eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
Current issue Archive Manuscripts accepted About the journal Abstracting and indexing Subscription Contact Instructions for authors
SCImago Journal & Country Rank

vol. 43
Review paper

IgG4-related disease: why is it so important?

Marta Legatowicz-Koprowska

(Centr Eur J Immunol 2018; 43 (2): 204-208)
Online publish date: 2018/06/30
View full text
Get citation
JabRef, Mendeley
Papers, Reference Manager, RefWorks, Zotero
IgG4-related disease (IgG4-RD) is a recently defined systemic inflammatory and fibrous condition of unknown etiology and multiple clinical presentations. Characteristic features include elevated serum IgG4 levels in approximately 70% of patients; diffuse lymphoplasmocytic infiltrates rich in IgG4(+) cells; a “storiform” fibrosis pattern; and obliterative phlebitis affecting various organs. The disease responds well to corticosteroid treatment, with a second-line therapy involving B-cell-directed immunosuppressive biologic agents. Despite intense studies, the pathogenesis of IgG4-RD remains unclear. The inflammatory infiltrates present in affected tissues contain also multiple polyclonal T and B cells, plasma cells, and – often – eosinophils. Cytokines secreted by type 2 helper T-cells and regulatory T-cells are known to cause B-cell differentiation into IgG4-producing plasma cells. On the other hand, large numbers of IgG4(+) plasma cells can be observed in nonspecific chronic inflammatory conditions, areas adjacent to neoplastic lesions with an inflammatory response, and in autoimmune inflammatory infiltrates. Thus, the fundamental question about the role of IgG4(+) cells in the pathogenesis of inflammation, tissue damage, and fibrosis in IgG4-RD still remains unanswered: does IgG4 stimulate or rather – which is more consistent with its natural properties – play a regulatory function in the inflammatory process?

diagnosis, pathogenesis, IgG4-related disease, IgG4 immunoglobulin

Nirula A, Glaser SM, Kalled SL, et al. (2011): What is IgG4? A review of the biology of a unique immunoglobulin subtype. Curr Opin Rheumatol 23: 119-124.
Umehara H, Okazaki K, Masaki Y, et al. (2012): A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 22: 1-14.
Smit W, Barnes E (2014): The emerging mysteries of IgG4-related disease. Clin Med 14 (Suppl 6): 56-60.
Strehl JD, Hartmann A, Agaimy A (2011): Numerous IgG4- positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol 64: 237-243.
Takeuchi M, Sato Y, Ohno K, et al. (2014): T helper 2 and regulatory T-cell cytokine production by mast cells: a key factor in the pathogenesis of IgG4-related disease. Mod Pathol 27: 1126-1136.
Wallace ZS, Mattoo H, Carruthers M, et al. (2015): Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis 74: 190-195.
Rosenbloom J, Mendoza FA, Jimenez SA (2013): Strategies for anti-fibrotic therapies. Biochim Biophys Acta 1832: 1088-1103.
Comings DE, Skubi KB, Van Eyes J, et al. (1967): Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 66: 884-892.
Vasaitis L (2016): IgG4-related disease: A relatively new concept for clinicians. Eur J Intern Med 27: 1-9.
Deshpande V, Zen Y, Chan JKC, et al. (2012): Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25: 1181-1192.
Inoue D, Yoshida K, Yoneda N, et al. (2015): IgG4-related disease: dataset of 235 consecutive patients. Medicine 94: 1-8.
Jokisch F, Kleinlein I, Haller B, et al. (2016): A small subgroup of Hashimoto’s thyroiditis is associated with IgG4-related disease. Virchows Arch 468: 321-327.
Kamisawa T, Zen Y, Pillai S, Stone JH (2015): IgG4-related disease. Lancet 385: 1460-1471.
Quick links
© 2019 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.
PayU - płatności internetowe