An analysis of primary sclerosing cholangitis: a literature review

Abstract

Objective: To analyze new information about Primary Sclerosing Cholangitis (PSC). Bibliographic review: PSC is a rare and multifactorial chronic cholestatic disease of autoimmune etiology with an unknown pathogenesis. It is characterized by chronic lesions of large and small caliber bile stems that promote areas of stenosis and tortuosity in the biliary tree. The clinical manifestations of PSC correlate with the degree of inflammation, fibrosis and destruction of the bile ducts, being asymptomatic in its early stages. The diagnosis is made from the investigation and exclusion of infectious, inflammatory or toxic causes that may be causing serum and radiological changes in routine liver exams. Liver transplantation is the most efficient therapy to reduce mortality and, in some cases, cure the disease. Final considerations: Until new therapies are developed, the knowledge of health professionals about the disease and its early diagnosis, continuous monitoring, prevention and treatment of possible complications remain as one of the main measures to prolong patient survival until performing a liver transplant.