The Ministry of Health included primary biliary cholangitis in the list of rare diseases

The Ministry of Health has included primary biliary cholangitis (PBC) in the list of rare (orphan) diseases, follows from the list published by the department.

It is listed as a separate nosological form with the code K74.3. The former name of the disease is primary biliary cirrhosis.

PCB is an autoimmune cholestatic liver disease in which small bile ducts are affected. The disease has a chronic progressive course and can lead to fibrosis, and at a late stage to cirrhosis and liver failure.

Orphan diseases are considered in Russia, the prevalence of which does not exceed ten cases per 100 thousand people. The official list of such diseases is maintained by the Ministry of Health.

For some life-threatening and chronic progressive rare diseases, a separate mechanism of drug provision operates. In November 2025, the government approved the criteria by which regions can receive additional funds for the purchase of drugs if they do not cope with such costs on their own.