BISPO, M. et al. Colangite Esclerosante Primária: Uma Forma de Apresentação Potencialmente Fatal. []. , 14, 5, pp.236-240. ISSN 0872-8178.

Primary sclerosing cholangitis is an idiopathic disease, characterized by inflammation, progressive sclerosis and obliteration of bile ducts. It is a rare disease, with higher prevalence in males, often associated with ulcerative colitis. The most frequent clinical presentation is a cholestatic picture. Although acute cholangitis is a known complication, it seldom occurs as a form of presentation. The authors describe a case report of a 67 year-old male, who was admitted with sepsis, in a context of probable acute cholangitis. The clinical, laboratory, imaging and histologic data which lead to primary sclerosing cholangitis diagnosis are analyzed. The laboratory finding of markedly elevated CA19.9 associated with cholangitis is emphasized. Some features on the prognosis and follow-up of this disease are reviewed.

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