Primary biliary cirrhosis is a slowly progressive cholestaticautoimmune liver disease that mainly affects middle-aged women with an estimated prevalence rangingfrom 6.7 to 402 cases per million. Hereditary hemorrhagictelangiectasia, or Rendu-Osler-Weber disease,is an autosomal dominant disorder characterized byangiodysplastic lesions (telangiectases and arteriovenousmalformations) that can affect many organs, includingliver, with a prevalence of 1-2 cases per 10000.We describe the coexistence, for the first time to ourknowledge, of these two rare diseases in a 50-year oldCaucasian woman. In this setting, the relevance of anaccurate medical history, the role of liver histology andthe characterization of liver involvement through dynamicimaging techniques can be emphasized.
|Numero di pagine||4|
|Rivista||World Journal of Hepatology|
|Stato di pubblicazione||Published - 2013|
All Science Journal Classification (ASJC) codes
Cabibi, D., Cabibbo, G., Macaluso, F. S., & Maida, M. F. (2013). Primary biliary cirrhosis and hereditary hemorrhagictelangiectasia: When two rare diseases coexist. World Journal of Hepatology, 5, 288-291.