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Autoimmune liver disease
Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the three major autoimmune liver diseases. So-called overlap syndromes are diseases exhibiting ...
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Michael P. Manns
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641-642
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The benefit of animal models for autoimmune hepatitis
Autoimmune hepatitis (AIH) is a chronic liver disease which is normally recognized during late stage of the disease. Due to limited knowledge about the onset and course of disease and need for chronic...
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Elmar Jaeckel,
Matthias Hardtke-Wolenski,
Katja Fischer
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643-651
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Pathogenesis of autoimmune hepatitis
The mechanisms underlying the pathogenesis of autoimmune hepatitis are not fully understood, though there is growing evidence that genetic predisposition, molecular mimicry and/or impairment of regula...
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Rodrigo Liberal,
Maria Serena Longhi,
Giorgina Mieli-Vergani,
Diego Vergani
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653-664
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Diagnostic criteria for autoimmune hepatitis
The clinical spectrum of autoimmune hepatitis is very wide. In addition, autoimmune hepatitis can present in any age group. Diagnosis is usually made by a combination of clinical, laboratory and histo...
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Ansgar W. Lohse,
Christiane Wiegard
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665-671
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Therapy of autoimmune hepatitis
Autoimmune hepatitis was the first chronic liver disease with a favourable response to drug therapy and a dismal prognosis when left untreated. Since its original description in 1950 and first treatme...
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Christian P. Strassburg,
Michael P. Manns
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673-687
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Autoimmune hepatitis in special patient populations
Autoimmune hepatitis has diverse clinical phenotypes that challenge conventional diagnostic criteria and treatment strategies. The goals of this review are to characterize these special populations an...
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Albert J. Czaja
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689-700
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Diagnosis of primary biliary cirrhosis
Primary biliary cirrhosis is the archetypal autoimmune liver disease, with the disease label describing a chronic granulomatous lymphocytic small bile duct cholangitis, which now most commonly present...
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Gideon M. Hirschfield
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701-712
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Genetics in primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is a chronic and severe inflammatory disease leading to fibrotic bile duct destruction and in most cases liver cirrhosis. As in other complex genetic diseases, the...
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Trine Folseraas,
Espen Melum,
Andre Franke,
Tom H. Karlsen
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713-726
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Pathogenesis of primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. ...
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Marion J. Pollheimer,
Emina Halilbasic,
Peter Fickert,
Michael Trauner
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727-739
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Medical and endoscopic therapy of primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease mainly affecting young male patients. PSC is characterised by chronic inflammation and fibrotic strictures of the intra- and ex...
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Tobias J. Weismüller,
Tim O. Lankisch
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741-752
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Primary sclerosing cholangitis and malignancy
Cholangiocarcinoma complicates primary sclerosing cholangitis (PSC) in approximately 10% of cases, but no risk factor that can identify this subgroup of patients is known. No imaging modalities or ser...
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Kirsten Muri Boberg,
Guro E. Lind
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753-764
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Liver transplantation in autoimmune liver diseases
Liver transplantation is indicated for terminal phases of autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. Indications for transplantation in autoimmune liver diseas...
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Jawad A. Ilyas,
Christine A. O’Mahony,
John M. Vierling
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765-782
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Autoimmune liver diseases in children – What is different from adulthood?
Autoimmune liver disorders in childhood include autoimmune hepatitis, autoimmune sclerosing cholangitis and de novo autoimmune hepatitis after liver transplant. These inflammatory liver disorders are ...
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Giorgina Mieli-Vergani,
Diego Vergani
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783-795
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