Immunoglobulin G4–related sclerosing disease Mimicking sjogren’s syndrome: A case report

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.