High-dose anakinra (8 mg/kg/d) led to fast clinical and laboratory improvement, disease remission, and complete recovery of cardiac function in a patient with refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis.
Case Report (n=1)
No
Does high-dose anakinra improve outcomes in refractory adult-onset Still's disease complicated by macrophage activation syndrome and acute myocarditis?
High-dose anakinra may be an effective rescue therapy for refractory adult-onset Still's disease complicated by life-threatening macrophage activation syndrome and acute myocarditis.
RATIONALE: 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. DIAGNOSES: Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest. INTERVENTIONS: 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. OUTCOMES: Fast clinical and laboratoristic improvement and subsequent disease remission with complete recovery of cardiac function. LESSONS: 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.
Parisi et al. (Thu,) conducted a case report in Adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis (n=1). Anakinra was evaluated on Clinical and laboratory improvement and recovery of cardiac function. High-dose anakinra (8 mg/kg/d) led to fast clinical and laboratory improvement, disease remission, and complete recovery of cardiac function in a patient with refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis.