Update on Epidemiology of IgG4-related Disease Involving the Liver and Pancreas
Main Article Content
Abstract
IgG4-related disease (IgG4-RD) is a rare condition characterized by an immune-mediated fibro-inflammation affecting various organs (liver, pancreas, heart, kidney, brain among others) with peculiar histopathologic feature. Few epidemiological data have been published so far, although a dramatic increase in the number of patients diagnosed with IgG4-RD has been recorded in the last years. The clinical manifestations of IgG4-RD involve frequently liver and pancreas. Specifically, a crucial challenge in differential diagnosis is IgG4-related sclerosing cholangitis which is frequently accompanied by pancreatic involvement. Inflammatory alterations of liver parenchyma have also been described, with a new nosology of IgG4-autoimmune hepatitis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-RD. The first-ever epidemiological study to estimate the point prevalence of IgG4-related sclerosing cholangitis has been recently conducted in Japan. Moreover, several demographic studies on IgG4-RD involving liver and pancreas have been published in other countries, although the majority of them are cohort studies and data on incidence/prevalence are lacking. This review aims to update the recent epidemiological and clinical knowledge of IgG4-RD involving liver and pancreas, focusing also on the risk of malignancy.
Article Details
How to Cite
FLOREANI, Annarosa; GABBIA, Daniela; MARTIN, Sara De.
Update on Epidemiology of IgG4-related Disease Involving the Liver and Pancreas.
Medical Research Archives, [S.l.], v. 11, n. 2, feb. 2023.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/3451>. Date accessed: 30 dec. 2024.
doi: https://doi.org/10.18103/mra.v11i2.3451.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
Amarapurkar, A. D., & Amarapurkar, D. N. (2015). Immunoglobulin IgG4 and autoimmune hepatitis. Tropical Gastroenterology: Official Journal of the Digestive Diseases Foundation, 36(2), 112–117. https://doi.org/10.7869/tg.263
Arase, Y., Matsumoto, K., Anzai, K., Tsuruya, K., Sugiyama, S., Yoshihara, S., Hirose, S., Uojima, H., Hidaka, H., Nakazawa, T., Deguchi, R., Kojima, S., Takashimizu, S., Shiraishi, K., Shirai, T., & Kagawa, T. (2021). Clinicopathological Features of Autoimmune Hepatitis with IgG4-Positive Plasma Cell Infiltration. Digestive Diseases (Basel, Switzerland), 39(3), 225–233. https://doi.org/10.1159/000510562
Aydemir, Y., Akcoren, Z., Demir, H., Saltik Temizel, I. N., Ozen, H., & Yuce, A. (2019). Clinical and histopathological features of immunoglobulin G4-associated autoimmune hepatitis in children. Journal of Gastroenterology and Hepatology, 34(4), 742–746. https://doi.org/10.1111/jgh.14525
Backhus, J., Neumann, C., Perkhofer, L., Schulte, L. A., Mayer, B., Seufferlein, T., Müller, M., & Kleger, A. (2021). A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab. Journal of Clinical Medicine, 10(6), 1329. https://doi.org/10.3390/jcm10061329
Canivet, C. M., Anty, R., Patouraux, S., Saint-Paul, M.-C., Lebeaupin, C., Gual, P., Duclos-Vallee, J.-C., & Tran, A. (2016). Immunoglobulin G4-associated autoimmune hepatitis may be found in Western countries. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 48(3), 302–308. https://doi.org/10.1016/j.dld.2015.10.005
Carballo, I., González-Quintela, A., Sopeña, B., & Vidal, C. (2021). Immunoglobulin G4–Related Disease: What an Allergist Should Know. Journal of Investigational Allergology and Clinical Immunology, 31(3), 212–227. https://doi.org/10.18176/jiaci.0633
Chari, S. T., Smyrk, T. C., Levy, M. J., Topazian, M. D., Takahashi, N., Zhang, L., Clain, J. E., Pearson, R. K., Petersen, B. T., Vege, S. S., & Farnell, M. B. (2006). Diagnosis of autoimmune pancreatitis: The Mayo Clinic experience. Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association, 4(8), 1010–1016; quiz 934. https://doi.org/10.1016/j.cgh.2006.05.017
Chung, H., Watanabe, T., Kudo, M., Maenishi, O., Wakatsuki, Y., & Chiba, T. (2010). Identification and characterization of IgG4-associated autoimmune hepatitis. Liver International: Official Journal of the International Association for the Study of the Liver, 30(2), 222–231. https://doi.org/10.1111/j.1478-3231.2009.02092.x
Deshpande, V. (2015). IgG4-Related Disease of the Gastrointestinal Tract: A 21st Century Chameleon. Archives of Pathology & Laboratory Medicine, 139(6), 742–749. https://doi.org/10.5858/arpa.2014-0181-RA
