Chowdhary Vaidehi R, Crowson Cynthia S, Poterucha John J, Moder Kevin G
Division of Rheumatology, Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
J Rheumatol. 2008 Nov;35(11):2159-64. doi: 10.3899/jrheum.080336. Epub 2008 Sep 15.
Subclinical liver involvement is frequent in systemic lupus erythematosus (SLE). We sought to determine the presence of endstage liver disease in patients with SLE.
We carried out a retrospective chart review of our cohort of patients with SLE. Endstage liver disease was defined as presence or development of cirrhosis, portal hypertension, or hepatic encephalopathy.
Forty patients with liver enzyme abnormalities were identified. Major clinical diagnostic groups were drug-induced (n = 4), viral hepatitis (hepatitis B or C and cytomegalovirus; n = 8), nonalcoholic fatty liver disease (NAFLD; n = 8), autoimmune hepatitis (AIH; n = 6), primary biliary cirrhosis (PBC; n = 3), and miscellaneous [n = 11; liver involvement from infection (2), cryptogenic cirrhosis (2), lymphoma (1), and indeterminate (6)]. There were no differences in mean age, total and direct bilirubin, or aspartate aminotransferase and alkaline phosphatase levels. Alanine aminotransferase levels were higher in the miscellaneous group. Biopsies were performed in 20 patients and showed changes of NAFLD (n = 5), AIH (n = 4), PBC (n = 3), hepatitis C (n = 3), and cryptogenic cirrhosis (n = 2), and 1 each with phenytoin-induced liver injury, hepatic granulomas due to systemic candidiasis, and lymphomatous involvement of the liver. The median followup was 44 months (range 10-576). The estimated 5-year serious liver disease-free survival was 93% (95% confidence interval 84%-100%). Eight patients died. Mortality was not directly related to liver disease in any patient.
Complications of portal hypertension, cirrhosis, and hepatic encephalopathy are rare manifestations of SLE unless coexistent liver disease such as NAFLD, viral hepatitis, or AIH is present.
亚临床肝脏受累在系统性红斑狼疮（SLE）中很常见。我们试图确定SLE患者中终末期肝病的存在情况。
我们对我们的SLE患者队列进行了回顾性病历审查。终末期肝病定义为存在或发展为肝硬化、门静脉高压或肝性脑病。
确定了40例肝酶异常患者。主要临床诊断组为药物性（n = 4）、病毒性肝炎（乙型或丙型肝炎及巨细胞病毒；n = 8）、非酒精性脂肪性肝病（NAFLD；n = 8）、自身免疫性肝炎（AIH；n = 6）、原发性胆汁性肝硬化（PBC；n = 3）以及其他[ n = 11；感染引起的肝脏受累（2例）、隐源性肝硬化（2例）、淋巴瘤（1例）和不明原因（6例）]。平均年龄、总胆红素和直接胆红素、或天冬氨酸转氨酶和碱性磷酸酶水平无差异。丙氨酸转氨酶水平在其他组中较高。20例患者进行了活检，显示为NAFLD（n = 5）、AIH（n = 4）、PBC（n = 3）、丙型肝炎（n = 3）和隐源性肝硬化（n = 2）的改变，各有1例分别为苯妥英钠引起的肝损伤、系统性念珠菌病引起的肝肉芽肿和肝脏淋巴瘤浸润。中位随访时间为44个月（范围10 - 576个月）。估计5年无严重肝病生存率为93%（95%置信区间84% - 100%）。8例患者死亡。在任何患者中，死亡率均与肝病无直接关系。
除非存在如NAFLD、病毒性肝炎或AIH等并存的肝病，门静脉高压、肝硬化和肝性脑病的并发症是SLE的罕见表现。
