title: [Systemic manifestations of primary sclerosing cholangitis].
abstract: 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.
authors.forename: E A
authors.initials: EA
authors.lastname: Aleksandrova
authors.forename: E Z
authors.initials: EZ
authors.lastname: Burnevich
authors.forename: E A
authors.initials: EA
authors.lastname: Arion
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D015551:Autoimmunity / Q000276:immunology*; D002648:Child; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000453:epidemiology* / Q000276:immunology; D018450:Disease Progression; D016145:Endoscopy, Digestive System; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012426:Russia / Q000453:epidemiology; D055815:Young Adult