Deshpande, V., Zen, Y., Chan, J. K., Yi, E. E., Sato, Y., Yoshino, T., Klöppel, G., Heathcote, J. G., Khosroshahi, A., Ferry, J. A., Aalberse, R. C., Bloch, D. B., Brugge, W. R., Bateman, A. C., Carruthers, M. N., Chari, S. T., Cheuk, W., Cornell, L. D., Fernandez-Del Castillo, C., … Stone, J. H. (2012). Consensus statement on the pathology of IgG4-related disease. Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc, 25(9), 1181–1192. https://doi.org/10.1038/modpathol.2012.72
Ghazale, A., & Chari, S. T. (2007). Optimising corticosteroid treatment for autoimmune pancreatitis. Gut, 56(12), 1650–1652. https://doi.org/10.1136/gut.2007.129833
Ghazale, A., Chari, S. T., Zhang, L., Smyrk, T. C., Takahashi, N., Levy, M. J., Topazian, M. D., Clain, J. E., Pearson, R. K., Petersen, B. T., Vege, S. S., Lindor, K., & Farnell, M. B. (2008). Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy. Gastroenterology, 134(3), 706–715. https://doi.org/10.1053/j.gastro.2007.12.009
Hart, P. A., Law, R. J., Dierkhising, R. A., Smyrk, T. C., Takahashi, N., & Chari, S. T. (2014). Risk of cancer in autoimmune pancreatitis: A case-control study and review of the literature. Pancreas, 43(3), 417–421. https://doi.org/10.1097/MPA.0000000000000053
Hirano, K., Tada, M., Sasahira, N., Isayama, H., Mizuno, S., Takagi, K., Watanabe, T., Saito, T., Kawahata, S., Uchino, R., Hamada, T., Miyabayashi, K., Mohri, D., Sasaki, T., Kogure, H., Yamamoto, N., Nakai, Y., Yoshida, H., Ito, Y., … Koike, K. (2014). Incidence of malignancies in patients with IgG4-related disease. Internal Medicine (Tokyo, Japan), 53(3), 171–176. https://doi.org/10.2169/internalmedicine.53.1342
Huggett, M. T., Culver, E. L., Kumar, M., Hurst, J. M., Rodriguez-Justo, M., Chapman, M. H., Johnson, G. J., Pereira, S. P., Chapman, R. W., Webster, G. J. M., & Barnes, E. (2014). Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. The American Journal of Gastroenterology, 109(10), 1675–1683. https://doi.org/10.1038/ajg.2014.223
Ikeura, T., Miyoshi, H., Uchida, K., Fukui, T., Shimatani, M., Fukui, Y., Sumimoto, K., Matsushita, M., Takaoka, M., & Okazaki, K. (2014). Relationship between autoimmune pancreatitis and pancreatic cancer: A single-center experience. Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 14(5), 373–379. https://doi.org/10.1016/j.pan.2014.04.029
Kamisawa, T., Funata, N., Hayashi, Y., Eishi, Y., Koike, M., Tsuruta, K., Okamoto, A., Egawa, N., & Nakajima, H. (2003). A new clinicopathological entity of IgG4-related autoimmune disease. Journal of Gastroenterology, 38(10), 982–984. https://doi.org/10.1007/s00535-003-1175-y
Kamisawa, T., Tsuruta, K., Okamoto, A., Horiguchi, S., Hayashi, Y., Yun, X., Yamaguchi, T., & Sasaki, T. (2009). Frequent and significant K-ras mutation in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis. Pancreas, 38(8), 890–895. https://doi.org/10.1097/MPA.0b013e3181b65a1c
Kastin, D., Siegel, M., Anderson, R., & Aronsohn, A. (2021). IgG 4 Autoimmune Hepatitis Presenting as Idiopathic Hypereosinophilia Syndrome. Hepatology (Baltimore, Md.), 73(4), 1615–1617. https://doi.org/10.1002/hep.31492
Kemp, W., Majeed, A., Mitchell, J., Majumdar, A., Tse, E., Skoien, R., Croagh, D., Dev, A., Gao, H., Weltman, M., Craig, P., Stuart, K., Cheng, W., Edmunds, S., Lee, E., Sood, S., Metz, A., Thompson, A., Sinclair, M., … Roberts, S. K. (2021). Management, outcomes and survival of an Australian IgG4-SC cohort: The MOSAIC study. Liver International: Official Journal of the International Association for the Study of the Liver, 41(12), 2934–2943. https://doi.org/10.1111/liv.15036
Kim, K.-P., Kim, M.-H., Kim, J. C., Lee, S. S., Seo, D. W., & Lee, S. K. (2006). Diagnostic criteria for autoimmune chronic pancreatitis revisited. World Journal of Gastroenterology, 12(16), 2487–2496. https://doi.org/10.3748/wjg.v12.i16.2487
Lanzillotta, M., Della-Torre, E., Wallace, Z. S., Stone, J. H., Karadag, O., Fernández-Codina, A., Arcidiacono, P. G., Falconi, M., Dagna, L., & Capurso, G. (2021). Efficacy and safety of rituximab for IgG4-related pancreato-biliary disease: A systematic review and meta-analysis. Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 21(7), 1395–1401. https://doi.org/10.1016/j.pan.2021.06.009
