IgG4-related disease is a rare, immune-mediated, systemic disease that is characterized by soft tissue lymphocyte infiltration and resultant fibrosis. The salivary glands are among the most commonly affected organs. Patients present with subacute submandibular and/or parotid swelling and sialadenitis. Diagnosis incorporates clinical, serologic, radiologic, and pathologic findings. Most cases respond quickly to systemic glucocorticoids. IgG4-related disease mimics many infectious, inflammatory, and neoplastic diseases. Therefore, IgG4-related disease is frequently misdiagnosed. A knowledge of the pathophysiology, diagnosis, and management of IgG4-related disease is important for providers who treat salivary gland diseases.
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