title: [A case of fulminant hepatic failure in Wilson's disease combined with systemic lupus erythematosus].
abstract: Patients with systemic lupus erythematosus (SLE) have a chance of developing liver involvement in their lifetime. The main cause of liver involvement in SLE patients is previous treatment with hepatotoxic drugs or hepatotropic viral hepatitis. Wilson's disease is a hereditary disorder and is usually diagnosed in patients presenting either neuropsychiatric disorders or manifestations related to chronic liver disease. Fulminant hepatic failure as the initial manifestation of Wilson's disease is rare. The relationship between systemic lupus erythematosus and Wilson's disease has not been established. We report a case of a 12-year-old girl with SLE who presented fulminant hepatic failure as an initial manifestation of Wilson's disease. The diagnosis was established with decreased serum ceruloplasmin level and the presence of Kayser-Fleischer ring. We treated with repeated plasma exchange. Despite repeated plasma exchange she died of multi-organ failure on the 16th hospital day. Considering this case, Wilson's disease should be considered as a cause of fulminant hepatic failure, especially in juvenile age cases.
authors.affiliation: Institute of Digestive Disease, The Hospital for Rheumatic Diseases, Hanyang University College of Medicine, Seoul, Korea. eastlee@hanyang.ac.kr
authors.forename: Tae Yeob
authors.initials: TY
authors.lastname: Kim
authors.forename: Soong Hwan
authors.initials: SH
authors.lastname: Lee
authors.forename: Tae Jong
authors.initials: TJ
authors.lastname: Kim
authors.forename: Kyung Ran
authors.initials: KR
authors.lastname: Cho
authors.forename: Seung Chul
authors.initials: SC
authors.lastname: Cho
authors.forename: Sung Hee
authors.initials: SH
authors.lastname: Han
authors.forename: Sun Young
authors.initials: SY
authors.lastname: Yang
authors.forename: Yong Woo
authors.initials: YW
authors.lastname: Chung
authors.forename: Joo Hyun
authors.initials: JH
authors.lastname: Sohn
authors.forename: Sang Hyun
authors.initials: SH
authors.lastname: Baik
authors.forename: Sang Cheol
authors.initials: SC
authors.lastname: Bae
authors.forename: Dong Hoo
authors.initials: DH
authors.lastname: Lee
authors.forename: Choon Suhk
authors.initials: CS
authors.lastname: Kee
mesh_terms: D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D017093:Liver Failure / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*