IgG4-Related Disease: History and Overview

  • Shigeyuki KawaEmail author


IgG4-related disease (IgG4-RD) is a recently established disease concept closely associated with increased levels of IgG4 in serum and elevated concentrations of IgG4+ plasma cells within affected tissues. IgG4-RD is currently considered to be an immune-mediated condition with possible contributions from autoimmunity, but the precise mechanisms remain to be established. The characteristic features of IgG4-RD can be summarized as follows: (1) systemic organ involvement, in which individual organ manifestations may require different approaches to management; (2) the ability to involve multiple organs either simultaneously or in a metachronous fashion; (3) imaging findings of swelling, nodules, or increased organ wall thickness; (4) elevated serum IgG4 concentrations, frequently to dramatic levels; (5) lymphoplasmacytic infiltrate within affected organs, characterized by abundant IgG4-bearing plasma cells; and, (6) favorable response to glucocorticoid therapy in most cases.

The establishment of this entity originated with the discovery of high serum IgG4 concentration and marked IgG4-bearing plasma cell infiltration in patients with autoimmune pancreatitis (AIP). This was followed by the recognition of systemic extra-pancreatic lesions complicating AIP that exhibited the same clinical features, such as IgG4-bearing plasma cell infiltration and a favorable response to glucocorticoid therapy. These observations suggested a common pathophysiological background for AIP and its extra-pancreatic lesions and culminated in the proposal of a systemic disease that encompassed IgG4, AIP, and systemic lesions. Although considerable advances in IgG4-RD diagnosis and treatment are being made, the etiology, exact disease spectrum and long-term outcomes of this new disease entity require further study.


IgG4-related disease Autoimmune pancreatitis Mikulicz’s disease IgG4 



We thank Trevor Ralph for his English editorial assistance. This work was supported partially by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare of Japan and in part by Grants-in-aid for Scientific Research from the Ministry of Education, Science, Sports, and Culture of Japan (15 K09045).


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© Springer Japan 2016

Authors and Affiliations

  1. 1.Center for Health, Safety, and Environmental ManagementShinshu UniversityMatsumotoJapan

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