Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a fibroinflammatory condition that has the potential to affect nearly every organ system. Classic histological findings include storiform fibrosis and lymphoplasmacytic infiltrates of immunoglobulin G4 (IgG4)-positive plasma cells. The clinical features of IgG4-RSD may be an under-recognized disease process that can mimic other autoimmune disorders, including Sjogren's syndrome. We describe a rare case of IgG4-RSD involving the salivary glands, initially misdiagnosed as Sjogren's syndrome. Clinical features of IgG4-RSD can mimic those of other autoimmune disorders affecting the head and neck. Therefore, otolaryngologists should have IgG4-RSD on their differential when evaluating patients with diffuse salivary gland swelling. Laryngoscope, 126:2242-2245, 2016.
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