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IgG4-related disease (IgG4-RD) is a complex fibroinflammatory disease first identified in 2003.  It  has been described in every organ.  Type- 2 helper T cell immune-mediated responses are thought to be pathogenic. Serum IgG4 levels are not always elevated and immunohistochemical analysis is the gold standard for diagnosis.  When available, histopathology typically reveals lymphoplasmacytic infiltration, fibrosis, and abundant IgG4-positive plasma cells.

Here, we review clinically salient features of IgG4-related disease including its epidemiology, pathophysiology, diagnosis, and emerging treatment strategies.