Löhr, J.-M., Vujasinovic, M., Rosendahl, J., Stone, J. H., & Beuers, U. (2022). IgG4-related diseases of the digestive tract. Nature Reviews. Gastroenterology & Hepatology, 19(3), 185–197. https://doi.org/10.1038/s41575-021-00529-y
Minaga, K., Watanabe, T., Chung, H., & Kudo, M. (2019). Autoimmune hepatitis and IgG4-related disease. World Journal of Gastroenterology, 25(19), 2308–2314. https://doi.org/10.3748/wjg.v25.i19.2308
Naitoh, I., Kamisawa, T., Tanaka, A., Nakazawa, T., Kubota, K., Takikawa, H., Unno, M., Masamune, A., Kawa, S., Nakamura, S., Okazaki, K., & collaborators. (2021). Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 53(10), 1308–1314. https://doi.org/10.1016/j.dld.2021.02.009
Naitoh, I., & Nakazawa, T. (2022). Classification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis. Gut and Liver, 16(1), 28–36. https://doi.org/10.5009/gnl210116
Nakanuma, Y., Ishizu, Y., Zen, Y., Harada, K., & Umemura, T. (2016). Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease. Seminars in Liver Disease, 36(3), 229–241. https://doi.org/10.1055/s-0036-1584320
Nakazawa, T., Kamisawa, T., Okazaki, K., Kawa, S., Tazuma, S., Nishino, T., Inoue, D., Naitoh, I., Watanabe, T., Notohara, K., Kubota, K., Ohara, H., Tanaka, A., Takikawa, H., Masamune, A., & Unno, M. (2021). Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012). Journal of Hepato-Biliary-Pancreatic Sciences, 28(3), 235–242. https://doi.org/10.1002/jhbp.913
Nakazawa, T., Ohara, H., Sano, H., Ando, T., & Joh, T. (2006). Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas, 32(2), 229. https://doi.org/10.1097/01.mpa.0000202941.85955.07
Okamoto, A., Watanabe, T., Kamata, K., Minaga, K., & Kudo, M. (2019). Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis. Internal Medicine, 58(11), 1533–1539. https://doi.org/10.2169/internalmedicine.2210-18
Okazaki, K. (2019). Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment. Digestive Diseases and Sciences, 64(9), 2385–2394. https://doi.org/10.1007/s10620-019-05746-9
Okazaki, K., Kawa, S., Kamisawa, T., Naruse, S., Tanaka, S., Nishimori, I., Ohara, H., Ito, T., Kiriyama, S., Inui, K., Shimosegawa, T., Koizumi, M., Suda, K., Shiratori, K., Yamaguchi, K., Yamaguchi, T., Sugiyama, M., & Otsuki, M. (2006). Clinical diagnostic criteria of autoimmune pancreatitis: Revised proposal. Journal of Gastroenterology, 41(7), 626. https://doi.org/10.1007/s00535-006-1868-0
Otsuki, M., Chung, J. B., Okazaki, K., Kim, M.-H., Kamisawa, T., Kawa, S., Park, S. W., Shimosegawa, T., Lee, K., Ito, T., Nishimori, I., Notohara, K., Naruse, S., Ko, S. B. H., Kihara, Y., & Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labour and Welfare of Japan and the Korean Society of Pancreatobiliary Diseases. (2008). Asian diagnostic criteria for autoimmune pancreatitis: Consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. Journal of Gastroenterology, 43(6), 403–408. https://doi.org/10.1007/s00535-008-2205-6
Rebours, V., & Lévy, P. (2020). Pancreatic and biliary tract involvement in IgG4-related disease. Presse Medicale (Paris, France: 1983), 49(1), 104015. https://doi.org/10.1016/j.lpm.2020.104015
Shimosegawa, T., Chari, S. T., Frulloni, L., Kamisawa, T., Kawa, S., Mino-Kenudson, M., Kim, M.-H., Klöppel, G., Lerch, M. M., Löhr, M., Notohara, K., Okazaki, K., Schneider, A., & Zhang, L. (2011). International Consensus Diagnostic Criteria for Autoimmune Pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas, 40(3), 352–358. https://doi.org/10.1097/MPA.0b013e3182142fd2
Shiokawa, M., Kodama, Y., Yoshimura, K., Kawanami, C., Mimura, J., Yamashita, Y., Asada, M., Kikuyama, M., Okabe, Y., Inokuma, T., Ohana, M., Kokuryu, H., Takeda, K., Tsuji, Y., Minami, R., Sakuma, Y., Kuriyama, K., Ota, Y., Tanabe, W., … Chiba, T. (2013). Risk of cancer in patients with autoimmune pancreatitis. The American Journal of Gastroenterology, 108(4), 610–617. https://doi.org/10.1038/ajg.2012.465
Smit, W. L., Culver, E. L., & Chapman, R. W. (2016). New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis. Clinics in Liver Disease, 20(1), 47–65. https://doi.org/10.1016/j.cld.2015.08.004
Stone, J. H., Zen, Y., & Deshpande, V. (2012). IgG4-related disease. The New England Journal of Medicine, 366(6), 539–551. https://doi.org/10.1056/NEJMra1104650
Sumimoto, K., Uchida, K., Ikeura, T., Hirano, K., Yamamoto, M., Takahashi, H., Nishino, T., Mizushima, I., Kawano, M., Kamisawa, T., Saeki, T., Maguchi, H., Ushijima, T., Shiokawa, M., Seno, H., Goto, H., Nakamura, S., Okazaki, K., & Research Committee for an Intractable Disease of IgG4-related disease. (2022). Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan. Journal of Gastroenterology and Hepatology, 37(6), 1022–1033. https://doi.org/10.1111/jgh.15809
