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1 Imaging Diagnosis of Sclerosing Cholangitis Toshifumi Gabata, M.D., 1) Dai Inoue, M.D., 1) Osamu Matsui, M.D., 1) Hiroki Tatsu, M.D., 2) and Yasunari Fujinaga, M.D. 3) 1) Department of Radiology, Kanazawa University 2) Department of Pathology, Toyama City Hospital 3) Department of Radiology, Shinshu University NICHIDOKU-IHO Vol. 53 No (2008) Summary Sclerosing cholangitis is pathologically classified into two entities: primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis. The differentiation of these two entities is important because IgG4-related cholangitis is well responded to the steroid therapy. In PSC, intrahepatic and extrahepatic bile ducts show stenosis due to wall thickening and dilatation, while in IgG4-related disease, hepatic hilar and extrahepatic bile ducts are mainly involved and frequently associated with (autoimmune) sclerosing pancreatitis. IgG4-related cholangitis is sometimes accompanied by inflammatory pseudotumor of the hepatic hilum mimicking cholangiocarcinoma. Differentiation between hilar cholangiocarcinoma and IgG4-related inflammatory pseudotumor may be obtained by assessing serum IgG4 level as well as tumor markers. Attention needs to be paid to pancreatic lesion (sclerosing pancreatitis). sclerosing cholangitissc SC primary sclerosing cholangitispsc PSC IIV PSC IgG4 SC SC PSC PSCIgG4 SC 17 PSC beaded appearance 12 obtuse angle 2 ERCMRCP
2 ERCPMRCP beaded appearance CT S4
3 MRCP T2T1 MRI MRI CT 23 PSCMRT2 72periportal abnormal intensity T2 146 T1 MRI 8 PSC T1 2 9 PSC PSC PSC
4 LHDRHD CBDNERCPMRCP PSCCTHA PVPH
5 16Zen Y, Harada K, Sasaki M, et al: IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol : , 2004 PSC secondary biliary cirrhosissbc 3 T1 T2 10 superparamagnetic iron oxidespio inflammatory pseudotumorip SC SC sclerosing pancreatitis-associated sclerosing cholangitis SP-SC Zen 17 SC SP-SC 56 SC
6 CT IgG4 SC with hepatic IP 7 PSC SP-SC SC with hepatic IP 2 IgG4 PSC IgG4 ZenSP-SCSC with hepatic IP IgG4
7 FDG-PET T2 T1 MRI T1 MR CBD IgG4
8 CT MRCP IgG4 Zen SC with hepatic IP
9 1418 CT 18 F-FDG-PET CT PSCIgG4 SC 2 = IgG4 SC IgG4 1Revelon G, Rashid A, Kawamoto S, et al: Primary sclerosing cholangitis: MR imaging findigs with pathologic correlation. AJR Am J Roentgenol : , Vitellas KM, Keogan MT, Freed KS, et al: Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics : , Dodd GD 3rd, Baron RL, Oliver JH 3rd, et al: End-stage primary sclerosing cholangitis: CT findings of hepatic morphology in 36 patients. Radiology : , Ito K, Mitchell DG, Outwater EK, et al: Primary sclerosing cholangitis: MR imaging features. AJR Am J Roentgenol : , Fulcher AS, Turner MA, Franklin KJ, et al: Primary sclerosing cholangitis: evaluation with MR cholangiographya case-contdrol study. Radiology : 71-80, Bader TR, Beavers KL, Semelka RC: MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology : , Knowlton JQ, Taylor AJ, Reichelderfer M, et al: Imaging of biliary tract inflammation: an update. AJR Am J Roentgenol : , Gabata T, Matsui O, Kadoya M, et al: Segmental hyperintensity on T1-weighted MRI of he liver: indication of segmental cholestasis. J Magn Reson Imaging : ,1997 9Gabata T, Kadoya M, Matsui O, et al: Intrahepatic biliary calculi: correlation of unusual MR findings with pathologic findigs. Abdom Imaging : , Gabata T, Matsui O, Kadoya M, et al: Giant hyperplasia of the caudate lobe of the cirrhotic liver: correlation with an anomaly of the caudate portal branch. Abdom Imaging : , Nishio T, Toki F, Oyama H, et al: Biliary tract involvement in autoimmune pancreatitis. Pancreas : 76-82, Uehara T, Hamano H, Kawa S, et al: Distinct clinicopathological entity autoimmune pancreatitisassociated sclerosing cholangitis. Pathol Int : , Nakanuma Y, Tsuneyama K, Masuda S, et al: Hepatic inflammatory pseudotumor associated with chronic cholangitis: report of three cases. Human Pathol : 86-91, Nonomura A, Minato H, Shimizu K, et al: Hepatic hilar inflammatory pseudotumor mimicking cholangiocarcinoma with cholangitis and phlebitisa variant of primary sclerosing cholangitis? Pathol Res Pract : , Hamano H, Kawa S, Uehara T, et al: Immunoglobulin G4- related lymphoplasmacytic sclerosing cholangitis that mimics infilrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest Endosc : , Zen Y, Harada K, Sasaki M, et al: IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated
10 sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol : , Zen Y, Fujii T, Sato Y, et al: Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4- related disease. Mod Pathol : , Tublin ME, Moser AJ, Marsh JW, et al: Biliary inflammatory pseudotumor: imaging features in seven patients. AJR Am J Roentgenol : W44-W48, 2007
fiúàÕ53-3†E4_fiÁ‘W02_‚S.indd
Pathological Features of IgG4-related Disease 1) Division of Pathology, Kanazawa University Hospital 2) Department of Human Pathology, Kanazawa University Graduate School of Medicine NICHIDOKU-IHO Vol.
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