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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

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juvenile rheumatoid arthritis

Juvenile Rheumatoid Arthritis

Dr Althaf Ahmed

2005 MBBS

objectives
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 arthritis1
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
JUVENILE RHEUMATOID ARTHRITISAmerican 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
JUVENILE RHEUMATOID ARTHRITISEpidemiology
  • 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
JUVENILE RHEUMATOID ARTHRITISEtiology and Pathogenesis
  • unknown
  • combination of factors
    • environment (infection, trauma, stress)
    • autoimmunity
    • immunogenetic
juvenile rheumatoid arthritis onset types
JUVENILE RHEUMATOID ARTHRITISOnset Types

pauciarticular (< 4 joints)

polyarticular (> 5 joints)

systemic (arthritis with fever and rash)

ilar proposed classification criteria
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
JUVENILE RHEUMATOID ARTHRITISClinical 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
JUVENILE RHEUMATOID ARTHRITISLaboratory 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
JUVENILE RHEUMATOID ARTHRITISClinical 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
JUVENILE RHEUMATOID ARTHRITISLaboratory 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
JUVENILE RHEUMATOID ARTHRITISClinical 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
JUVENILE RHEUMATOID ARTHRITISLaboratory 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
JUVENILE RHEUMATOID ARTHRITISExtra-articular Manifestations
  • generalized or local growth disturbances
  • delayed puberty
  • pericarditis, myocarditis, rarely endocarditis
  • plural effusion, rarely
  • pneumonitis, pulmonary fibrosis
  • hepatitis
  • hematuria
juvenile rheumatoid arthritis2

JUVENILE RHEUMATOID ARTHRITIS

Differential Diagnosis

juvenile rheumatoid arthritis treatment
JUVENILE RHEUMATOID ARTHRITISTreatment
  • 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
Pauci Articular
  • NSAID 2-4 weeks No response Change NSAID (2-4 weeks) No response Methotrexate no response Intra Articular Steroid
poly articular
Poly Articular
  • NSAID (2-4 weeks) no response Change NSAID (2-4 weeks) no response 2 Methotrexate (10-15 mg/m/ week Steroid ( Bridge therapy) Newer drugs
systemic onset
Systemic Onset

Less severe disease NSAIDs for 2 weeks. No response Change NSAIDs( 2 weeks) Oral steroids and taper slowly

medications in the treatment of jra
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
JUVENILE RHEUMATOID ARTHRITISTreatment: physical measures

heat: analgesia

muscle relaxation

splinting: provide joint rest

maintain functional position

correct deformities

exercise: passive, active assisted and

active range of motion

general conditioning

rest

juvenile rheumatoid arthritis treatment education and supportive counseling
JUVENILE RHEUMATOID ARTHRITISTreatment: 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
JUVENILE RHEUMATOID ARTHRITISPrognosis
  • 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
JUVENILE RHEUMATOID ARTHRITISPoor 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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