subtypes

JUVENILE RHEUMATOID ARTHRITIS Laboratory features: polyarticular disease   WBC  , Hgb  , platelets WNL to  ESR  to    ANA may be positive  RF may be positive  Synovial fluid: class II (inflammatory)  X-ray findings: soft tissue swelling, periarticular osteoporosis, joint space narrowing, erosions

JUVENILE RHEUMATOID ARTHRITIS Clinical features: systemic disease  10-20% of patients with JRA  prominent systemic symptoms: fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, pleuritis  arthritis may be absent for months to years  uveitis uncommon

JUVENILE RHEUMATOID ARTHRITIS Laboratory studies: systemic disease  WBC    ESR   , Hgb  , platelets  to    to   ,  ANA and RF usually negative  x-rays : soft tissue swelling

JUVENILE RHEUMATOID ARTHRITIS Extra-articular Manifestations  generalized or local growth disturbances  delayed puberty  pericarditis, myocarditis, rarely endocarditis  plural effusion, rarely  pneumonitis, pulmonary fibrosis  hepatitis  hematuria

JUVENILE RHEUMATOID ARTHRITIS

Differential Diagnosis

JUVENILE RHEUMATOID ARTHRITIS Treatment  supportive not curative  involves multidisciplinary team approach  goals:    to suppress articular and/or systemic inflammation with as little risk as possible to maintain function/prevent disabilities to foster normal psychological and social development  heterogenity of disease mandates individualization

Pauci Articular

 NSAID 2-4 weeks No response Change NSAID (2-4 weeks) No response Methotrexate no response Intra Articular Steroid

Poly Articular

 NSAID ( 2-4 weeks) no response Change NSAID no response (2-4 weeks) 2 Methotrexate (10-15 mg/m/ week Steroid ( Bridge therapy) Newer drugs

Systemic Onset

Less severe disease

Life threatening/ severe features

Acute onset flare Pulse steroid MP( 30 mg/kg/day) x 3 days.

NSAIDs for 2 weeks.

No response Change NSAIDs( 2 weeks) Oral steroid Oral steroids and taper slowly

Medications in the Treatment of JRA

NSAID

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intra-articular steroids

sulfasalazine hydroxychloroquine (auranofin) 

methotrexate

(IM gold) (D-penicillamine) 

etanercept

azathioprine cyclophosphamide cyclosporin

JUVENILE RHEUMATOID ARTHRITIS Treatment: physical measures heat: splinting: exercise: analgesia muscle relaxation provide joint rest maintain functional position correct deformities passive, active assisted and active range of motion general conditioning rest

JUVENILE RHEUMATOID ARTHRITIS Treatment: education and supportive counseling  understand disease process, treatment and prognosis  understand roles in care  as normal possible:  discipline/family life  school  peer relationships  counseling

JUVENILE RHEUMATOID ARTHRITIS Prognosis  chronic disease which cannot be cured  characterized by flares and remissions  after 10 years or more:  31%-55% persistent active disease  31% (9% - 48%) Steinbrocker Class III and IV

JUVENILE RHEUMATOID ARTHRITIS Poor Prognostic Signs  pauciarticular    long duration of active disease conversion to polyarticular disease (30%) chronic uveitis  polyarticular  long duration of active disease   articular erosions RF positivity/rheumatoid nodules  systemic  conversion to polyarticular disease (25-50%)

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