Liver Involvement in Systemic Lupus Erythematosus: Case Review of 40 Patients

Abstract

ObjectiveSubclinical liver involvement is frequent in systemic lupus erythematosus (SLE). We sought to determine the presence of endstage liver disease in patients with SLE.MethodsWe 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.ResultsForty 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), non-alcoholic 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.ConclusionComplications 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.