Immune mechanisms of fibrosis and inflammation in IgG4-related disease

Purpose of review To summarize recent advances in the understanding of the pathogenesis of IgG4-related disease. Recent findings Limited data exist to explain genetic susceptibility to IgG4-related disease and the underlying triggers for this disease have not yet been identified. Cytotoxic CD4 + T cells and activated B cells infiltrate affected organs and express proinflammatory and profibrotic molecules. Antigen presented by activated B cells...