Severe cholangitis following pancreaticoduodenectomy for pseudotumoral form of lymphoplasmacytic sclerosing pancreatitis

Cholangitis associated with lymphoplasmacytic sclerosing pancreatitis may occur simultaneously or following diagnosis of pancreatitis. The natural history following inappropriate pancreatic surgery and treatment of cholangitis in this setting are ill-defined. Three patients underwent pancreaticoduodenectomy for pseudotumoral lymphoplasmacytic sclerosing pancreatitis. Jaundice or ascending cholangitis revealed severe biliary strictures at 1, 6, and 11 months, respectively, following surgery. Treatment combining corticosteroids with or without biliary stenting was efficacious in all patients. One patient with subsequent clinical and morphological relapse responded well to reintroduction of steroids. Biliary changes appeared to be immune-related based on pathological examination and response to corticosteroids.