[Adult-onset Still disease with hemophagocytic syndrome treated with cyclosporine]

Presse Med. 2005 Dec 3;34(21):1634-6. doi: 10.1016/s0755-4982(05)84239-3.
[Article in French]

Abstract

Introduction: Adult-onset Still disease can have severe manifestations, the treatment of which is not yet codified.

Case: The authors report a case of life-threatening adult-onset Still disease with hemophagocytic syndrome, which improved dramatically with cyclosporin A. Despite the presence of severe pancytopenia, adult-onset Still disease is suggested by the combination of sore throat, myalgia, rash, hepatitis, high serum ferritin, low glycosylated ferritin, and negative etiologic findings.

Discussion: Still disease may be present in patients with febrile pancytopenia, and cyclosporine may be a useful treatment after corticosteroid failure.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Antirheumatic Agents / therapeutic use*
  • Cyclosporine / therapeutic use*
  • Female
  • Fever / etiology
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / etiology*
  • Middle Aged
  • Pancytopenia / etiology
  • Still's Disease, Adult-Onset / complications*
  • Still's Disease, Adult-Onset / drug therapy*
  • Treatment Outcome

Substances

  • Antirheumatic Agents
  • Cyclosporine