IgG4-related disease and its pathogenesis--cross-talk between innate and acquired immunity

IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4+ plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published. However, many questions still remain, including questions about the pathogenesis of IgG4-RD and the roles of IgG4. In this review, we discuss the pathogenesis of IgG4-RD by focusing on the cross-talk between innate and acquired immunity.

Source: International Immunology - October 18, 2014 Category: Allergy & Immunology Authors: Umehara, H., Nakajima, A., Nakamura, T., Kawanami, T., Tanaka, M., Dong, L., Kawano, M. Tags: Invited review Source Type: research