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

Publication

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

Journal Paper/Review - Aug 13, 2020

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

Citation

Efe C, Nilsson E, Heurgué-Berlot A, Demir N, Semela D, Kiyici M, Schiano T, Montano-Loza A, Berg T, Ozaslan E, Yoshida E, Bonder A, Marschall H, Beretta-Piccoli B, Önnerhag K, Rorsman F, Torgutalp M, Henriksson I, Alalkim F, Lytvyak E, Trivedi H, Eren F, Fischer J, Chayanupatkul M, Coppo C, Purnak T, Muratori L, Werner M, Muratori P, Wahlin S. Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome. J Gastroenterol Hepatol 2020

Type

Journal Paper/Review (English)

Journal

J Gastroenterol Hepatol 2020

Publication Date

Aug 13, 2020

Issn Electronic

1440-1746

Brief description/objective

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.