title: Facing the Challenge of Acute Autoimmune Liver Disease: Report of a Case and Review of the Literature.
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.
authors.affiliation: The Fourth Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Mastoraki
authors.affiliation: The Fourth Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: Evaggelia
authors.initials: E
authors.lastname: Papantoni
authors.affiliation: Department of Gastroenterology and Hepatology, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: Ioannis S
authors.initials: IS
authors.lastname: Papanikolaou
authors.affiliation: The Fourth Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: Olympia
authors.initials: O
authors.lastname: Kotsilianou
authors.affiliation: University of Athens, Department of Dermatopathology, 75 Mikras Asias str., 11527, Athens, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Kanakis
authors.affiliation: The Fourth Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: George
authors.initials: G
authors.lastname: Sakorafas
authors.affiliation: The Fourth Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
authors.forename: Michael
authors.initials: M
authors.lastname: Safioleas
keywords: Acute autoimmune liver disease; Diagnostic approach; Overlap syndromes; Therapeutic management