Noncirrhotic portal hypertension in primary biliary cholangitis with coexisting CREST syndrome.
Clin Res Hepatol Gastroenterol
; 47(5): 102114, 2023 05.
Article
em En
| MEDLINE
| ID: mdl-36967074
ABSTRACT
Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion. Liver biopsy excluded cirrhosis, resulting in a diagnosis of noncirrhotic portal hypertension. This case report describes the pathophysiology of presinusoidal portal hypertension as a rare complication of PBC and its association with coexisiting CREST.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Varizes Esofágicas e Gástricas
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Síndrome CREST
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Derivação Portossistêmica Transjugular Intra-Hepática
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Hipertensão Portal
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Cirrose Hepática Biliar
Tipo de estudo:
Etiology_studies
Limite:
Adult
/
Humans
Idioma:
En
Revista:
Clin Res Hepatol Gastroenterol
Ano de publicação:
2023
Tipo de documento:
Article