Abstract
Although IgG4-related disease (IgG4-RD) is a novel and important disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells, its clinical diagnostic criteria have not been fully established. Many patients with IgG4-RD have lesions in multiple organs, either synchronously or metachronously, and the pathological features of each organ differ. We established comprehensive diagnostic criteria for IgG4-RD for the practical use of specialists and nonspecialists.
The two authors contributed equally to this work.
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References
Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22:1–14.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Eng J Med. 2012;366:539–51.
Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.
Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol. 2012;22:21–30.
Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.
Kamisawa T, Nakajima H, Egawa N, et al. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6:132–7.
Okazaki K, Uchida K, Miyoshi H, et al. Recent concepts of autoimmune pancreatitis and IgG4-related disease. Clin Rev Allergy Immunol. 2011;41:126–38.
Okazaki K, Uchida K, Koyabu M, et al. Recent advances in the concept and diagnosis of auto-immune pancreatitis and IgG4-related disease. J Gastroenterol. 2011;46:277–88.
Yamamoto M, Ohara M, Suzuki C, et al. Elevated IgG4 concentrations in serum of patients with Mikulicz’s disease. Scand J Rhumatol. 2004;33:432–3.
Masaki Y, Dong L, Kurose N, et al. Proposal for a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis. 2009;63:1310–5.
Dahlgren M, Khosroshahi A, Nielsen GP, et al. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken). 2010;62:1312–8.
Kitagawa S, Zen Y, Harada K, et al. Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Küttner’s tumor). Am J Surg Pathol. 2005;29:783–91.
Hamano H, Kawa S, Ochi Y, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359:1403–4.
Zen Y, Onodera M, Inoue D, et al. Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol. 2009;33:1833–9.
Zen Y, Kitagawa S, Minato H, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36:710–7.
Saeki T, Saito A, Yamazaki H, et al. Tubulointerstitial nephritis associated with IgG4-related systemic disease. Clin Exp Nephrol. 2007;11:168–73.
Saeki T, Nishi S, Imai N, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2010;78:1016–23.
Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886–93.
Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009;251:260–70.
Sato Y, Notohara K, Kojima M, et al. IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int. 2010;60:247–58.
Yamamoto M, Takahashi H, Suzuki C, et al. Analysis of serum IgG subclasses in Churg–Strauss syndrome-the meaning of elevated serum levels of IgG4. Intern Med. 2010;49:1365–70.
Masaki Y, Sugai S, Umehara H. IgG4-related diseases including Mikulicz’s disease and sclerosing pancreatitis: diagnostic insights. J Rheumatol. 2010;37:1380–5.
Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the Inter-national Association of Pancreatology. Pancreas. 2011;40:352–8.
Kawano M, Saeki T, Nakashima H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15:615–26.
Deshpande V, Zen Y, Chan JKC, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.
Stone JH, Khosroshahi A, Deshpande V, et al. IgG4-related disease: recommendations for the nomenclature of this condition and its individual organ system manifestations. Arthritis Rheum. 2012;64:3061–7.
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Umehara, H., Okazaki, K. (2014). Comprehensive Diagnostic Criteria for IgG4-Related Disease. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_6
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DOI: https://doi.org/10.1007/978-4-431-54228-5_6
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