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The clinical characteristics, pre‐ and post–liver transplantation outcomes in patients having autoimmune overlap syndromes
Author(s) -
Chayanupatkul Maneerat,
Fiel Maria Isabel,
Schiano Thomas D.
Publication year - 2020
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/ctr.13841
Subject(s) - medicine , primary sclerosing cholangitis , autoimmune hepatitis , gastroenterology , liver transplantation , primary biliary cirrhosis , overlap syndrome , bile duct , transplantation , hepatitis , disease
Background There are little data on the pre‐ and post–liver transplantation (LT) outcomes of patients having autoimmune hepatitis‐primary biliary cholangitis (AIH‐PBC), AIH‐primary sclerosing cholangitis (AIH‐PSC), and AIH‐small‐duct PSC (AIH‐SDPSC). The aim of this study was to analyze pre‐ and post‐LT outcomes and survival of patients having different overlap syndromes (OS) undergoing LT. Methods Patients with compatible clinical and pathologic features of AIH‐PBC (n = 86), AIH‐PSC (n = 22), and AIH‐SDPSC (n = 9) were included in the study. Demographic, laboratory, clinical, and survival data were analyzed. Multivariable analyses were performed to determine factors predicting transplant‐free survival. Results AIH‐primary sclerosing cholangitis patients were less treatment‐responsive and were more likely to undergo LT than other OS. No survival difference was noted among the 3 groups. Liver decompensation was independently associated with higher mortality (HR 21.78; 95% CI 2.50‐190.01). Thirteen patients with OS underwent LT. One‐year survival post‐LT was 91.7%. Overall recurrence rate for OS post‐LT was 8%. Conclusions AIH‐primary sclerosing cholangitis patients were more likely to require LT compared with patients having AIH‐PBC. Transplant‐free survival was similar among the three AIH‐overlap syndromes. Allograft recurrence of OS occurred in about 10% of cases. Patients with OS appear to have good short‐ and medium‐term post‐LT outcomes in terms of graft function and overall survival.