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, F., Paglionico, A., Varriano, V., Ferraccioli, G., Gremese, E., Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: A case report treated with high doses (8mg/kg/d) of anakinra., <<MEDICINE>>, 2017; 96 (24): e6656-N/A. [doi:10.1097/MD.0000000000006656] [http://hdl.handle.net/10807/122673]

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

Parisi, Federico;Paglionico, Annamaria;Varriano, Valentina;Ferraccioli, Gianfranco;Gremese, Elisa
2017

Abstract

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.
Inglese
http://journals.lww.com/md-journal
Parisi, F., Paglionico, A., Varriano, V., Ferraccioli, G., Gremese, E., Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: A case report treated with high doses (8mg/kg/d) of anakinra., <>, 2017; 96 (24): e6656-N/A. [doi:10.1097/MD.0000000000006656] [http://hdl.handle.net/10807/122673]
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10807/122673
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