IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by an infiltration of lymphoplasmacytic enriched IgG4-positive plasma cells, variable degrees of fibrosis and mass forming lesions. Previously, this disorder was known as IgG4 multi-organ lymphoproliferative syndrome (IgG4 MOSLP), IgG4 sclerosing disease, or IgG4-related systemic plasmocytic syndrome (SIPS). The most common clinical manifestations are parotid inflammation, lymphadenopathy and autoimmune pancreatitis.
Diana L Franco, Jennifer Horsley-Silva and Keith D Lindor
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