Share this content in WeChat
Current situation of imaging diagnosis for IgG4-related sclerosing cholangitis
DONG Lining  HOU Xinmeng  JIN Erhu 

Cite this article as: Dong LN, Hou XM, Jin EH. Current situation of imaging diagnosis for IgG4-related sclerosing cholangitis[J]. Chin J Magn Reson Imaging, 2022, 13(7): 133-137, 159. DOI:10.12015/issn.1674-8034.2022.07.026.

[Abstract] IgG4-related sclerosing cholangitis (IgG4-SC) is the manifestation of IgG4-related disease involving the bile duct system. Patients with IgG4-SC show extrahepatic and/or intrahepatic bile duct stenosis and dilatation in the imaging examination, higher serum IgG4 level in the laboratory test, frequently associated with autoimmune pancreatitis type 1. Pathological examination reveals lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. IgG4-SC responds well to steroid therapy. Because most patients are older and have obstructive jaundice at the initial diagnosis, the diagnosis need to be differentiated from other diseases that cause the bile duct stricture and jaundice, such as cholangiocellular carcinoma and pancreatic cancer. This article introduces the imaging features, diagnostic criteria, main differential diagnoses and disease recurrence of IgG4-SC based on radiologic findings.
[Keywords] IgG4-related sclerosing cholangitis;IgG4-related disease;primary sclerosing cholangitis;autoimmune pancreatitis;obstructive jaundice;diagnosis;X-ray computed tomography;magnetic resonance imaging

DONG Lining   HOU Xinmeng   JIN Erhu*  

Department of Radiology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China

Jin EH, E-mail:

Conflicts of interest   None.

Received  2022-03-12
Accepted  2022-07-05
DOI: 10.12015/issn.1674-8034.2022.07.026
Cite this article as: Dong LN, Hou XM, Jin EH. Current situation of imaging diagnosis for IgG4-related sclerosing cholangitis[J]. Chin J Magn Reson Imaging, 2022, 13(7): 133-137, 159. DOI:10.12015/issn.1674-8034.2022.07.026.

Hara A, Watanabe T, Minaga K, et al. Biomarkers in autoimmune pancreatitis and immunoglobulin G4-related disease[J]. World J Gastroenterol, 2021, 27(19): 2257-2269. DOI: 10.3748/wjg.v27.i19.2257.
Ishikawa T, Kawashima H, Ohno E, et al. Risks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients[J]. J Gastroenterol Hepatol, 2020, 35(12): 2281-2288. DOI: 10.1111/jgh.15163.
Kurita Y, Fujita Y, Sekino Y, et al. IgG4-related sclerosing cholangitis may be a risk factor for cancer[J]. J Hepatobiliary Pancreat Sci, 2021, 28(6): 524-532. DOI: 10.1002/jhbp.957.
Tanaka A, Mori M, Kubota K, et al. Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan[J]. J Hepatobiliary Pancreat Sci, 2020, 27(9): 598-603. DOI: 10.1002/jhbp.793.
Tanaka A, Tazuma S, Okazaki K, et al. Clinical features, response to treatment, and outcomes of IgG4-related sclerosing cholangitis[J]. Clin Gastroenterol Hepatol, 2017, 15(6): 920-926. DOI: 10.1016/j.cgh.2016.12.038.
Chen J, Deshpande V. IgG4-related disease and the liver[J]. Gastroenterol Clin, 2017, 46(2): 195-216. DOI: 10.1016/j.gtc.2017.01.001.
Zen Y. The pathology of IgG4-related disease in the bile duct and pancreas[J]. Semin Liver Dis, 2016, 36(3): 242-256. DOI: 10.1055/s-0036-1584319.
Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease[J]. Mod Pathol, 2012, 25(9): 1181-1192. DOI: 10.1038/modpathol.2012.72.
Ohara H, Nakazawa T, Kawa S, et al. Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort[J]. J Gastroenterol Hepatol, 2013, 28(7): 1247-1251. DOI: 10.1111/jgh.12248.
Boonstra K, Culver EL, de Buy Wenniger LM, et al. Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis[J]. Hepatology, 2014, 59(5): 1954-1963. DOI: 10.1002/hep.26977.
Inoue D, Yoshida K, Yoneda N, et al. IgG4-related disease: dataset of 235 consecutive patients[J/OL]. Medicine (Baltimore), 2015, 94(15) [2022-03-12]. DOI: 10.1097/MD.0000000000000680.
Zen Y, Kawakami H, Kim JH. IgG4-related sclerosing cholangitis: all we need to know[J]. J Gastroenterol, 2016, 51(4): 295-312. DOI: 10.1007/s00535-016-1163-7.
Kato Y, Azuma K, Someda H, et al. Case of IgG4-associated sclerosing cholangitis with normal serum IgG4 concentration, diagnosed by anti-laminin 511-E8 antibody: a novel autoantibody in patients with autoimmune pancreatitis[J]. Gut, 2020, 69(3): 607-609. DOI: 10.1136/gutjnl-2018-317934.
Hubers LM, Vos H, Schuurman AR, et al. Annexin A11 is targeted by IgG4 and IgG1 autoantibodies in IgG4-related disease[J]. Gut, 2018, 67(4): 728-735. DOI: 10.1136/gutjnl-2017-314548.
Nakazawa T, Ohara H, Sano H, et al. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography[J]. Pancreas, 2006, 32(2): 229. DOI: 10.1097/01.mpa.0000202941.85955.07.
Naitoh I, Nakazawa T. Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography in the diagnosis of autoimmune pancreatitis and IgG4-related sclerosing cholangitis[J]. J Med Ultrason (2001), 2021, 48(4): 573-580. DOI: 10.1007/s10396-021-01114-1.
Nakazawa T, Kamisawa T, Okazaki K, et al. Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012)[J]. J Hepatobiliary Pancreat Sci, 2021, 28(3): 235-242. DOI: 10.1002/jhbp.913.
Becq A, Camus M, Arrivé L, et al. IgG4-related sclerosing cholangitis presenting as an isolated intrahepatic stenosis: a rare presentation of a rare disease[J/OL]. Endoscopy, 2021, 53(6) [2022-03-12]. DOI: 10.1055/a-1244-9065.
Zhang BB, Zhang J, Jin EH, et al. CT and magnetic resonance cholangiopancreatography features of IgG4-related sclerosing cholangitis[J]. Chin J Interv Imaging Ther, 2015, 12(6): 336-340. DOI: 10.13929/j.1672-8475.2015.06.004.
Watanabe K, Kamisawa T, Chiba K, et al. Gallbladder wall thickening in patients with IgG4-related diseases, with special emphasis on IgG4-related cholecystitis[J]. Scand J Gastroenterol, 2021, 56(12): 1456-1461. DOI: 10.1080/00365521.2021.1971758.
