IgG4-Related Disease and the Salivary Glands: A Review of Pathophysiology, Diagnosis, and Management

Abstract

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.

Keywords: IgG4-related disease; Küttner; Mikulicz; Salivary glands; Sialadenitis.