Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or...

IgG4-related disease (IgG4-RD) is a recently described systemic fibro-inflammatory condition characterised by tumefactive lesions and dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells [1]. IgG4RD has been described in a variety of organ systems, including the lacrimal gland, salivary gland, lungs, pancreas and kidneys, and often responds favourably to corticosteroids. The p...