An Unusual Case of Steroid Resistant IgG4-RD Affecting the Maxillary Alveolar Process

Immunoglobulin G4-related diseaseis an autoimmune fibro-inflammatory condition which is characterisedhistologically by marked lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. Immunostaining reveals IgG4+ plasma cells. Immunoglobulin G4-related diseaseaffecting the head and neck region is becoming increasingly recognised and generally shows a good initial response to steroids. Common manifestations within the head and neck include the orbit and the salivary glands. This article presents a rare case of immunoglobulin G4-related diseaseoriginating in the alveolar process of the maxilla with orbital and maxillary sinus involvement which wasunresponsive to management with steroids.