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

AbstractINTRODUCTION: 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.
AuthorsM Hamidou, D Boutoille, A Masseau, R Garand, F Raffi (Affiliation: Service de médecine interne, CHU Hôtel-Dieu, Nantes (44). mohamed.hamidou at chu-nantes.fr)
JournalPresse médicale (Paris, France : 1983) (Presse Med) Vol. 34 Issue 21 Pg. 1634-6 (Dec 3 2005) ISSN: 0755-4982 France
Vernacular TitleMaladie de Still de l'adulte avec syndrome d'activation macrophagique. Intérêt de la ciclosporine.
PMID16327702 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Antirheumatic Agents
  • Cyclosporine
Topics
  • Antirheumatic Agents (therapeutic use)
  • Cyclosporine (therapeutic use)
  • Female
  • Fever (etiology)
  • Humans
  • Lymphohistiocytosis, Hemophagocytic (etiology)
  • Middle Aged
  • Pancytopenia (etiology)
  • Still's Disease, Adult-Onset (complications, drug therapy)
  • Treatment Outcome