Editorial: IgG4-related Inflammatory Disease – a New Entity

IgG4-related inflammatory disease represents a new entity of unknown aetiology with a fribro-inflammatory condition associated with dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and often but not always elevated serum IgG4 concentration. This condition has been recently reviewed in the New England Journal of Medicine [1]. Auto-immune pancreatitis, described in 2001, was one of the first manifestations of the disease.

• References

• 1 Stone JH, Zen Y, Deshpande V. IgG4-related disease. New Engl J Med 2012; 366: 539-551
• 2 Balaskas K, de Leval L, La Corte R et al. Infliximab therapy for a severe case of IgG4-related ocular adnexal disorder recalcitrant to corticosteroid treatment. Ocul Immunol Inflammation 2012; 20: 478-480
• 3 Lindfield D. Rituximab in IgG4-related inflammatory disease of the orbit and ocular adnexae. Eye 2012; 26: 1386
• 4 Ferry JA, Deshpande V. IgG4-related disease in the head and neck. Sem Diagnos Pathol 2012; 29: 235-244