Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary manifestation of a systemic condition known as IgG4-related disease (IgG4-RD). IgG4-RD may involve various organs with type 1 autoimmune pancreatitis (AIP) being the leading manifestation. Older men are more affected by IgG4-SC than women (ratio of 4:1), and the most common clinical manifestation is obstructive jaundice. The pathogenic mechanism of IgG4-SC remains unclear. Steroid therapy can lead to clinical and radiological improvement when provided in the inflammatory phase of this disease. However, long-term outcome data in patients with IgG4-SC are lacking. In this chapter, we focus on the prognosis of IgG4-SC, including the natural course, treatment response, risk of malignancy, and long-term outcome, based on current evidence.
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References
Okazaki K, Uchida K, Koyabu M, Miyoshi H, Ikeura T, Takaoka M. IgG4 cholangiopathy: current concept, diagnosis, and pathogenesis. J Hepatol. 2014;61:690–5.
Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas. 2006;32:229.
Culver EL, Chapman RW. IgG4-related hepatobiliary disease: an overview. Nat Rev Gastroenterol Hepatol. 2016;13:601–12.
Kamisawa T, Yoshiike M, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology. 2005;5:234–40.
Kubota K, Iida H, Fujisawa T, Yoneda M, Inamori M, Abe Y, et al. Clinical factors predictive of spontaneous remission or relapse in cases of autoimmune pancreatitis. Gastrointest Endosc. 2007;66:1142–51.
Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. Second international symposium on IgG4-related disease. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. 2015;67:1688–99.
Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.
Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.
Sandanayake NS, Church NI, Chapman MH, Johnson GJ, Dhar DK, Amin Z, et al. Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2009;7:1089–96.
Huggett MT, Culver EL, Kumar M, Hurst JM, Rodriguez-Justo M, Chapman MH, et al. Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. Am J Gastroenterol. 2014;109:1675–83.
Hart PA, Kamisawa T, Brugge WR, Chung JB, Culver EL, Czakó L, et al. Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut. 2013;62:1771–6.
Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2013;62:1607–15.
Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2008;6:364–6.
Zen Y, Kawakami H, Kim JH. IgG4-related sclerosing cholangitis: all we need to know. J Gastroenterol. 2016;51(4):295–312.
Shiokawa M, Kodama Y, Yoshimura K, Kawanami C, Mimura J, Yamashita Y, et al. Risk of cancer in patients with autoimmune pancreatitis. Am J Gastroenterol. 2013;108:610–7.
Hart PA, Law RJ, Dierkhising RA, Smyrk TC, Takahashi N, Chari ST. Risk of cancer in autoimmune pancreatitis: a case-control study and review of the literature. Pancreas. 2014;43:417–21.
Oh HC, Kim JG, Kim JW, Lee KS, Kim MK, Chi KC, et al. Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis. Intern Med. 2008;47:2025–8.
Straub BK, Esposito I, Gotthardt D, Radeleff B, Antolovic D, Flechtenmacher C, et al. IgG4-associated cholangitis with cholangiocarcinoma. Virchows Arch. 2011;458:761–5.
Tanaka A, Tazuma S, Okazaki K, Tsubouchi H, Inui K, Takikawa H. Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan. J Hepatobiliary Pancreat Sci. 2014;21:43–50.
Hirano A, Nakazawa T, Ohara H, Ando T, Hayashi K, Tanaka H, et al. Liver atrophy and portal stenosis in two cases of sclerosing cholangitis associated with autoimmune pancreatitis. Intern Med. 2008;47:1689–94.
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Kuwada, T., Shiokawa, M., Tomono, T., Uza, N., Kodama, Y. (2019). Prognosis. In: Kamisawa, T., Kim, MH. (eds) IgG4-Related Sclerosing Cholangitis. Springer, Singapore. https://doi.org/10.1007/978-981-10-4548-6_19
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DOI: https://doi.org/10.1007/978-981-10-4548-6_19
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