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Inicio Medicina Clínica (English Edition) Primary biliary cholangitis
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Vol. 151. Issue 6.
Pages 242-249 (September 2018)
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Vol. 151. Issue 6.
Pages 242-249 (September 2018)
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DOI: 10.1016/j.medcle.2018.07.009
Primary biliary cholangitis
Colangitis biliar primaria
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Albert Parés
Unidad de Hepatología, Hospital Clínic, Universidad de Barcelona, IDIBAPS, CIBERehd, Barcelona, Spain
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Table 1. Autoimmune diseases associated with primary biliary cholangitis (results expressed as percentage).
Table 2. Biochemical criteria of response to ursodeoxycholic acid.
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Abstract

Primary cholangitis (cirrhosis) is a chronic cholestatic disease with an unquestionable female predominance. It is characterized by inflammation of the small and medium size bile ducts, and can eventually develop to cirrhosis. Most patients remain asymptomatic and are diagnosed by the casual finding of an anicteric biochemical cholestasis with increased alkaline phosphatase. The pathogenesis is unknown and of presumed autoimmune origin in genetic susceptible subjects. M2-type antimitochondrial antibodies, and specific antinuclear antibodies (gp210 and Sp100) are typical and specific of the disease. The positivity of these antibodies and a biochemical cholestasis are sufficient for diagnosis, without the need for liver biopsy. Ursodeoxycholic acid is the specific treatment with an excellent response in more than 60% of patients. When this optimal response is not observed, it can be combined with new agents, but those that have shown to be effective are those that improve cholestasis such as fibrates and obeticholic acid.

Keywords:
Cholestasis
Ursodeoxicolic acid
Pruritus
Osteoporosis
Resumen

La colangitis (cirrosis) biliar primaria es una enfermedad colestásica crónica de predominio claramente femenino. Se caracteriza por una inflamación de los conductos biliares intrahepáticos de pequeño y mediano calibre, y puede progresar hasta una cirrosis. La mayoría de los pacientes permanecen asintomáticos y se diagnostican por el hallazgo fortuito de una colestasis bioquímica anictérica con aumento de fosfatasa alcalina. La patogenia es desconocida y de presunto origen autoinmune en sujetos con una predisposición genética. La presencia de anticuerpos antimitocondriales de tipo M2 y de anticuerpos antinucleares específicos (gp210 y Sp100) es típica de la enfermedad. La positividad de estos anticuerpos y una colestasis bioquímica son suficientes para el diagnóstico, sin necesidad de biopsia hepática. El ácido ursodeoxicólico es el tratamiento específico, con una excelente respuesta en más del 60% de los pacientes. Cuando no se observa esta óptima respuesta se puede combinar con nuevos agentes, pero los que han mostrado eficacia son los que mejoran la colestasis, como los fibratos y el ácido obeticólico.

Palabras clave:
Colestasis
Ácido ursodeoxicólico
Prurito
Osteoporosis

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