IgG4 Autoimmune diseases

IgG4 Autoimmune diseases

IgG4 autoimmune diseases (IgG4-AID) are a group of severe autoimmunopathies that are characterized by pathogenic IgG4 autoantibodies (abs) against defined target antigens (some examples shown in Table 1). IgG4-AID may affect different organs, with the largest number of diseases affecting the peripheral and central nervous systems(1). Interestingly, they share common mechanistic and therapeutic properties, but due to their diverse clinical involvements, their relation has not been recognized until recently. IgG4-AID are rare disorders, but their cumulative prevalence adds up to 11 patients per 10,000, thus >490,000 patients in the EU. IgG4-AID are clinically severe, difficult to diagnose and challenging to treat.

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