Adult-Onset Still Disease — SCE Rheumatology MCQ

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ModerateRheumatology EmergenciesAdult-Onset Still DiseaseSCE Rheumatology

A 50-year-old man with adult-onset Still disease presents with quotidian (daily) spiking fevers salmon-pink evanescent rash pharyngitis lymphadenopathy and markedly elevated ferritin at 18000 microg/L with glycosylated ferritin fraction of 12% (normal >50%). What treatment is most effective for refractory adult-onset Still disease?

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Correct answer: C — IL-1 inhibition (Anakinra or Canakinumab)

Adult-onset Still disease (AOSD) is an autoinflammatory condition with characteristic features: quotidian fevers evanescent rash pharyngitis serositis hepatosplenomegaly and markedly elevated ferritin (often >10000) with characteristically low glycosylated ferritin (<20%). IL-1 inhibition (Anakinra Canakinumab) has transformed the management of refractory AOSD with rapid and dramatic responses. Glucocorticoids are first-line but relapse is common on tapering. Methotrexate is used as a steroid-sparing agent. Tocilizumab (anti-IL-6) is an alternative biologic option. MAS is a life-threatening complication.

Reference: Yamaguchi M et al 1992 AOSD criteria; Giacomelli R et al 2018 AOSD treatment