전신성 홍반성 루푸스(systemic lupus erythematosus:SLE)환자는 일생동안 비정상적인 간기능 검사소견이 25-50%에서 나타나며, SLE 자체의 간침범은 드물다. 비정상 간기능 소견을 보이는 대부분의 원인은 치료약제의 간독성 혹은 바이러스 간염이 주된 간 질환의 원인이다.1,2 드물게 이러한 원인들이 배제된 경우에는 SLE와 유사한 임상양상을 보이는 자가면역성 간염과 SLE 자체의 간침범에 의한 경우를 감별해야 한다.3 그밖에 다양한 간질환이 SLE와 동반되어 나타날 수 있다. 윌슨병(Wilson's disease)은 유전적 대사질환으로 대부분 신경 정신 질환 혹은 만성 간 질환으로 나타나며 처음 증상발현이 전격성 간부전으로 나타나는 경우는 드물다.4 그러나 30세 이하에서 전격성 간부전을 보이는 경우에는 원인질환으로 윌슨병을 감별해야 한다.4 전격성 간부전으로 처음 증상 발현이 된 경우는 광범위한 간 괴사가 수일 혹은 수주 이내에 일어나므로 치명적이다. 일반적으로 윌슨병으로 인한 전격성 간부전은 간이식술 없이는 치사율이 높으므로 조기에 진단하는 것이 중요하다.The Korean Journal of Hepatology : Vol. 8. No. 1. 2002 Tae Yeob Kim, et al. SLE and Wilsonian Fulminant Hepatic Failure Figure 1. Kayser-Fleischer ring is present at the periphery of the cornea. 저자들은 SLE로 경과 관찰 중 윌슨병에 의한 전격성 간부전이 나타난 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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.(Korean J Hepatol 2002;8:100-104)

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.(Korean J Hepatol 2002;8:100-104)

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