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Background: Autoimmune pancreatitis has been designated as sclerosing pancreatocholangitis, because this disease shows a high prevalence of bile-duct lesions. We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas., Methods: Clinical and pathologic findings of 3 patients with immunoglobulin (Ig) G4 related sclerosing cholangitis who had no apparent pancreatic lesions comparable with autoimmune pancreatitis were analyzed., Observations: All patients were middle-aged or elderly individuals with slightly elevated serum IgG4 concentrations and showed long-segment narrowing of the bile-duct system, mimicking infiltrating hilar cholangiocarcinoma without significant pancreatic change. The first patient was treated with a corticosteroid, resulting in amelioration of the narrowing of the bile duct. The second patient underwent surgery based on a diagnosis of cholangiocarcinoma. In the third patient, the bile-duct stricture reversed spontaneously 1 month after the drainage procedure. Pathologic findings of the bile ducts for all patients disclosed significant lymphoplasmacytic infiltration, including abundant IgG4-bearing plasma cells., Conclusions: The use of IgG4 immunostaining in biopsy specimens of the bile duct may identify the presence of corticosteroid-responsive lymphoplasmacytic sclerosing cholangitis.

Background: There are no descriptions of the appearance of the main duodenal papilla in sclerosing pancreatitis. The intent of the present study was to clarify the characteristics of the main duodenal papilla in patients with sclerosing pancreatitis., Methods: Macroscopic findings at ERCP with reference to the main duodenal papilla of 17 patients with sclerosing pancreatitis were compared with those of 24 patients with normal ERCP findings, 11 with chronic pancreatitis, 13 with primary sclerosing cholangitis, 21 with pancreatic cancer, and 18 with bile duct cancer. Endoscopic photographs of the papilla were reviewed retrospectively by 3 observers blinded to the underlying pancreaticobiliary pathology. Degree of swelling was scored in all patients. Biopsy specimens from swollen papillae were assessed histopathologically in 3 patients with sclerosing pancreatitis., Results: Severe swelling of the main duodenal papilla was observed in 7 (41%) of 17 patients with sclerosing pancreatitis. The total score for the degree of swelling in patients with sclerosing pancreatitis was significantly higher than that for patients with a normal ERCP, chronic pancreatitis, primary sclerosing cholangitis, pancreatic cancer, and bile duct cancer (p < 0.01). T-lymphocyte infiltration of the papilla was evident in the biopsies from 3 patients with sclerosing pancreatitis., Conclusions: A swollen main duodenal papilla was a characteristic finding in patients with sclerosing pancreatitis. T-lymphocyte infiltration is present in the swollen main duodenal papilla. These features may be useful in the diagnosis of sclerosing pancreatitis.