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.

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

Risultato della ricerca: Contributo in rivistaArticolo in rivista

28 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)e6656-N/A
RivistaMEDICINE
Volume96
DOI
Stato di pubblicazionePubblicato - 2017

Keywords

  • Adult
  • Antirheumatic Agents
  • Drug Resistance
  • Female
  • Humans
  • Interleukin 1 Receptor Antagonist Protein
  • Macrophage Activation Syndrome
  • Medicine (all)
  • Myocarditis
  • Still's Disease, Adult-Onset
  • adult-onset Still disease
  • anakinra
  • macrophage activation syndrome
  • myiocarditis

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