General principles of IgG4-related disease

The concept of IgG4-related disease is rapidly evolving. The disease has been described in virtually every organ system and can mimic a number of other important malignant and inflammatory conditions. Consensus on nomenclature was proposed at an international IgG4-related disease symposium in Boston in 2011. Diagnostic criteria have been established to include characteristic histopathology, clinical and radiological manifestations, serology and response to treatment. Consensus histopathology guidelines have been agreed by an international panel of experts. Treatment options continue to expand and include corticosteroids, immunosuppressive therapy and B-cell depletion strategies. The role of IgG4 in pathogenesis remains uncertain but elucidating this will be crucial in the further understanding of this disease. This review will summarize the principles of IgG4-related disease from a clinical perspective.