4.2 Article

Adult-onset Still's Disease Complicated by Autoimmune Hepatitis: Successful Treatment with Infliximab

Journal

INTERNAL MEDICINE
Volume 51, Issue 9, Pages 1125-1128

Publisher

JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.51.6824

Keywords

adult-onset Still's disease; autoimmune hepatitis; Sjogren's syndrome; IgG4-related disease; TNF inhibitor

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A 17-year-old woman was previously diagnosed with autoimmune hepatitis (AIH) by liver biopsy. Adult-onset Still's disease (AOSD) was subsequently diagnosed on the basis of high fever, arthralgia, erythema, leukocytosis (>80% granulocytes), cervical lymph node swelling, splenomegaly, and hyperferritinemia. Her symptoms and liver dysfunction improved with prednisolone of 60 mg daily and subsequently methotrexate was added. However her symptoms and liver dysfunction relapsed when prednisolone was tapered to 20 mg/day. Therefore infliximab was introduced additionally and her symptoms and liver dysfunction subsided. To our knowledge, this is the first reported case of AOSD with AIH diagnosed by liver biopsy.

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