Colangitis esclerosante primaria

Dra. Fabiola Cárdenas Quirós
Médico General. Emergencias Médicas Monteverde. San José, Costa Rica.
Miembro del Colegio de Médicos y Cirujanos de Costa Rica.
Costa Rica.

Dr. Sebastián Rojas Chaves
Médico General. Clínica Dr. Marcial Fallas Díaz. San José, Costa Rica.
Miembro del Colegio de Médicos y Cirujanos de Costa Rica.
Costa Rica.

Dr. Ricardo Álvarez Chaves
Cirujano General. Hospital San Juan de Dios. San José, Costa Rica.
Miembro del Colegio de Médicos y Cirujanos de Costa Rica.
Costa Rica.

Resumen

La colangitis esclerosante primaria (CEP), según estudios realizados fue descrita por primera vez por Delbet en 1924 como una enfermedad hepática crónica caracterizada por presentar fibrosis e inflamación de los conductos biliares intrahepáticos y extrahepáticos (Feldman, Friedman y Heisenger, 2002). La etiopatogenia es desconocida pero se ha visto interacción multifactorial en ella; entre los trastornos de la inmunidad, agentes tóxicos o infecciosos intestinales, deterioro isquémico de los conductos biliares y posiblemente una variación en los transportadores hepatobiliares. En su epidemiología, caracteriza mayor frecuencia de aparición en hombres que en mujeres, con una media de edad de 40 años. Clínicamente puede ser asintomática o con afección de las vías biliares intrahepáticas o asociada a hepatitis autoinmunitaria, en la mayoría de los casos se asocia con colitis y presenta un proceso colestásico crónico que finalmente conduce a una cirrosis biliar. Bioquímicamente muestra una analítica de colestasis, pero se considera de mayor utilidad diagnóstica el uso de la colangiografía retrógrada endoscópica (CPRE) o de la colangiorresonancia (CPRM) como el primer procedimiento ya que es igualmente informativa y no invasiva, mientras que la biopsia hepática no es esencial para el diagnóstico. Respecto al tratamiento de la enfermedad, no existe un fármaco curativo y, a pesar de los intentos con el uso del ácido ursodesoxicólico, solo ha demostrado mejoría en las alteraciones bioquímicas de colestasis, acompañado de un panorama internacional muy controvertido acerca de su utilidad, dejando al trasplante hepático como último recurso terapéutico con buenas expectativas de supervivencia, aunque con una probabilidad de recidiva de la enfermedad en el hígado trasplantado.

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Palabras claves

Colangitis, esclerosante, primaria, colangiocitos, CPRE, CPRM, CEP.

Abstract

Primary sclerosing cholangitis (PSC), according to studies was first described by Delbet in 1924, as a chronic liver disease characterized by fibrosis and inflammation of the intrahepatic and extrahepatic bile ducts (Feldman, Friedman & Sleisenger, 2002). The etiopathogenesis is unknown but it has seen multifactorial interaction in it; among the immunity disorders, intestinal toxic or infectious agents, ischemic deterioration of the bile ducts and possibly a variation in the hepatobiliary transporters. In its epidemiology it characterizes a higher frequency of appearance in men than in women, with an average age of 40 years. Clinically it can be asymptomatic or with intrahepatic bile duct disease or associated with autoimmune hepatitis, in most cases it is associated with colitis and presents a chronic cholestatic process that finally leads to biliary cirrhosis.

Biochemically it shows an analytic of cholestasis, but it does consider the diagnostic utility the use of endoscopic retrograde cholangiography or cholangioresonance as the first procedure that is equally informative and non-invasive, while liver biopsy is not essential for diagnosis. Regarding the treatment of the disease, there is no curative drug, and despite the attempts with the use of ursodeoxycholic acid it has only been shown to be better in the biochemical alterations of cholestasis, accompanying a very controversial international panorama about its usefulness, leaving the liver transplantation as a last therapeutic resource with good survival expectations, although with a probability of recurrence of the disease in the transplanted liver.

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Keywords

Cholangitis, Sclerosing, Primary, Cholangiocytes, ERCP, MRCP, PSC.

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