Colangite Esclerosante Primária: Uma Forma de Apresentação Potencialmente Fatal

M. Bispo ¹, P. R. Pina ², S. Sousa ², I. Seves ¹, M. L. C. Moura ³, J. P. Graça ²

 

 

Resumo

A colangite esclerosante primária é uma doença idiopática, caracterizada por inflamação, esclerose e obliteração progressiva das vias biliares. É uma doença rara, mais prevalente nos homens e frequentemente associada à colite ulcerosa.

A apresentação clínica habitual é um quadro colestático. Apesar da colangite aguda ser uma complicação conhecida, raramente constitui a forma de apresentação desta doença.

Os autores descrevem o caso clínico de um homem de 67 anos, internado por sépsis, no contexto duma provável colangite aguda. São integrados os dados clínicos, laboratoriais, imagiológicos e histológicos que conduziram ao diagnóstico de colangite esclerosante primária. Salienta-se o achado laboratorial duma elevação significativa do CA19.9 associada à colangite. São ainda revistos alguns aspectos a ter em conta no prognóstico e seguimento desta doença.

 

 

Summary

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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Bibliografia

 

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(1) Serviço de Gastrenterologia.

(2) Serviço de Medicina II.

(3) Serviço de Anatomia Patológica.

Centro Hospitalar de Lisboa Ocidental, E.P.E. – Hospital de Egas Moniz,

Lisboa, Portugal.

 

Correspondência:

Miguel Bispo

Serviço de Gastrenterologia

Centro Hospitalar de Lisboa Ocidental, E.P.E. – Hospital

de Egas Moniz

Rua da Junqueira, 126

1349-019 Lisboa

Tel.: 919 002 599

e-mail: bispomiguel@hotmail.com

Recebido para publicação: 00/00/2007

Aceite para publicação: 00/00/2007