Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?
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.


METHODS
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.
Department of Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
Hideaki
H
Hamano
Shigeyuki
S
Kawa
Takeshi
T
Uehara
Yasuhide
Y
Ochi
Mari
M
Takayama
Kenichi
K
Komatsu
Takashi
T
Muraki
Jun
J
Umino
Kendo
K
Kiyosawa
Shinichi
S
Miyagawa
D000368:Aged; D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology*; D001650:Bile Duct Neoplasms / Q000175:diagnosis*; D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging / Q000473:pathology; D001706:Biopsy; D018281:Cholangiocarcinoma / Q000175:diagnosis*; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000276:immunology*; D003937:Diagnosis, Differential; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging
D000888:Antibodies, Anti-Idiotypic; D007074:Immunoglobulin G