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IgG4 related disease is fibro-inflammatory condition characterized by infiltration and increased levels of G4 immunoglobulin, multi-organ involvement and periorbital swelling. It is more common among men especially in middle to old ages. The diagnosis should be confirmed with histopathology. The diagnostic three important features of disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. Treatment is Commonly based on corticosteroids. Abstract Elahedoi:10.29252/aums.9.2.231 fatcat:bwh5vy6c7fe2nkb6o43dyadghi