Tanaka, A., Mori, M., Kubota, K., Naitoh, I., Nakazawa, T., Takikawa, H., Unno, M., Kamisawa, T., Kawa, S., & Okazaki, K. (2020). Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan. Journal of Hepato-Biliary-Pancreatic Sciences, 27(9), 598–603. https://doi.org/10.1002/jhbp.793
Tanaka, A., & Notohara, K. (2021). Immunoglobulin G4 (IgG4)-related autoimmune hepatitis and IgG4-hepatopathy: A histopathological and clinical perspective. Hepatology Research: The Official Journal of the Japan Society of Hepatology, 51(8), 850–859. https://doi.org/10.1111/hepr.13683
Tanaka, A., Tazuma, S., Okazaki, K., Nakazawa, T., Inui, K., Chiba, T., & Takikawa, H. (2017). Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis. Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association, 15(6), 920-926.e3. https://doi.org/10.1016/j.cgh.2016.12.038
Uchida, K., Masamune, A., Shimosegawa, T., & Okazaki, K. (2012). Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009. International Journal of Rheumatology, 2012, 358371. https://doi.org/10.1155/2012/358371
Umehara, H., Okazaki, K., Kawa, S., Takahashi, H., Goto, H., Matsui, S., Ishizaka, N., Akamizu, T., Sato, Y., Kawano, M., & Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. (2021). The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Modern Rheumatology, 31(3), 529–533. https://doi.org/10.1080/14397595.2020.1859710
Umemura, T., Zen, Y., Hamano, H., Ichijo, T., Kawa, S., Nakanuma, Y., & Kiyosawa, K. (2007). IgG4 associated autoimmune hepatitis: A differential diagnosis for classical autoimmune hepatitis. Gut, 56(10), 1471–1472. https://doi.org/10.1136/gut.2007.122283
Umemura, T., Zen, Y., Hamano, H., Joshita, S., Ichijo, T., Yoshizawa, K., Kiyosawa, K., Ota, M., Kawa, S., Nakanuma, Y., & Tanaka, E. (2011). Clinical significance of immunoglobulin G4-associated autoimmune hepatitis. Journal of Gastroenterology, 46 Suppl 1, 48–55. https://doi.org/10.1007/s00535-010-0323-4
Vlachou, P. A., Khalili, K., Jang, H.-J., Fischer, S., Hirschfield, G. M., & Kim, T. K. (2011). IgG4-related sclerosing disease: Autoimmune pancreatitis and extrapancreatic manifestations. Radiographics: A Review Publication of the Radiological Society of North America, Inc, 31(5), 1379–1402. https://doi.org/10.1148/rg.315105735
Wallace, Z., Miles, G., Smolkina, E. D., Petruski-Ivleva, N., Madziva, D., Cook, C., Fu, X., Zhang, Y., Ttone, john, & Choi, H. (2022). The Epidemiology, Clinical Outcomes, and Healthcare Resource Utilization of IgG4-Related Disease Among Commercially Insured People in the United States. Arthritis Rheumatol, 74, suppl 9. https://acrabstracts.org/abstract/the-epidemiology-clinical-outcomes-and-healthcare-resource-utilization-of-igg4-related-disease-among-commercially-insured-people-in-the-united-states/
Wallace, Z. S., Wallace, C. J., Lu, N., Choi, H. K., & Stone, J. H. (2016). Association of IgG4-Related Disease with History of Malignancy. Arthritis & Rheumatology (Hoboken, N.J.), 68(9), 2283–2289. https://doi.org/10.1002/art.39773
Wang, L., Zhang, P., Zhang, X., Lin, W., Tang, H., Li, J., Wang, M., Liu, X., Fei, Y., Chen, H., Peng, L., Zhang, L., Lai, Y., Zeng, X., Li, X., Xue, H., Zhao, Y., Zhang, F., & Zhang, W. (2019). Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: A prospective study of 403 patients. Rheumatology (Oxford, England), 58(5), 820–830. https://doi.org/10.1093/rheumatology/key397
Xiao, J., Xu, P., Li, B., Hong, T., Liu, W., He, X., Zheng, C., & Zhao, Y. (2018). Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis: A retrospective cohort study of 39 IAC patients. Medicine, 97(8), e9767. https://doi.org/10.1097/MD.0000000000009767
Yamamoto, M., Takahashi, H., Tabeya, T., Suzuki, C., Naishiro, Y., Ishigami, K., Yajima, H., Shimizu, Y., Obara, M., Yamamoto, H., Himi, T., Imai, K., & Shinomura, Y. (2012). Risk of malignancies in IgG4-related disease. Modern Rheumatology, 22(3), 414–418. https://doi.org/10.1007/s10165-011-0520-x
Yokoyama, Y., Akutsu, N., Numata, Y., Okuda, K., Morita, R., Masaki, Y., Sasaki, H., Suzuki, R., Yabana, T., Arimura, Y., Kondo, Y., & Nakase, H. (2021). Immunoglobulin G4-related autoimmune hepatitis simultaneously concomitant with autoimmune pancreatitis: A case report. Clinical Journal of Gastroenterology, 14(6), 1740–1745. https://doi.org/10.1007/s12328-021-01509-0
