Epidemiology

  • Incidence: 0.50 cases per 100,000 person-years
  • Peak incidence: 20-30 years
  • Sex ratio: 1:1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Clinical features

Features
  • arthralgia
  • elevated serum ferritin
  • rash: salmon-pink, maculopapular
  • pyrexia
    • typically rises in the late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms and rash
  • lymphadenopathy

Investigations

Autoantibodies
  • rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative

Management

Management
  • NSAIDs
    • should be used first-line to manage fever, joint pain and serositis
    • they should be trialled for at least a week before steroids are added.
  • steroids
    • may control symptoms but won't improve prognosis
  • if symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered