Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome

Cumali Efe, Murat Torgutalp, Ida Henriksson, Fatema Alalkim, Ellina Lytvyak, Hirsh Trivedi, Fatih Eren, Janett Fischer, Maneerat Chayanupatkul, Claudia Coppo, Tugrul Purnak, Luigi Muratori, Mårten Werner, Paolo Muratori, Fredrik Rorsman, Kristina Onnerhag, Emma Nilsson, Alexandra Heurgué-Berlot, Nurhan Demir, David SemelaMurat Kıyıcı, Thomas D. Schiano, Aldo J. Montano-Loza, Thomas Berg, Ersan Ozaslan, Eric M. Yoshida, Alan Bonder, Hanns Ulrich Marschall, Benedetta Terziroli Beretta-Piccoli, Staffan Wahlin

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27 Scopus citations


Background and Aim: The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAIDs) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods: The medical records of 1554 patients with PBC from 20 international centers were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal bleeding, and/or hepatic encephalopathy) and hepatocellular carcinoma were considered clinical endpoints. Results: A total of 35 different EHAIDs were diagnosed in 440 (28.3%) patients with PBC. Patients with EHAIDs were more often female (92.5% vs 86.1%, P < 0.001) and seropositive for anti-mitochondrial antibodies (88% vs 84%, P = 0.05) and antinuclear antibodies and/or smooth muscle antibodies (53.8% vs 43.6%, P = 0.005). At presentation, patients with EHAIDs had significantly lower levels of alkaline phosphatase (1.76 vs 1.98 × upper limit of normal [ULN], P = 0.006), aspartate aminotransferase (1.29 vs 1.50 × ULN, P < 0.001), and total bilirubin (0.53 vs 0.58 × ULN, P = 0.002). Patients with EHAIDs and without EHAIDs had similar rates of GLOBE high-risk status (12.3% vs 16.1%, P = 0.07) and Paris II response (71.4% vs 69.4%, P = 0.59). Overall, event-free survival was not different in patients with and without EHAIDs (90.8% vs 90.7%, P = 0.53, log rank). Coexistence of each autoimmune thyroid diseases (10.6%), Sjögren disease (8.3%), systemic sclerosis (2.9%), rheumatoid arthritis (2.7%), systemic lupus erythematosus (1.7%), celiac disease (1.7%), psoriasis (1.5%), and inflammatory bowel diseases (1.3%) did not influence the outcome. Conclusions: Our study confirms that EHAIDs are frequently diagnosed in patients with PBC. The presence of EHAIDs may influence the clinical phenotype of PBC at presentation but has no impact on PBC outcome.

Original languageEnglish
Pages (from-to)936-942
Number of pages7
JournalJournal of Gastroenterology and Hepatology (Australia)
Issue number4
StatePublished - Apr 2021


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