Yunyun, F., Yu, P., Panpan, Z., Xia, Z., Linyi, P., Jiaxin, Z., Li, Z., Shangzhu, Z., Jinjing, L., Di, W., Yamin, L., Xiaowei, L., Huadan, X., Xuan, Z., Xiaofeng, Z., Fengchun, Z., Yan, Z., & Wen, Z. (2019). Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: A randomized clinical trial. Rheumatology (Oxford, England), 58(1), 52–60. https://doi.org/10.1093/rheumatology/key227
Zen, Y. (2016). The Pathology of IgG4-Related Disease in the Bile Duct and Pancreas. Seminars in Liver Disease, 36(3), 242–256. https://doi.org/10.1055/s-0036-1584319
Zen, Y., Harada, K., Sasaki, M., Sato, Y., Tsuneyama, K., Haratake, J., Kurumaya, H., Katayanagi, K., Masuda, S., Niwa, H., Morimoto, H., Miwa, A., Uchiyama, A., Portmann, B. C., & Nakanuma, Y. (2004). 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? The American Journal of Surgical Pathology, 28(9), 1193–1203. https://doi.org/10.1097/01.pas.0000136449.37936.6c
Arase, Y., Matsumoto, K., Anzai, K., Tsuruya, K., Sugiyama, S., Yoshihara, S., Hirose, S., Uojima, H., Hidaka, H., Nakazawa, T., Deguchi, R., Kojima, S., Takashimizu, S., Shiraishi, K., Shirai, T., & Kagawa, T. (2021). Clinicopathological Features of Autoimmune Hepatitis with IgG4-Positive Plasma Cell Infiltration. Digestive Diseases (Basel, Switzerland), 39(3), 225–233. https://doi.org/10.1159/000510562
Aydemir, Y., Akcoren, Z., Demir, H., Saltik Temizel, I. N., Ozen, H., & Yuce, A. (2019). Clinical and histopathological features of immunoglobulin G4-associated autoimmune hepatitis in children. Journal of Gastroenterology and Hepatology, 34(4), 742–746. https://doi.org/10.1111/jgh.14525
Backhus, J., Neumann, C., Perkhofer, L., Schulte, L. A., Mayer, B., Seufferlein, T., Müller, M., & Kleger, A. (2021). A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab. Journal of Clinical Medicine, 10(6), 1329. https://doi.org/10.3390/jcm10061329
Canivet, C. M., Anty, R., Patouraux, S., Saint-Paul, M.-C., Lebeaupin, C., Gual, P., Duclos-Vallee, J.-C., & Tran, A. (2016). Immunoglobulin G4-associated autoimmune hepatitis may be found in Western countries. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 48(3), 302–308. https://doi.org/10.1016/j.dld.2015.10.005
Carballo, I., González-Quintela, A., Sopeña, B., & Vidal, C. (2021). Immunoglobulin G4–Related Disease: What an Allergist Should Know. Journal of Investigational Allergology and Clinical Immunology, 31(3), 212–227. https://doi.org/10.18176/jiaci.0633
Chari, S. T., Smyrk, T. C., Levy, M. J., Topazian, M. D., Takahashi, N., Zhang, L., Clain, J. E., Pearson, R. K., Petersen, B. T., Vege, S. S., & Farnell, M. B. (2006). Diagnosis of autoimmune pancreatitis: The Mayo Clinic experience. Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association, 4(8), 1010–1016; quiz 934. https://doi.org/10.1016/j.cgh.2006.05.017
Chung, H., Watanabe, T., Kudo, M., Maenishi, O., Wakatsuki, Y., & Chiba, T. (2010). Identification and characterization of IgG4-associated autoimmune hepatitis. Liver International: Official Journal of the International Association for the Study of the Liver, 30(2), 222–231. https://doi.org/10.1111/j.1478-3231.2009.02092.x
Deshpande, V. (2015). IgG4-Related Disease of the Gastrointestinal Tract: A 21st Century Chameleon. Archives of Pathology & Laboratory Medicine, 139(6), 742–749. https://doi.org/10.5858/arpa.2014-0181-RA
Deshpande, V., Zen, Y., Chan, J. K., Yi, E. E., Sato, Y., Yoshino, T., Klöppel, G., Heathcote, J. G., Khosroshahi, A., Ferry, J. A., Aalberse, R. C., Bloch, D. B., Brugge, W. R., Bateman, A. C., Carruthers, M. N., Chari, S. T., Cheuk, W., Cornell, L. D., Fernandez-Del Castillo, C., … Stone, J. H. (2012). Consensus statement on the pathology of IgG4-related disease. Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc, 25(9), 1181–1192. https://doi.org/10.1038/modpathol.2012.72
Ghazale, A., & Chari, S. T. (2007). Optimising corticosteroid treatment for autoimmune pancreatitis. Gut, 56(12), 1650–1652. https://doi.org/10.1136/gut.2007.129833
Ghazale, A., Chari, S. T., Zhang, L., Smyrk, T. C., Takahashi, N., Levy, M. J., Topazian, M. D., Clain, J. E., Pearson, R. K., Petersen, B. T., Vege, S. S., Lindor, K., & Farnell, M. B. (2008). Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy. Gastroenterology, 134(3), 706–715. https://doi.org/10.1053/j.gastro.2007.12.009
