American Journal of Gastroenterology ISSN 0002-9270 C  2005 by Am. Coll. of Gastroenterology doi: 10.1111/j.1572-0241.2005.00270.x Published by Blackwell Publishing CASE REPORT Severe Cholangitis Following Pancreaticoduodenectomy for Pseudotumoral Form of Lymphoplasmacytic Sclerosing Pancreatitis Fr´ ed´ eric Marrache, M.D., 1 Pascal Hammel, M.D., Ph.D., 1 Dermot O’Toole, M.D., Ph.D., 1 Dominique Cazals-Hatem, M.D., 2 Marie Pierre Vullierme, M.D., 3 Annie Sibert, M.D., 3 Philippe Ponsot, M.D., 1 Fr´ ed´ erique Maire, M.D., 1 Olivia Hentic, M.D., 1 Alain Sauvanet, M.D., 4 Philippe L´ evy, M.D., 1 and Philippe Ruszniewski, M.D. 1 1 F´ ed´ eration M´ edico-Chirurgicale d’H´ epato-Gastroent´ erologie, Service de Gatroent´ erologie, 2 Service d’Anatomie et Cytologie pathologiques, 3 Service de Radiologie, and 4 F´ ed´ eration M´ edico-Chirurgicale d’H´ epato-Gastroent´ erologie, Service de Chirurgie digestive, hˆ opital Beaujon, AP-HP, Clichy, France 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. (Am J Gastroenterol 2005;100:2808–2813) INTRODUCTION The majority of cases of chronic pancreatitis are secondary to chronic alcohol abuse and other causes are rare outside of hereditary forms (1). Lymphoplasmacytic sclerosing pan- creatitis affects patients of mid- to elderly age, with few if any symptoms. The latter are usually mild and episodes of acute pancreatitis are rare and diffuse enlargement of the pancreas may be encountered on imaging (2). Associ- ation with Sj¨ ogren’s syndrome, primary sclerosing cholan- gitis, primary biliary cirrhosis, inflammatory bowel disease, and multifocal fibrosclerosis can occur (3–5). Biochemical abnormalities such as hypergammaglobulinemia, elevated serum IgG 4 levels, and/or presence of autoantibodies in- cluding antinuclear, anticarbonic anhydrase II, or antilacto- ferrin have been described (6–9). Typical histological fea- tures consist of narrowing of pancreatic ducts with fibrotic changes and periductular lymphoplasmacytic infiltration and the disease usually improves with the administration of cor- ticosteroids (5, 7, 9). Thus the term of autoimmune pan- creatitis/lymphoplasmacytic sclerosing pancreatitis has been proposed. Lymphoplasmacytic sclerosing pancreatitis is of- ten diagnosed following surgical resection in patients with a disease mimicking a malignant tumor. While the postoper- ative course may be uneventful in many patients, the long- term disease outcome remains controversial (10, 11). Cases of cholangitis associated with lymphoplasmacytic scleros- ing pancreatitis have been previously reported and differ from primary sclerosing cholangitis in that they have been found to improve following steroid therapy (12–14). We re- port observations in 3 patients who underwent pancreatico- duodenectomy for pseudotumoral lymphoplasmacytic scle- rosing pancreatitis in whom severe and diffuse cholangitis occurred several months after surgery. CASES REPORTS Three male patients aged 44, 45, and 70 yr, respectively, underwent pancreaticoduodenectomy for a pseudotumoral form of lymphoplasmacytic sclerosing pancreatitis involving mainly the head of the pancreas. Bilio-enteric continuity was restored using choledocojejunostomy. The immediate post- operative course was uneventful in all 3 patients. At macro- scopic examination, the pancreatic head appeared entirely infiltrated with loss of parenchymal lobulations in all cases (Fig. 1A). The transverse diameter of the pancreatic head measured 5, 5.5, and 7 cm, respectively, without evidence of necrosis, cystic lesions or calculi. The common bile duct was surrounded by fibrosis. Pathological examination showed typical features of lymphoplasmacytic sclerosing pancreatitis and lack of malignant tumor. The histological findings were similar in the 3 cases (Fig. 1B–C) and were characterized by diffuse lymphoplasmacytic infiltration of the pancreas with marked interstitial fibrosis and acinar atrophy. Periductal in- flammation created stenosis or destruction of the pancreatic 2808