IgG4-mediated disease and potential risk of malignancy

IgG4-related disease (IgG4-RD) is considered a systemic, chronic and inflammatory disorder that is characterized by the enlargement of involved organs, elevated levels of IgG4 and abundant infiltration of plasmacytes with IgG4 and fibrosis in involved organs. The involved organs share a number of core pathologic features and striking clinical and serologic similarities, including tumour-like swelling of involved organs, a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, and a variable degree of fibrosis. We welcome the review from Dr Poo and colleagues from Imperial College, London in which they retrospectively analysed the presentation and clinical course of a cohort of IgG4-RD patients. They report that 15% of their patients had a history of malignancy. The precise reasons for this association have not been established. Various hypotheses suggested include an aberrant immunological response to cancer therapies or neo-antigen exposure in malignancy and the development of autoimmune mediated disease and IgG4-RD.
Source: QJM - Category: Internal Medicine Source Type: research