Analysis of prognosis and coexistence with other autoimmune diseases in 232 patients with autoimmune liver disease

Abstract

Objective To study the probability of other autoimmune diseases in primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) and explore its effects on the prognosis. Methods From January 1994 to March 2014, the data of 232 patients with autoimmune liver diseases (AILD) were collected. The type and case number of coexisting with other autoimmune diseases of patients with PBC, AIH and PSC were analyzed and compared. Cox regression model was performed to analyze the effects of coexisting with autoimmune diseases on the prognosis of AILD. Results Among 135 PBC patients, there were 64 cases that coexisted with Sjogren's syndrome (SS), seven cases with systemic lupus erythematosus (SLE), seven cases with rheumatoid arthritis (RA), nine cases with systemic sclerosis (SSc), three cases with polymyositis and/or dermatomyositis (PM/DM) and one case with Crohn's disease. Among 55 AIH patients, threre were 19 cases that coexisted with SS, 10 cases with SLE, one case with RA, two cases with SSc and two cases with PM/DM. Among 24 PSC patients, there were seven cases combined with ulceric colitis, one case with Crohn's disease and one case with RA. Among 18 patients with PBC-AIH overlap syndrome, there were five cases with SS and one case with RA. Compared with PBC patients, the risk of pulmonary interstitial fibrosis increased in PBC patiento coexisting with SS (OR=34.0, 95%CI 8.9 to 130.1). After gender, age, disease course and medicine intervention were adjusted, the prognosis of AILD which included death, liver transplantation and liver cirrhosis complications was not affected by the coexistence with other autoimmune diseases. Conclusions AILD patients coexisting with other autoimmune diseases is common, most of which are SS, SLE, SSc and RA. PBC patients coexisting with SS is the risk factor of pulmonary interstitial fibrosis, and coexisting with other autoimmune disease does not independently affect the prognosis of AILD. Key words: Hepatitis, autoimmune; Comorbidity; Autoimmune diseases; Prognosis