Successful treatment with cyclosporin in adult-onset Still disease manifesting as acute hepatitis with marked hyperferritinemia.
A 48-year-old woman was admitted because of spiking high fever, sore throat, and jaundice. A diagnosis was made of adult-onset Still disease (AOSD) presenting with acute hepatitis and very high serum ferritin levels (32,240 ng/mL), and she was treated with 2 courses of pulse therapy of methylprednisolone (2 g/day for 3 days) followed by 40 mg/day prednisolone. Subsequently, the serum level of ferritin decreased, but serum total bilirubin increased to 17.3 mg/dL. Therefore, cyclosporin was administered orally. Within the next 3 months, results of liver function tests, as well as serum levels of ferritin, soluble interleukin-2 receptor, interferon-gamma, interleukin-6, and tumor necrosis factor-alpha gradually returned to within normal limits, and cyclosporin administration was subsequently reduced gradually. The clinical presentation suggests that AOSD should be considered when liver dysfunction is accompanied with high fever and extreme hyperferritinemia, and that treatment with cyclosporin or other immunosuppressive drugs that selectively suppress cytokine production by helper T cells is a valuable option in the treatment of AOSD with very high serum ferritin levels.
D000284:Administration, Oral; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D005293:Ferritins / Q000097:blood*; D006505:Hepatitis / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008099:Liver / Q000187:drug effects; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D016706:Still's Disease, Adult-Onset / Q000188:drug therapy*
D018501:Antirheumatic Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D005293:Ferritins; D001219:Aspartate Aminotransferases; D001663:Bilirubin; D008775:Methylprednisolone