[A case of fulminant hepatic failure in Wilson's disease combined with systemic lupus erythematosus].
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.
Kim
Tae Yeob
TY
Institute of Digestive Disease, The Hospital for Rheumatic Diseases, Hanyang University College of Medicine, Seoul, Korea. eastlee@hanyang.ac.kr
Lee
Soong Hwan
SH
Kim
Tae Jong
TJ
Cho
Kyung Ran
KR
Cho
Seung Chul
SC
Han
Sung Hee
SH
Yang
Sun Young
SY
Chung
Yong Woo
YW
Sohn
Joo Hyun
JH
Baik
Sang Hyun
SH
Bae
Sang Cheol
SC
Lee
Dong Hoo
DH
Kee
Choon Suhk
CS
D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D017093:Liver Failure / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*