Facing the Challenge of Acute Autoimmune Liver Disease: Report of a Case and Review of the Literature

Aikaterini Mastoraki, Evaggelia Papantoni, Ioannis S Papanikolaou, Olympia Kotsilianou, Dimitrios Kanakis, George Sakorafas, Michael Safioleas

Abstract


Autoimmune liver diseases (ALD) comprise two broad categories: those with a hepatic predominance, autoimmune hepatitis (AIH), and those with a predominance of cholestatic features including primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). An overlap syndrome (OS) is thought to exist where a patient appears with clinical features of more than one immune-mediated liver disease. Based on one personal observation we report a case of histologically proven OS. The relevant literature is briefly reviewed. A 68-year-old male was admitted in our department with general malaise, anorexia and jaundice. Trans-abdominal ultrasonography as well as computerized tomography showed a slightly enlarged liver with mild fatty deposition and the patient was submitted to transcutaneous liver biopsy. Histological features included inflammatory infiltration of intra-hepatic bile ducts with the presence of endoepithelial lymphocytes and plasma cells, degenerative distortions and even focal damage of relevant epithelial cellular structures, periductal fibrosis, portal inflammation, interface hepatitis, ductular proliferation and canalicular cholestasis. It is apparent that exact definitions pertaining the OS are limited and general consensus on the precise determinations of overlap/variant syndromes is warranted in order to enable more accurate future research and establish common guidelines in the management of the condition.




doi:10.4021/gr2009.05.1295

Publish ahead of print June 1, 2009


Keywords


Acute autoimmune liver disease; Overlap syndromes; Diagnostic approach; Therapeutic management

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