An overview of the diagnosis and management of immunoglobulin G4-related disease.

The article offers an overview of the diagnosis and management of immunoglobulin G4 (IgG4)-related disease, a multisystem fibroinflammatory disease that can process to organ failure if left untreated. Topics include the pathophysiologic mechanisms involved in IgG4-related disease, the geoepidemiology of the disease, and diagnosis which include routine blood tests, immunologic blood tests, and histologic assessment. The sensitivity of IgG4-related disease to glucocorticoid therapy is noted.