Cumali Efe1, Murat Torgutalp2, Ida Henriksson3, Fatema Alalkim4, Ellina Lytvyak5, Hirsh Trivedi6, Fatih Eren7, Janett Fischer8, Maneerat Chayanupatkul9,10, Claudia Coppo11, Tugrul Purnak12, Luigi Muratori11, Mårten Werner13, Paolo Muratori11, Fredrik Rorsman14, Kristina Onnerhag15, Emma Nilsson16, Alexandra Heurgué-Berlot17, Nurhan Demir1, David Semela18, Murat Kıyıcı7, Thomas D Schiano9, Aldo J Montano-Loza5, Thomas Berg8, Ersan Ozaslan19, Eric M Yoshida4, Alan Bonder6, Hanns-Ulrich Marschall20, Benedetta Terziroli Beretta-Piccoli21, Staffan Wahlin22. 1. Department of Gastroenterology, Gazi Yaşargil Education and Research Hospital, Diyarbakir, Turkey. 2. Department of Rheumatology, Ankara University Hospital, Ankara, Turkey. 3. Department of Gastroenterology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. 4. Division of Gastroenterology, University of British Columbia and Vancouver General Hospital, Vancouver, British Columbia, Canada. 5. University of Alberta Division of Gastroenterology and Liver Unit, Edmonton, Alberta, Canada. 6. Division of GI and Hepatology, Beth Israel Medical Center, Harvard Medical School, Boston, Massachusetts, USA. 7. Department of Gastroenterology, Medical Faculty, Uludag University, Bursa, Turkey. 8. Division of Gastroenterology, Clinic and Polyclinic for Oncology, Hepatology, Infectious Diseases and Pneumology, University Clinic Leipzig, Leipzig, Germany. 9. Division of Liver Diseases, The Mount Sinai Medical Center, New York, New York, USA. 10. Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. 11. Center for the Study and Treatment of Autoimmune Diseases of the Liver and Biliary System, University of Bologna, Bologna, Italy. 12. Department of Gastroenterology, Hacettepe University, Ankara, Turkey. 13. Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. 14. Department of Gastroenterology and Hepatology, Uppsala University Hospital, Uppsala, Sweden. 15. Department of Gastroenterology and Hepatology, Skåne University Hospital, Malmö, Sweden. 16. Department of Clinical Sciences, Gastroenterology Division, Skåne University Hospital, Lund, Sweden. 17. Department of Hepato-Gastroenterology, CHU Reims, Reims, France. 18. Division of Gastroenterology and Hepatology, Kantonsspital St. Gallen, St. Gallen, Switzerland. 19. Department of Gastroenterology, Ankara City Hospital, Ankara, Turkey. 20. Department of Molecular and Clinical Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. 21. Epatocentro Ticino, Lugano, Switzerland. 22. Hepatology Division, Centre for Digestive Diseases, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
Abstract
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.
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.
Authors: Fatih Karakaya; Sura Oztekin; Yelda Ozturk; Cagdas Kalkan; Zeynep Melekoglu Ellik; Atilla Halil Elhan; Irfan Soykan; Ramazan Idilman Journal: Hepatol Forum Date: 2021-01-08