MAS Treatment in Still Disease — SCE Rheumatology MCQ

Instant feedback + full explanation. One question, done properly.

HardRheumatology EmergenciesMAS Treatment in Still DiseaseSCE Rheumatology

A 30-year-old man with adult-onset Still disease develops macrophage activation syndrome (MAS). His ferritin is 42000 microg/L platelets 45 x10^9/L fibrinogen 0.8 g/L (low) and triglycerides 6.2 mmol/L. What is the first-line treatment for MAS?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: D — High-dose IV Methylprednisolone with early consideration of Anakinra (IL-1 receptor antagonist) or Ciclosporin

MAS in adult-onset Still disease is a life-threatening emergency requiring immediate high-dose IV Methylprednisolone (1 g daily for 3-5 days). Anakinra (IL-1 receptor antagonist) has shown rapid efficacy in MAS associated with Still disease and is increasingly used early in the treatment algorithm. Ciclosporin is also effective and may be combined with steroids. Etoposide (used in primary HLH) is reserved for refractory cases. Key monitoring includes ferritin (which should fall with treatment) FBC fibrinogen (rising fibrinogen indicates response) LFTs and triglycerides. ITU admission is often required for severe MAS.

Reference: Ravelli A et al 2016 ACR/EULAR MAS criteria; Giacomelli R et al 2018 Still disease treatment