La cholangite à lésion épithéliale granulocytique : une nouvelle entité avec un traitement spécifique

Abstract

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 IgG4related disease. However, in our case, we found no IgG4tissue 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.