Juvenile Rheumatoid Arthritis

Dorothy W. Wortmann, MD
Clinical Associate Professor OU Department of Pediatrics

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 noninflammatory 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 RFoligoarticular
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 , Hgb , platelets to , ESR 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

Medications in the Treatment of JRA

NSAID 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%)