Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: A case report treated with high doses (8 mg/kg/d) of anakinra

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Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases).

Patient concerns:

A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure.


Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest.


After failure of conventional therapies (steroids plus cyclosporine and then biological therapy with Anakinra 100 mg/day), the patient was treated with anakinra 100 mg sc 1 fl 4 times a day.


Fast clinical and laboratoristic improvement and subsequent disease remission with complete recovery of cardiac function.


This is the first case report in which high doses of Anakinra have been used to treat a refractory AOSD complicated by MAS and myocarditis. In AOSD complicated by life-threatening conditions, probably we need to consider aggressive therapeutic approaches with higher doses of Il-1 receptor blocker to switch off the hyper-inflammation.

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