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Infection

Juvenile idiopathic arthritis - diagnosis and treatment

JRA subtypes, chronic monoarthritis, distinguishing infection, DMARD treatment.

7 questions 1 source pages

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7 questions
Q1What is the differential diagnosis of recurrent knee effusion in a child?▸
  • Infection
  • Trauma (NAI)
  • Inflammatory (JRA)
Q2What is juvenile rheumatoid arthritis (JRA) / juvenile idiopathic arthritis?▸
  • A persistent autoimmune inflammatory arthritis lasting >6 weeks
  • In a patient younger than 16 years of age
  • Female > male
Q3What is the genetic association and what are the types of JRA?▸
  • HLA-DR4 (polyarthritis); HLA-DR8, 5, 2.1; HLA-B27 (pauciarthritis)
  • Polyarthritis >5 joints (similar to RA) - 30%
  • Oligoarthritis <5 joints (similar to seronegative, commonly eye problems) - 50%
  • Systemic (Still disease) - 20%
Q4What are the diagnostic criteria for JRA?▸
  • Diagnosis by exclusion plus one of the following:
  • Rash, presence of RF, iridocyclitis, C-spine involvement
  • Pericarditis, tenosynovitis, intermittent fever, morning stiffness
Q5What are the clinical features of JRA?▸
  • General: intermittent fever, rash, pericarditis, iridocyclitis (inflammation of iris and ciliary body)
  • Ortho: C-spine involvement (kyphosis, facet ankylosis, atlantoaxial subluxation), tenosynovitis, morning stiffness
  • Osteoporosis with easy fracture; growth retardation (corticosteroid suppression, Shapiro III/V)
  • Ankylosis of joints in systemic type
  • Deformity: external torsion of tibia, distal ulna dysplasia, underdeveloped mandible (micrognathia), short neck and scoliosis
Q6What is Still disease and what is the prognosis of JRA?▸
  • Still disease: acute presentation with multiple joint involvement, fever, salmon coloured rash, splenomegaly, usually <3 years old
  • Prognosis: 50% spontaneous resolve, 25% slight limitation, 25% severe disability
  • Best prognosis pauciarticular > polyarticular > systemic
Q7What investigations and treatments are used in JRA?▸
  • X-ray: juxta-articular, late osteopenia and joint destruction; C-spine X-ray for atlantoaxial instability
  • Blood: RF (worse prognosis if +ve), ANA (+ve is diagnostic), HLA typing
  • Physiotherapy to maintain range and power
  • DMARD (azathioprine, etanercept) and biological agents (rituximab)
  • Surgery: synovectomy, a la carte
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