Adult-onset Still's disease medical therapy

Adult-onset Still's disease
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief:

Medical Therapy

Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. The following agents may be used in the management of AOSD:

• 1.1 Adults
  • Preferred regimen (1): Prednisone PO 0.8-1 mg/kg/day
• 1.2 Adults (Steroid dependence)
  • Preferred regimen (1): Methotrexate 7.5-20 mg/week
  • Alternative regimen (1): Combination of prednisolone 20 mg/day plus oral methotrexate 5 mg/week plus cyclosporin A 5.5 mg/kg/day
• 1.3 Adults (Refractory AOSD)
  • 1.3.1 Chronic arthritis
    • Preferred regimen (1): Infliximab IV initial 3 mg/kg at 0, 2, and 6 weeks; then 3 to 6 mg/kg/dose every 8 weeks
    • Preferred regimen (2): Tocilizumab IV  8 mg/kg
    • Alternative regimen (1): Leflunomide <20 kg: 100 mg as a single dose followed by 10 mg every other day; 20 kg to 40 kg: 100 mg once daily for 2 days followed by 10 mg once daily; >40 kg: 100 mg once daily for 3 days followed by 20 mg once daily and Azathioprine initial1 mg/kg/day (50 to 100 mg) given once daily or divided twice daily; after 6 to 8 weeks, may increase by 0.5 mg/kg every 4 weeks until response or up to 2.5 mg/kg/day; an adequate trial should be a minimum of 12 weeks
  • 1.3.2 Systemic flares
    • Preferred regimen (1): Anakinra SC initial 1 to 2 mg/kg once daily; maximum initial dose: 100 mg; if no response after 1 to 2 weeks, may titrate up to 4 mg/kg once daily (maximum: 200 mg/day)
    • Alternative regimen (1): Canakinumab  SC children ≥2 years and ≥7.5 kg and Adolescents: 4 mg/kg every 4 weeks (maximum: 300 mg per dose)
    • Alternative regimen (2): Rilonacept
• 1.4 Adults (Complicated AOSD)
  • Preferred regimen (1): Methylprednisone IV
  • Preferred regimen (2):  Immunoglobulin IV (IVIG) 2 g/kg body weight administered in 2–5 days.
  • Preferred regimen (3): Cyclophosphamide PO 1 to 5 mg/kg/day (initial and maintenance dosing)
  • Preferred regimen (4): Plasma exchange therapy