Primary biliary cirrhosis is a slowly progressive cholestatic autoimmune liver disease that mainly affects middle- aged women with an estimated prevalence ranging from 6.7 to 402 cases per million. Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an autosomal dominant disorder characterized by angiodysplastic lesions (telangiectases and arteriovenous malformations) that can affect many organs, including liver, with a prevalence of 1-2 cases per 10000. We describe the coexistence, for the first time to our knowledge, of these two rare diseases in a 50-year old Caucasian woman. In this setting, the relevance of an accurate medical history, the role of liver histology and the characterization of liver involvement through dynamic imaging techniques can be emphasized.

Macaluso, F.S., Maida, M.F., Nicola, A., Cabibbo, G., & Cabibi, D. (2013). Primary biliary cirrhosis and hereditary hemorrhagic telangiectasia: When two rare diseases coexist. WORLD JOURNAL OF HEPATOLOGY, 5(5), 288-291 [http://dx.doi.org/10.4254/wjh.v5.i5.288].

Primary biliary cirrhosis and hereditary hemorrhagic telangiectasia: When two rare diseases coexist

MACALUSO, Fabio Salvatore;MAIDA, Marcello Fabio;CABIBBO, Giuseppe;CABIBI, Daniela
2013

Abstract

Primary biliary cirrhosis is a slowly progressive cholestatic autoimmune liver disease that mainly affects middle- aged women with an estimated prevalence ranging from 6.7 to 402 cases per million. Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an autosomal dominant disorder characterized by angiodysplastic lesions (telangiectases and arteriovenous malformations) that can affect many organs, including liver, with a prevalence of 1-2 cases per 10000. We describe the coexistence, for the first time to our knowledge, of these two rare diseases in a 50-year old Caucasian woman. In this setting, the relevance of an accurate medical history, the role of liver histology and the characterization of liver involvement through dynamic imaging techniques can be emphasized.
Settore MED/08 - Anatomia Patologica
Settore MED/12 - Gastroenterologia
Macaluso, F.S., Maida, M.F., Nicola, A., Cabibbo, G., & Cabibi, D. (2013). Primary biliary cirrhosis and hereditary hemorrhagic telangiectasia: When two rare diseases coexist. WORLD JOURNAL OF HEPATOLOGY, 5(5), 288-291 [http://dx.doi.org/10.4254/wjh.v5.i5.288].
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10447/73297
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