IgG4-related kidney disease

IgG4-related disease is a recently-described disease. It involves multiple organs including pancreas, salivary glands, kidneys, lungs, lymph nodes in which a characteristic histological change is identified. A dense lymphoplasmacytic infiltration with "storiform" fibrosis is replacing the normal organ parenchyma, frequently with a mass-like appearance observed radiologically. The plasma cells containing predominantly IgG4, a subclass of IgG, are the hallmark of the disease.