Background: 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).
Methods: 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.
Results: 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.
Conclusions: 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.
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