Kim Tae Yeob, Lee Soong Hwan, Kim Tae Jong, Cho Kyung Ran, Cho Seung Chul, Han Sung Hee, Yang Sun Young, Chung Yong Woo, Sohn Joo Hyun, Baik Sang Hyun, Bae Sang Cheol, Lee Dong Hoo, Kee Choon Suhk
Institute of Digestive Disease, The Hospital for Rheumatic Diseases, Hanyang University College of Medicine, Seoul, Korea.
Taehan Kan Hakhoe Chi. 2002 Mar;8(1):100-4.
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.
系统性红斑狼疮（SLE）患者一生中都有发生肝脏受累的可能。SLE患者肝脏受累的主要原因是既往使用过肝毒性药物或感染嗜肝性病毒性肝炎。威尔逊病是一种遗传性疾病，通常在出现神经精神障碍或与慢性肝病相关表现的患者中被诊断出来。以暴发性肝衰竭作为威尔逊病的初始表现较为罕见。系统性红斑狼疮与威尔逊病之间的关系尚未确立。我们报告一例12岁患SLE的女孩，其以暴发性肝衰竭作为威尔逊病的初始表现。通过血清铜蓝蛋白水平降低和凯-弗环的出现得以确诊。我们采用了重复血浆置换进行治疗。尽管进行了重复血浆置换，她仍在住院第16天死于多器官功能衰竭。考虑到该病例，威尔逊病应被视为暴发性肝衰竭的一个病因，尤其是在青少年病例中。
