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.

Introduction: Ursodeoxycholic acid (AUDC) is the standard treatment for primary biliary cholangitis (PBC). Prescribing it at an early stage slows the progression of the disease and improves survival. Thus, the biological response to AUDC is considered as the main predictor of survival without liver transplantation. New scores, the Globe-score, and UK-score have recently been validated as the main prognostic factor during PBC. The purpose was to study the association between the Globe-score, UK-Score and the AUDC response during PBC. Patients and Methods: This is a retrospective study of all PBC cases treated by AUDC at a dose of 13-15mg/kg over a 22-year period (January 1998-May 2019). Treatment response was defined by the Paris II criteria at 1 year (a serum alkaline phosphatase (PAL) level 1.5 times the upper limit of normal (LSN), an aspartate aminotransferase (ASAT) level 1.5 times the LSN, normal bilirubinemia). The Globescore and UK-PBC score have been calculated for all our patients. The statistical analysis of the data was done using the SPSS software. The comparison between good treatment responders and non-responders was made using the Chi2 test for qualitative variables and the Mann-Whitney test for quantitative variables. A combination of variables was considered statistically significant if p < 0.05. Results: During the study period, 90 cases of PBC were collected. There were 85 women and 5 men. The average age was 49 12.3. 52.3% patients (n=34) had a complete therapeutic response while 31 patients (47.7%) retained non-response after one year of AUDC treatment. The average Globe score for good responders was-0.62[-0.72;-0.36] and for non-responders was 1.53[1.32; 1.75] with a statistically significant difference (p0.001).