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

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.

Clinical significance of concomitant extrahepatic autoimmune disease in patients with autoimmune liver disease

“…These results were compatible with previous reports [8,11,13] but lower than those of the Italian group. Autoimmune thyroid disease was the most frequently observed, with a prevalence of 10% and 15%, [5,8,[11][12][13] which was similar to our results (20%). The differences mostly seen in the pattern of the EAD.…”

“…Among the EAD, autoimmune thyroid disease was most commonly encountered (20%), followed by Sjogren syndrome (12%). This finding is compatible with previous studies, [5,7,8,[11][12][13] which reported a close association between EAD and ALD, and autoimmune thyroid disease was most frequently encountered. Because the association between AIH and EAD is well known, these results indicate that high frequency of concomitant EAD was also seen in patients with OS and PBC.…”

“…Several investigators previously reported the prevalence of EAD in patients with ALD between 28.3% and 43.6%. [8,11,13] In the present study, the overall prevalence of EAD in patients with ALD was 38.6%, whereas it was 43.3% in patients with PBC. These results were compatible with previous reports [8,11,13] but lower than those of the Italian group.…”

“…[8,11,13] In the present study, the overall prevalence of EAD in patients with ALD was 38.6%, whereas it was 43.3% in patients with PBC. These results were compatible with previous reports [8,11,13] but lower than those of the Italian group. Autoimmune thyroid disease was the most frequently observed, with a prevalence of 10% and 15%, [5,8,[11][12][13] which was similar to our results (20%).…”

Diagnosis and Management of Autoimmune Hemolytic Anemia in Patients with Liver and Bowel Disorders

“…The prevalence of AIHA in PBC patients is variable. In a large international cohort of 1554 patients with PBC, AIHA was diagnosed in 0.2% [ 50 ]. In a retrospective study of 71 patients with AIH/PBC overlap syndrome, 43.6% had an extrahepatic autoimmune manifestation and 1.4% developed AIHA [ 51 ].…”