COMPARATIVE CHARACTERISTICS OF AMAPOSITIVE AND AMA-NEGATIVE PRIMARY BILIARY CHOLANGITIS

Primary biliary cholangitis (PBC, formerly known as primary biliary cirrhosis) is one of the most significant diseases that is predominantly verified in women. The prevalence of PBC is 1.9-40.2 per 100,000 population. Antimitochondrial antibodies (AMA) are of major importance for the diagnosis of primary biliary cirrhosis (PBC), and it has also been suggested that they may be involved in the pathogenesis of the disease. Objective: The aim of this study was to characterize the clinical, biochemical parameters and response to UDCA (Paris I criteria) PBC patients depending on AMA status in the Republic of Kazakhstan. Material and methods. The study was conducted on the basis of the Research Institute of Cardiology and Internal Diseases from 2014 to 2019. A total of 212 patients with primary biliary cholangitis were recorded. Results. Among 212 patients, 171 (80.7%) were AMA-positive and 41(19,3%) - AMA-negative. Vast majority of patients in both groups were Asian (179; 84.4%) and female (206; 97.2%). Severe disease (F3/4) was revealed in 108 (63.2%) AMA-positive and in 18 (43.9 %) AMA-negative patients (p>0.05). AMA-positive and AMA-negative PBC were associated with autoimmune hepatitis in 88 (51.5%) and 10 (24.4%) cases (p<0.01), rheumatoid arthritis in 26 (15.2%) and 5 (12.2%), autoimmune thyroiditis in 35 (20.5%) and 4 (9.8%), vitamin D deficiency in 89 (52.1%) and 19 (46.3%), osteoporosis in 48(28.1%) and 7 (17.1%), gallstone disease in 43 (25.4%) and 8 (21.2%) respectively (p >0.05). Response to ursodeoxycholic acid (UDCA) treatment according to Paris I criteria was noted in 24 out of 81 (29.6%) AMA-positive and in 9 out of 22 (40.9%) AMA-negative patients (p>0.05). Conclusion. In patients with AMA-positive PBC compared with AMA-negative, there is a statistically significant high value of autoimmune hepatitis and autoimmune thyroiditis. Whereas, response to UDCA treatment according to Paris I criteria is higher in AMA-negative PBC.