Hart, P. A., Law, R. J., Dierkhising, R. A., Smyrk, T. C., Takahashi, N., & Chari, S. T. (2014). Risk of cancer in autoimmune pancreatitis: A case-control study and review of the literature. Pancreas, 43(3), 417–421. https://doi.org/10.1097/MPA.0000000000000053
Hirano, K., Tada, M., Sasahira, N., Isayama, H., Mizuno, S., Takagi, K., Watanabe, T., Saito, T., Kawahata, S., Uchino, R., Hamada, T., Miyabayashi, K., Mohri, D., Sasaki, T., Kogure, H., Yamamoto, N., Nakai, Y., Yoshida, H., Ito, Y., … Koike, K. (2014). Incidence of malignancies in patients with IgG4-related disease. Internal Medicine (Tokyo, Japan), 53(3), 171–176. https://doi.org/10.2169/internalmedicine.53.1342
Huggett, M. T., Culver, E. L., Kumar, M., Hurst, J. M., Rodriguez-Justo, M., Chapman, M. H., Johnson, G. J., Pereira, S. P., Chapman, R. W., Webster, G. J. M., & Barnes, E. (2014). Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. The American Journal of Gastroenterology, 109(10), 1675–1683. https://doi.org/10.1038/ajg.2014.223
Ikeura, T., Miyoshi, H., Uchida, K., Fukui, T., Shimatani, M., Fukui, Y., Sumimoto, K., Matsushita, M., Takaoka, M., & Okazaki, K. (2014). Relationship between autoimmune pancreatitis and pancreatic cancer: A single-center experience. Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 14(5), 373–379. https://doi.org/10.1016/j.pan.2014.04.029
Kamisawa, T., Funata, N., Hayashi, Y., Eishi, Y., Koike, M., Tsuruta, K., Okamoto, A., Egawa, N., & Nakajima, H. (2003). A new clinicopathological entity of IgG4-related autoimmune disease. Journal of Gastroenterology, 38(10), 982–984. https://doi.org/10.1007/s00535-003-1175-y
Kamisawa, T., Tsuruta, K., Okamoto, A., Horiguchi, S., Hayashi, Y., Yun, X., Yamaguchi, T., & Sasaki, T. (2009). Frequent and significant K-ras mutation in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis. Pancreas, 38(8), 890–895. https://doi.org/10.1097/MPA.0b013e3181b65a1c
Kastin, D., Siegel, M., Anderson, R., & Aronsohn, A. (2021). IgG 4 Autoimmune Hepatitis Presenting as Idiopathic Hypereosinophilia Syndrome. Hepatology (Baltimore, Md.), 73(4), 1615–1617. https://doi.org/10.1002/hep.31492
Kemp, W., Majeed, A., Mitchell, J., Majumdar, A., Tse, E., Skoien, R., Croagh, D., Dev, A., Gao, H., Weltman, M., Craig, P., Stuart, K., Cheng, W., Edmunds, S., Lee, E., Sood, S., Metz, A., Thompson, A., Sinclair, M., … Roberts, S. K. (2021). Management, outcomes and survival of an Australian IgG4-SC cohort: The MOSAIC study. Liver International: Official Journal of the International Association for the Study of the Liver, 41(12), 2934–2943. https://doi.org/10.1111/liv.15036
Kim, K.-P., Kim, M.-H., Kim, J. C., Lee, S. S., Seo, D. W., & Lee, S. K. (2006). Diagnostic criteria for autoimmune chronic pancreatitis revisited. World Journal of Gastroenterology, 12(16), 2487–2496. https://doi.org/10.3748/wjg.v12.i16.2487
Lanzillotta, M., Della-Torre, E., Wallace, Z. S., Stone, J. H., Karadag, O., Fernández-Codina, A., Arcidiacono, P. G., Falconi, M., Dagna, L., & Capurso, G. (2021). Efficacy and safety of rituximab for IgG4-related pancreato-biliary disease: A systematic review and meta-analysis. Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 21(7), 1395–1401. https://doi.org/10.1016/j.pan.2021.06.009
Löhr, J.-M., Vujasinovic, M., Rosendahl, J., Stone, J. H., & Beuers, U. (2022). IgG4-related diseases of the digestive tract. Nature Reviews. Gastroenterology & Hepatology, 19(3), 185–197. https://doi.org/10.1038/s41575-021-00529-y
Minaga, K., Watanabe, T., Chung, H., & Kudo, M. (2019). Autoimmune hepatitis and IgG4-related disease. World Journal of Gastroenterology, 25(19), 2308–2314. https://doi.org/10.3748/wjg.v25.i19.2308
Naitoh, I., Kamisawa, T., Tanaka, A., Nakazawa, T., Kubota, K., Takikawa, H., Unno, M., Masamune, A., Kawa, S., Nakamura, S., Okazaki, K., & collaborators. (2021). Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 53(10), 1308–1314. https://doi.org/10.1016/j.dld.2021.02.009
Naitoh, I., & Nakazawa, T. (2022). Classification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis. Gut and Liver, 16(1), 28–36. https://doi.org/10.5009/gnl210116
Nakanuma, Y., Ishizu, Y., Zen, Y., Harada, K., & Umemura, T. (2016). Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease. Seminars in Liver Disease, 36(3), 229–241. https://doi.org/10.1055/s-0036-1584320
