[Cholangitis with granulocytic epithelial lesion: a new entity with a specific treatment]

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.