Autoimmune Extrahepatic Disorders in Patients With Autoimmune Liver Disease

Abstract

Autoimmune liver diseases (ALDs) (primary biliary cholangitis, primary sclerosing cholangitis [PSC], autoimmune hepatitis [AIH]) can present extrahepatic autoimmune manifestations, the most frequent being inflammatory bowel disease (IBD), autoimmune thyroid disease, and Sjögren syndrome (SS). Retrospective study of patients who have undergone liver transplant (LT) with post-LT follow-up of at least 2 years. Descriptive analysis of clinical variables and overall and graft survival. ALD was an infrequent indication for LT (68 of 835, 8%), 39 primary biliary cholangitis, 17 AIH, and 12 PSC; 56 were women. The mean (standard deviation [SD]) pre-LT Model for End-Stage Liver Disease score was 17 (5.4). The mean (SD) age of LT recipients at LT was 40 (21) years. A total of 27 patients presented extrahepatic autoimmune diseases. The most frequent was IBD in 7 patients, preferentially in patients with PSC (10/12), followed by Sjögren syndrome and autoimmune thyroid disease. IBD was present in 12 patients: 8 ulcerative colitis (6 PSC and 2 AIH overlap syndrome), 2 Crohn disease both PSC, and another 2 PSC and IBD without conclusive diagnosis (neither for ulcerative colitis nor Crohn disease). Five presented IBD de novo post-LT; the other 7 debuted before LT. In 3 of these 7 patients with pre-LT IBD, the disease went into remission after LT. Colectomy was necessary in 3 patients. No statistically significant findings were found in the survival analysis. ALD is an infrequent reason for LT. Extrahepatic autoimmune diseases are associated in these patients, with IBD being the most frequent. IBD presents a torpid course but does not impact overall survival.