Primary sclerosing cholangitis (PSC) is a chronic slowly developing cholestatic diseases of liver characterized by non-purulent destructive inflammation and fibrosis of bile ducts leading to secondary biliary cirrhosis. PSC etiology remains unknown and the disease is regarded as an autoimmune pathology. In 60-80% of the patients PSC combines with other autoimmune diseases (over 40 nosological forms). This paper deals with the diversity and prevalence of systemic manifestations of PSC in a group of 93 patients admitted to Tareev Nephrology Clinic. Autoimmune diseases were diagnosed in 79.6% of the patients. PSC was most frequently associated with ulcerative colitis (49.5%), Crohn's disease (17.2%), hemorrhagic vasculitis (8.6%) and autoimmune thyroiditis (7.59%). Nervous system, kidneys, lungs and eyes were affected less frequently. 20.3% of the patients had more than one (2-5) manifestations of PSC in different combinations. It is concluded that the knowledge of extrahepatic PSC symptoms will promote its early diagnosis in patients with cholestasis of uncertain origin.