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Báo cáo y học: "Autoimmune liver disease - are there spectra that we do not know"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Critical Care giúp cho các bạn có thêm kiến thức về ngành y học đề tài: Autoimmune liver disease - are there spectra that we do not know? | Fallatah and Akbar Comparative Hepatology 2011 10 9 http www.comparative-hepatology.eom content 10 1 9 COMPARATIVE HEPATOLOGY CASE REPORT Open Access Autoimmune liver disease - are there spectra that we do not know Hind I Fallatah and Hisham O Akbar Abstract Autoimmune liver diseases AILDs are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis AIH is the typical example of hepatocellular AILD but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis PBC is the second most common AILD with a cholestatic presentation and characterized by positive antimitochondrial antibody AMA . It has an excellent response and long term outcome with the administration of ursodeoxycholic acid UDCA . Another AILD that is thought to be a variant of PBC is the autoimmune cholangitis being a disease that has biochemical and histological features similar to PBC but the AMA is negative. Primary sclerosing cholangitis PSC is a rare entity of AILD that has a cholestatic presentation and respond poorly to the treatment with the ultimate progression to advance liver cirrhosis in most patients. Other forms of AILD include the overlap syndromes OS which are diseases with mixed immunological and histological patterns of two AILD the most commonly recognized one is AIH-PBC overlap AIH-PSC overlap is less common . The treatment of OS involves the trial of UDCA and different immunosuppressants. Here we present three case reports of unusual forms of chronic liver diseases that most likely represent AILD. The first two patients had a cholestatic picture whereas the third one had a hepatocellular picture at presentation. We discussed their biochemical immunological and histological features as .