Search

Auto-immune hepatitis are liver diseases of unknown cause that occur in children and adults of all ages with a female dominance. The spectrum of presentation is very wide, ranging from no symptoms to acute liver failure. The diagnosis is based on high levels of serum globulins, characteristic circulating autoantibodies and histologic abnormalities (necrosis and inflammation). Auto-immune hepatitis is classified on the basis of autoantibody patterns: type 1 (antinuclear and/or smooth muscle antibodies) is the classic form whereas type II (liver-kidney microsome 1 antibody) is much less frequent and occurs mainly in childhood. Mixed forms of auto-immune hepatitis that share features with other putative autoimmune liver diseases, primary biliary cholangitis and primary sclerosing cholangitis, have been described. Because of therapeutic consequences, it is important to distinguish auto-immune hepatitis from other forms of hepatitis and use of diagnostic scoring systems may be helpful. The treatment bases of autoimmune hepatitis have not changed for 30 years. They consist of corticosteroids associated with azathioprine. Budesonide is associated with fewer side effects but should not be used in cirrhosis or very active hepatitis. This is a fast effective but usually only suspensive treatment. Relapse after treatment withdrawal is the rule (80% of cases). The main risk factor of recurrence is the degree of residual inflammation on biopsy. The frequency of side effects justifies an attempt of drug discontinuation provided that criteria of clinical, biochemical and histological remission are achieved after at least 2 years of treatment. [ABSTRACT FROM AUTHOR]

PRIMARY BILIARY CHOLANGITIS Primary biliary cholangitis (formally primary biliary cirrhosis, PBC) is the most common chronic cholestatic liver disease in humans. It is a presumed autoimmune liver disease, characterized by inflammation and progressive destruction of small bile ducts. Without treatment, PBC progresses towards liver fibrosis and, ultimately, cirrhosis. Women older than 40 are more likely affected. Pruritus and tiredness are the most common symptoms but they are frequently lacking. Diagnosis is made by the association of chronic biochemical features of cholestasis (parallel increase in ALP and GGT) and presence of specific auto-antibodies (particularly M2 anti-mitochondrial antibodies). Ursodeoxycholic acid (UDCA), which is the standard of care treatment for PBC, has dramatically improved the prognosis of the disease. However, 30 % to 40 % of patients have an inadequate biochemical response to UDCA and continue to be at high-risk of complications. In this situation, second-line treatments, including obeticholic acid or fibrates, should be considered in association with UDCA., Competing Interests: P.-A. Soret déclare n’avoir aucun lien d’intérêts. O. Chazouillères déclare être consultant pour Intercept, PeptiMimesis Pharma, Genfit, Pliant Therapeutics, et des exposés pour Gilead. C. Corpechot déclare des interventions ponctuelles pour Intercept, CymaBay, Genkyotex, Arrow, et sa prise en charge à l’occasion de déplacements pour congrès, par Intercept, CymaBay, Genkyotex, Biotest, Gilead, AbbVie.

Herein, we report a case of cholangitis with granulocytic epithelial lesion associated with pancreatitis in a 22-year-old patient. The association of bile duct lesions and pancreatitis is usually very suggestive of IgG4 related disease. However, in our case, we found no IgG4 tissue infiltration and we found a granulocytic epithelial on the liver biopsy. Recently, cholangitis with granulocytic epithelial lesion was described in the literature. This entity is identified in 2 % of patients with sclerosing cholangitis. Patients are more likely children or young adults and often have an associated inflammatory bowel disease or rarely a pancreatitis. It is defined by the presence of neutrophilic bile duct lesions on a liver biopsy. Although rare, the diagnosis of cholangitis with granulocytic epithelial lesion is important because of its excellent response to immunosuppressive treatment., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)