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

S Andrew Skillington; M Allison Ogden

doi:10.1016/j.otc.2021.02.002

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

Otolaryngol Clin North Am. 2021 Jun;54(3):497-508. doi: 10.1016/j.otc.2021.02.002.

Authors

S Andrew Skillington¹, M Allison Ogden²

Affiliations

¹ Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine in St. Louis, 660 South Euclid Avenue, PO Box 8115, St Louis, MO 63110, USA.
² Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine in St. Louis, 660 South Euclid Avenue, PO Box 8115, St Louis, MO 63110, USA. Electronic address: ogdenm@wustl.edu.

PMID: 34024478
DOI: 10.1016/j.otc.2021.02.002

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.

Publication types

Review

MeSH terms

Glucocorticoids
Humans
Immunoglobulin G
Immunoglobulin G4-Related Disease* / diagnosis
Immunoglobulin G4-Related Disease* / therapy
Salivary Glands
Sialadenitis* / diagnosis
Sialadenitis* / therapy

Substances

Glucocorticoids
Immunoglobulin G