The Korean Journal of Hepatology  2002;8(1):100-104

A Case of Fulminant Hepatic Failure in Wilson's Disease Combined with Systemic Lupus Erythematosus.

Tae Yeob KIM 1 ; Soong Hwan LEE ; Tae Jong KIM ; Kyung Ran CHO ; Seung Chul CHO ; Sung Hee HAN ; Sun Young YANG ; Yong Woo CHUNG ; Joo Hyun SOHN ; Sang Hyun BAIK ; Sang Cheol BAE ; Dong Hoo LEE ; Choon Suhk KEE

Affiliations

+expand

Keywords

Systemic lupus erythematosus; Wilson's disease; Fulminant hepatic failure

Country

Republic of Korea

Language

Korean

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.