ABSTRACT
La colangitis esclerosante primaria es una enfermedad crónica del hígado de etiología desconocida caracterizada por inflamación y fibrosis del árbol biliar. Provoca una cirrosis biliar e incrementa el riesgo de colangiocarcinoma. Afecta más a hombres en la cuarta década de vida. Debuta con escasos síntomas ó con un cuadro sintomático florido. La forma primaria se asocia a enfermedades inflamatorias intestinales, en el 90 por ciento a la colitis ulcerosa y rara vez a enfermedad de Crohn. Se presenta paciente femenina de 53 años, raza negra y antecedente de salud, que comenzó con coloración amarilla-verdosa de piel y mucosas, orinas oscuras, prurito intenso y cambios del hábito intestinal. Al examinarla se constató íctero y hepatomegalia. Se realizó diagnóstico enzimático, imagenológico, endoscópico e histopatológico de una colangitis esclerosante primaria asociada a una enfermedad de Crohn. El tratamiento medicamentoso con ácido ursodexicólico reportó beneficios, sin embargo, la paciente muere por complicaciones antes del trasplante hepático
Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The disease progresses to cirrhosis and liver failure and cholangiocarcinoma develops in many of patients. The mean age at diagnosis is around 40 years and men are affected more often. The disease started is usually insidious and many patients are asymptomatic at diagnosis or have many symptoms. It is often associated with inflammatory bowel disease, especially ulcerative colitis. A case of black female 53 years old patient, who began to present fatigue, abdominal discomfort, pruritus, weight loss, jaundice and choluria. She develops hepatomegaly. The diagnosis of primary sclerosing cholangitis was made by lab tests, immunological tests, retrograde endoscopic cholangiopancreatography and liver biopsy. Liver transplantation remains the only effective therapeutic option for these kinds of patients with end-stage liver disease, although high dose ursodeoxycholic acid may have a beneficial effect