Severe hyperferritinemia--a clue for severe hepatitis in a patient with adult-onset Still's disease

Clin Rheumatol. 2016 Mar;35(3):795-800. doi: 10.1007/s10067-014-2829-2. Epub 2014 Nov 22.

Abstract

A 19-year old previously healthy man developed the adult-onset Still's disease (AOSD) with high ferritin levels. Corticosteroids induced clinical remission with resolution of fever, arthritis, and rash. While tapering corticosteroids, the patient developed severe liver enzymes elevation, very high ferritin levels and, subsequently, acute liver failure. After other causes of liver disease (infections, metabolic, autoimmune hepatitis, lymphoma, and hemophagocytosis) were excluded, severe hepatitis was attributed to AOSD itself. Cyclosporine induced rapid normalization of liver enzymes and reduction in ferritin levels. Severe hepatitis and very high ferritin levels could be the only manifestation of disease activity in AOSD; therefore, monitoring of liver enzymes and ferritin levels is recommended even after resolution of the clinical symptoms of AOSD. Prompt initiation of cyclosporine can improve liver function and prevent progression to liver failure.

Keywords: Adult-onset Still’s disease; Cyclosporine; Ferritin; Hyperferritinemia; Severe hepatitis.

Publication types

  • Case Reports

MeSH terms

  • Cyclosporine / therapeutic use
  • Ferritins / blood*
  • Hepatitis / blood
  • Hepatitis / complications
  • Hepatitis / diagnosis*
  • Hepatitis / drug therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Still's Disease, Adult-Onset / blood*
  • Still's Disease, Adult-Onset / complications
  • Still's Disease, Adult-Onset / drug therapy
  • Treatment Outcome
  • Young Adult

Substances

  • Immunosuppressive Agents
  • Cyclosporine
  • Ferritins