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IgG4-Related Disease Presenting as a Neck Mass

IgG4-related disease (IgG4-RD) is now understood to be an immune-mediated condition affecting multiple anatomic sites. Disorders that were previously considered unrelated are now classified as IgG4-RD and share pathologic, serologic, and clinical features. Lacrimal and salivary gland swelling (formerly Mikulicz disease), bilateral submandibular gland enlargement (formerly Kuttner’s tumor), as well as extraocular muscle swelling and associated proptosis are common manifestations of IgG4-RD. Reidel’s thyroiditis is also considered to be one manifestation of IgG4-RD. Patients with IgG4-RD often present with swelling of lymph nodes or other organs that are enriched with IgG4-positive plasma cells; most patients have elevated serum IgG4 concentrations. Here we discuss the case of a patient with IgG4-RD presenting with a neck mass.