IgG4 Related Disease of the maxillary sinus - a case report

Immunoglobulin G4 related disease (IgG4-RD) is a fibroinflammatory disorder which can affect any tissue or organ. It is characterized by tissue swelling with lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells, irregularly whorled pattern of fibrosis and often elevated serum IgG4 levels. Previously described conditions that represent manifestations of IgG4-RD include(1,2) IgG4-related pancreatitis,IgG4-related sclerosing cholangitis,Mikuliczs disease, Sclerosing sialadenitis (Kttners tumor), Inflammatory orbital pseudotumor, IgG4-related retroperitoneal fibrosis, IgG4-related aortitis or periaortitis, Riedels thyroiditis, pulmonary inflammatory pseudotumors, IgG4- related kidney disease, IgG4-related hypophysitis and IgG4-related pachymeningitis. The disease shows a good initial response to glucocorticoids. Second line agents which have showed response include azathioprine and Mycophenolate mofetil(3,4).B cell depletion therapy with Rituximab have also been tried effectively (5-7).