Nakazawa, T., Kamisawa, T., Okazaki, K., Kawa, S., Tazuma, S., Nishino, T., Inoue, D., Naitoh, I., Watanabe, T., Notohara, K., Kubota, K., Ohara, H., Tanaka, A., Takikawa, H., Masamune, A., & Unno, M. (2021). Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012). Journal of Hepato-Biliary-Pancreatic Sciences, 28(3), 235–242. https://doi.org/10.1002/jhbp.913
Nakazawa, T., Ohara, H., Sano, H., Ando, T., & Joh, T. (2006). Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas, 32(2), 229. https://doi.org/10.1097/01.mpa.0000202941.85955.07
Okamoto, A., Watanabe, T., Kamata, K., Minaga, K., & Kudo, M. (2019). Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis. Internal Medicine, 58(11), 1533–1539. https://doi.org/10.2169/internalmedicine.2210-18
Okazaki, K. (2019). Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment. Digestive Diseases and Sciences, 64(9), 2385–2394. https://doi.org/10.1007/s10620-019-05746-9
Okazaki, K., Kawa, S., Kamisawa, T., Naruse, S., Tanaka, S., Nishimori, I., Ohara, H., Ito, T., Kiriyama, S., Inui, K., Shimosegawa, T., Koizumi, M., Suda, K., Shiratori, K., Yamaguchi, K., Yamaguchi, T., Sugiyama, M., & Otsuki, M. (2006). Clinical diagnostic criteria of autoimmune pancreatitis: Revised proposal. Journal of Gastroenterology, 41(7), 626. https://doi.org/10.1007/s00535-006-1868-0
Otsuki, M., Chung, J. B., Okazaki, K., Kim, M.-H., Kamisawa, T., Kawa, S., Park, S. W., Shimosegawa, T., Lee, K., Ito, T., Nishimori, I., Notohara, K., Naruse, S., Ko, S. B. H., Kihara, Y., & Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labour and Welfare of Japan and the Korean Society of Pancreatobiliary Diseases. (2008). Asian diagnostic criteria for autoimmune pancreatitis: Consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. Journal of Gastroenterology, 43(6), 403–408. https://doi.org/10.1007/s00535-008-2205-6
Rebours, V., & Lévy, P. (2020). Pancreatic and biliary tract involvement in IgG4-related disease. Presse Medicale (Paris, France: 1983), 49(1), 104015. https://doi.org/10.1016/j.lpm.2020.104015
Shimosegawa, T., Chari, S. T., Frulloni, L., Kamisawa, T., Kawa, S., Mino-Kenudson, M., Kim, M.-H., Klöppel, G., Lerch, M. M., Löhr, M., Notohara, K., Okazaki, K., Schneider, A., & Zhang, L. (2011). International Consensus Diagnostic Criteria for Autoimmune Pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas, 40(3), 352–358. https://doi.org/10.1097/MPA.0b013e3182142fd2
Shiokawa, M., Kodama, Y., Yoshimura, K., Kawanami, C., Mimura, J., Yamashita, Y., Asada, M., Kikuyama, M., Okabe, Y., Inokuma, T., Ohana, M., Kokuryu, H., Takeda, K., Tsuji, Y., Minami, R., Sakuma, Y., Kuriyama, K., Ota, Y., Tanabe, W., … Chiba, T. (2013). Risk of cancer in patients with autoimmune pancreatitis. The American Journal of Gastroenterology, 108(4), 610–617. https://doi.org/10.1038/ajg.2012.465
Smit, W. L., Culver, E. L., & Chapman, R. W. (2016). New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis. Clinics in Liver Disease, 20(1), 47–65. https://doi.org/10.1016/j.cld.2015.08.004
Stone, J. H., Zen, Y., & Deshpande, V. (2012). IgG4-related disease. The New England Journal of Medicine, 366(6), 539–551. https://doi.org/10.1056/NEJMra1104650
Sumimoto, K., Uchida, K., Ikeura, T., Hirano, K., Yamamoto, M., Takahashi, H., Nishino, T., Mizushima, I., Kawano, M., Kamisawa, T., Saeki, T., Maguchi, H., Ushijima, T., Shiokawa, M., Seno, H., Goto, H., Nakamura, S., Okazaki, K., & Research Committee for an Intractable Disease of IgG4-related disease. (2022). Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan. Journal of Gastroenterology and Hepatology, 37(6), 1022–1033. https://doi.org/10.1111/jgh.15809
Tanaka, A., Mori, M., Kubota, K., Naitoh, I., Nakazawa, T., Takikawa, H., Unno, M., Kamisawa, T., Kawa, S., & Okazaki, K. (2020). Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan. Journal of Hepato-Biliary-Pancreatic Sciences, 27(9), 598–603. https://doi.org/10.1002/jhbp.793
Tanaka, A., & Notohara, K. (2021). Immunoglobulin G4 (IgG4)-related autoimmune hepatitis and IgG4-hepatopathy: A histopathological and clinical perspective. Hepatology Research: The Official Journal of the Japan Society of Hepatology, 51(8), 850–859. https://doi.org/10.1111/hepr.13683
Tanaka, A., Tazuma, S., Okazaki, K., Nakazawa, T., Inui, K., Chiba, T., & Takikawa, H. (2017). Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis. Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association, 15(6), 920-926.e3. https://doi.org/10.1016/j.cgh.2016.12.038
