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Historia natural de la cirrosis biliar primaria temprana y temprana
J V Metcalf1, H C Mitchison, J M Palmer
1School of Clinical Medical Sciences, University of Newcastle, Newcastle upon Tyne, UK.
Lancet (London, England)
|November 23, 1996
Resumen
Los pacientes con anticuerpos antimitocondriales (AMA) tienen cirrosis biliar primaria (BCP) incluso sin síntomas o pruebas hepáticas anormales. Este estudio de 10 años muestra que la AMA indica la PBC temprana con progresión lenta, confirmando la AMA como un marcador de diagnóstico.
Área de la Ciencia:
- Hepatología Hepatología.
- Inmunología Inmunología.
- Gastroenterología y Gastroenterología.
Sus antecedentes:
- Un informe de 1986 identificó a 29 pacientes con anticuerpos antimitocondriales (AMA), pero con una función hepática normal y sin síntomas.
- La histología hepática en estos pacientes fue diagnóstica o compatible con cirrosis biliar primaria (CBP) en la mayoría de los casos.
- El estudio tenía como objetivo determinar si la AMA indica una BCP muy temprana y evaluar el pronóstico y la progresión de la enfermedad.
Objetivo del estudio:
- Para determinar si la positividad del anticuerpo antimitocondrial (AMA) significa una cirrosis biliar primaria muy temprana (CBP).
- Para evaluar las perspectivas a largo plazo de los pacientes que presentan inicialmente con AMA, pero no hay evidencia clínica o bioquímica de PBC.
- Para monitorear la progresión de la cirrosis biliar primaria (CBP) en esta cohorte durante un período de 10 años.
Principales métodos:
- Evaluaciones clínicas anuales y recopilación de datos bioquímicos en una clínica dedicada a la CBP.
- Revisión de los registros de pacientes, verificación de la causa de la muerte y repetición de la histología hepática según sea necesario.
- Análisis de muestras de suero originales y de seguimiento utilizando ELISA para los componentes del complejo E2 de la piruvato deshidrogenasa.
Principales resultados:
- A lo largo de 17,8 años de seguimiento medio, el 76% desarrolló síntomas de PBC y el 83% mostró colestasis en las pruebas de función hepática.
- Dos pacientes progresaron del grado 1 de Scheuer al grado 2, y dos del grado 1 al grado 3 de PBC.
- Ningún paciente desarrolló cirrosis clínicamente aparente o hipertensión portal; 21 de 27 pruebas de AMA positivas.
Conclusiones:
- La positividad del anticuerpo antimitocondrial (AMA) confirma el diagnóstico de cirrosis biliar primaria (CBP), incluso antes de que aparezcan los signos clínicos o bioquímicos.
- Esto extiende la historia natural conocida de la CBP, lo que indica una fase asintomática potencialmente larga.
- Los pacientes con AMA pero sin otros indicadores de PBC muestran una progresión lenta de la enfermedad, aunque los factores que influyen en el avance siguen sin estar claros.
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