A Rare Case of Subglottic Immunoglobulin G4-Related Disease: Case Report and Literature Review

Document Type

Article

Publication Date

8-6-2026

Publication Title

Indian Journal of Otolaryngology and Head and Neck Surgery

Abstract

Immunoglobulin G4-related disease (IgG4-RD) typically affects the retroperitoneum, hepatobiliary system, salivary glands, orbital structures, and lymph nodes. Involvement of the upper airway is rare, and there remains no consensus on treatment. To address this gap in knowledge, we present a case of IgG4-RD manifesting as a subglottic mass, followed by a review of the literature. We first describe the clinical course of a patient with IgG4-RD of the subglottis. This is followed by a review of all relevant articles from January 2010 through June 2025 including case reports and case series of IgG4-RD involving the oropharynx, hypopharynx, supraglottis, glottis, and subglottis. Upper airway IgG4-RD is rare and often a delayed diagnosis. Our case involving the subglottis has responded well to ongoing rituximab infusions. Based on the literature review, standard of care involves glucocorticoids in combination with azathioprine or rituximab. Surgical excision is reserved for severe cases.

Rights

Copyright © 2026, Association of Otolaryngologists of India

Link to Full Text

Share

COinS