Autoimmune Diseases Associated with Primary Biliary Cholangitis

Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease; the autoimmune mechanism seems the most likely. As a result, PBC is frequently associated with other autoimmune diseases. The goal of our work is to determine the prevalence and type of autoimmune diseases associated with PBC and to assess their impact on the prognosis of the disease. Materials and methods: This is a retrospective study over a period of 22 years (1998-2019) including all patients followed for CBP. In all these patients, we systematically looked for: autoimmune hepatitis, dysthyroidism, and type 1 diabetes, dry syndrome, and celiac disease, dermatological and joint damage. The statistical analysis of the data was done using the SPSS software. The comparison of the biochemical response to ursodeoxycholic acid between patients with isolated CBP and those with CBP associated with autoimmune pathology was performed using the Chi2 test. A combination of variables was considered statistically significant if p < 0.05. Results: 90 patients (85 women and 5 men) followed for CBP were collected. The average age was 49+/-12.3 years. Of these patients, 36 patients (42.9%) had an autoimmune disease associated with PBC. The discovery of these autoimmune diseases preceded the diagnosis of PBC in 9 cases (27.3%) and was concomitant in the remaining cases. Autoimmune hepatitis was found in 10 patients (12%), defining an overlapping syndrome. Other diseases were Hashimoto's thyroiditis (n = 9), basedow (n=1) dry syndrome (n = 10), celiac disease (n = 3), insulin-dependent diabetes (n = 2), systemic scleroderma (n = 1), rheumatoid arthritis (n = 1), Addisson disease (n=1) Psoriasis (n = 1) vitiligo (n = 1). The comparison of the biochemical response to ursodeoxycholic acid between patients with isolated CBP and those with CBP associated with autoimmune pathology was statistically non-significant with p=0.67. Conclusion: In our series, the prevalence of autoimmune diseases associated with PBC was 40%. These diseases were dominated by autoimmune hepatitis, Hashimoto's thyroiditis and dry syndrome. Although their association does not appear to alter the prognosis for CBP or the response to AUDC, their screening must be systematic in order to initiate early and appropriate treatment. Although ursodeoxycholic acid is beneficial in the treatment of primary biliary cirrhosis, it has had no measurable effect on the autoimmune disorders associated with the disease.