Uchida, K., Masamune, A., Shimosegawa, T., & Okazaki, K. (2012). Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009. International Journal of Rheumatology, 2012, 358371. https://doi.org/10.1155/2012/358371
Umehara, H., Okazaki, K., Kawa, S., Takahashi, H., Goto, H., Matsui, S., Ishizaka, N., Akamizu, T., Sato, Y., Kawano, M., & Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. (2021). The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Modern Rheumatology, 31(3), 529–533. https://doi.org/10.1080/14397595.2020.1859710
Umemura, T., Zen, Y., Hamano, H., Ichijo, T., Kawa, S., Nakanuma, Y., & Kiyosawa, K. (2007). IgG4 associated autoimmune hepatitis: A differential diagnosis for classical autoimmune hepatitis. Gut, 56(10), 1471–1472. https://doi.org/10.1136/gut.2007.122283
Umemura, T., Zen, Y., Hamano, H., Joshita, S., Ichijo, T., Yoshizawa, K., Kiyosawa, K., Ota, M., Kawa, S., Nakanuma, Y., & Tanaka, E. (2011). Clinical significance of immunoglobulin G4-associated autoimmune hepatitis. Journal of Gastroenterology, 46 Suppl 1, 48–55. https://doi.org/10.1007/s00535-010-0323-4
Vlachou, P. A., Khalili, K., Jang, H.-J., Fischer, S., Hirschfield, G. M., & Kim, T. K. (2011). IgG4-related sclerosing disease: Autoimmune pancreatitis and extrapancreatic manifestations. Radiographics: A Review Publication of the Radiological Society of North America, Inc, 31(5), 1379–1402. https://doi.org/10.1148/rg.315105735
Wallace, Z., Miles, G., Smolkina, E. D., Petruski-Ivleva, N., Madziva, D., Cook, C., Fu, X., Zhang, Y., Ttone, john, & Choi, H. (2022). The Epidemiology, Clinical Outcomes, and Healthcare Resource Utilization of IgG4-Related Disease Among Commercially Insured People in the United States. Arthritis Rheumatol, 74, suppl 9. https://acrabstracts.org/abstract/the-epidemiology-clinical-outcomes-and-healthcare-resource-utilization-of-igg4-related-disease-among-commercially-insured-people-in-the-united-states/
Wallace, Z. S., Wallace, C. J., Lu, N., Choi, H. K., & Stone, J. H. (2016). Association of IgG4-Related Disease with History of Malignancy. Arthritis & Rheumatology (Hoboken, N.J.), 68(9), 2283–2289. https://doi.org/10.1002/art.39773
Wang, L., Zhang, P., Zhang, X., Lin, W., Tang, H., Li, J., Wang, M., Liu, X., Fei, Y., Chen, H., Peng, L., Zhang, L., Lai, Y., Zeng, X., Li, X., Xue, H., Zhao, Y., Zhang, F., & Zhang, W. (2019). Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: A prospective study of 403 patients. Rheumatology (Oxford, England), 58(5), 820–830. https://doi.org/10.1093/rheumatology/key397
Xiao, J., Xu, P., Li, B., Hong, T., Liu, W., He, X., Zheng, C., & Zhao, Y. (2018). Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis: A retrospective cohort study of 39 IAC patients. Medicine, 97(8), e9767. https://doi.org/10.1097/MD.0000000000009767
Yamamoto, M., Takahashi, H., Tabeya, T., Suzuki, C., Naishiro, Y., Ishigami, K., Yajima, H., Shimizu, Y., Obara, M., Yamamoto, H., Himi, T., Imai, K., & Shinomura, Y. (2012). Risk of malignancies in IgG4-related disease. Modern Rheumatology, 22(3), 414–418. https://doi.org/10.1007/s10165-011-0520-x
Yokoyama, Y., Akutsu, N., Numata, Y., Okuda, K., Morita, R., Masaki, Y., Sasaki, H., Suzuki, R., Yabana, T., Arimura, Y., Kondo, Y., & Nakase, H. (2021). Immunoglobulin G4-related autoimmune hepatitis simultaneously concomitant with autoimmune pancreatitis: A case report. Clinical Journal of Gastroenterology, 14(6), 1740–1745. https://doi.org/10.1007/s12328-021-01509-0
Yunyun, F., Yu, P., Panpan, Z., Xia, Z., Linyi, P., Jiaxin, Z., Li, Z., Shangzhu, Z., Jinjing, L., Di, W., Yamin, L., Xiaowei, L., Huadan, X., Xuan, Z., Xiaofeng, Z., Fengchun, Z., Yan, Z., & Wen, Z. (2019). Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: A randomized clinical trial. Rheumatology (Oxford, England), 58(1), 52–60. https://doi.org/10.1093/rheumatology/key227
Zen, Y. (2016). The Pathology of IgG4-Related Disease in the Bile Duct and Pancreas. Seminars in Liver Disease, 36(3), 242–256. https://doi.org/10.1055/s-0036-1584319
Zen, Y., Harada, K., Sasaki, M., Sato, Y., Tsuneyama, K., Haratake, J., Kurumaya, H., Katayanagi, K., Masuda, S., Niwa, H., Morimoto, H., Miwa, A., Uchiyama, A., Portmann, B. C., & Nakanuma, Y. (2004). 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? The American Journal of Surgical Pathology, 28(9), 1193–1203. https://doi.org/10.1097/01.pas.0000136449.37936.6c