[Primary fibrosing cholangitis (author's transl)]

Primary fibrosing cholangitis is a rare and grave disorder. It consists of an inflammatory stenosis, localised or extensive, of the extra or intra-hepatic system, with intramural fibrous thickening, leaving the mucosa and the serosa undamaged. It has many similar features to primary biliary cirrhosis, and would seem to be the same response of the biliary system to an agression which remains of unknown aetiology. The resection of localised stenoses, bilio-digestive by-pass procedures or external drainage of the common duct can only delay progression towards fatal cholangitis.