Lee YC, Chon HK, Choi KH. IgG4-related sclerosing cholangitis involving the gallbladder mimicking a hilar cholangiocarcinoma[J/OL]. Endoscopy, 2022 [2022-03-12]. DOI: 10.1055/a-1778-3393.
Harada Y, Mihara K, Amemiya R, et al. Isolated IgG4-related cholecystitis with localized gallbladder wall thickening mimicking gallbladder cancer: a case report and literature review[J]. BMC Gastroenterol, 2022, 22(1): 99. DOI: 10.1186/s12876-022-02179-z.
Feng YL, Zhang SY, Zheng ZH, et al. Biliary inflammation scoring for immunoglobulin G4-related sclerosing cholangitis: an endoscopic approach with endoscopic ultrasound[J]. Surg Endosc, 2021, 35(12): 7068-7073. DOI: 10.1007/s00464-020-08222-y.
Naitoh I, Nakazawa T, Ohara H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis[J]. J Gastroenterol, 2009, 44(11): 1147-1155. DOI: 10.1007/s00535-009-0108-9.
Zhang BB, Jin EH. MRCP features of pancreatic duct lesions in autoimmune pancreatitis[J]. Chin J Med Imaging Technol, 2017, 33(2): 232-236. DOI: 10.13929/j.1003-3289.201608151.
Wang JN, Zhang J, Yang DW, et al. CT and MRI manifestations of pseudocapsule of autoimmune pancreatitis[J]. Chin J Med Imaging Technol, 2021, 37(9): 1358-1362. DOI: 10.13929/j.issn.1003-3289.2021.09.019.
Chari ST, Smyrk TC, Levy MJ, et al. Diagnosis of autoimmune pancreatitis: the mayo clinic experience[J]. Clin Gastroenterol Hepatol, 2006, 4(8): 1010-1016. DOI: 10.1016/j.cgh.2006.05.017.
Shao CW. Focus on the diagnosis of pancreatic diseases from the perspective of pancreatic duct changes[J]. Chin J Pancreatol, 2021, 21(6): 401-405. DOI: 10.3760/cma.j.cn115667-20211016-00184.
Frøkjær JB, Akisik F, Farooq A, et al. Guidelines for the diagnostic cross sectional imaging and severity scoring of chronic pancreatitis[J]. Pancreatology, 2018, 18(7): 764-773. DOI: 10.1016/j.pan.2018.08.012.
Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis[J]. J Hepatobiliary Pancreat Sci, 2019, 26(1): 9-42. DOI: 10.1002/jhbp.596.
Madhusudhan KS, Das P, Gunjan D, et al. IgG4-related sclerosing cholangitis: a clinical and imaging review[J]. AJR Am J Roentgenol, 2019, 213(6): 1221-1231. DOI: 10.2214/AJR.19.21519.
Mehta TI, Weissman S, Fung BM, et al. Global incidence, prevalence and features of primary sclerosing cholangitis: a systematic review and meta-analysis[J]. Liver Int, 2021, 41(10): 2418-2426. DOI: 10.1111/liv.15007.
Tan LM, Guan XL, Zeng TT, et al. The significance of serum IgG4 and CA19-9, autoantibodies in diagnosis and differential diagnosis of IgG4-related sclerosing cholangitis[J]. Scand J Gastroenterol, 2018, 53(2): 206-211. DOI: 10.1080/00365521.2017.1416159.
Radford-Smith DE, Selvaraj EA, Peters R, et al. A novel serum metabolomic panel distinguishes IgG4-related sclerosing cholangitis from primary sclerosing cholangitis[J]. Liver Int, 2022, 42(6): 1344-1354. DOI: 10.1111/liv.15192.
Zhang W, Dong LL, Zhu J, et al. Chinese expert consensus on the diagnosis and treatment of IgG 4 related diseases[J]. Chin J Intern Med, 2021, 60(3): 192-206. DOI: 10.3760/cma.j.cn112138-20200803-00726.
Hart PA, Topazian MD, Witzig TE, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the mayo clinic experience[J]. Gut, 2013, 62(11): 1607-1615. DOI: 10.1136/gutjnl-2012-302886.
Kemp W, Majeed A, Mitchell J, et al. Management, outcomes and survival of an Australian IgG4-SC cohort: the MOSAIC study[J]. Liver Int, 2021, 41(12): 2934-2943. DOI: 10.1111/liv.15036.
You MW, Kim JH, Byun JH, et al. Relapse of IgG4-related sclerosing cholangitis after steroid therapy: image findings and risk factors[J]. Eur Radiol, 2014, 24(5): 1039-1048. DOI: 10.1007/s00330-014-3127-8.

PREV Primary hepatic carcinosarcoma composed of hepatocellular carcinoma, cholangiocarcinoma and sarcoma: One case report
NEXT Advances in MRI study of brain structure and function changes and related factors of metabolic disorders in Parkinson,s disease

Tel & Fax: +8610-67113815    E-mail: