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Laryngeal IgG4-Related Disease: A Systematic Review of Clinical Features and Management
Shadi Shinnawi1, Majd Khoury2, Abdellatif Zhalka3
1Department of Otolaryngology-Head and Neck Surgery, University of California-Davis, Sacramento, California, USA.
Objective:
To synthesize clinical presentation, anatomic patterns, diagnostic evaluation, and treatment outcomes of laryngeal IgG4-related disease (IgG4-RD) and highlight features that may support earlier diagnosis in unexplained laryngeal stenosis or mass-like lesions.
Data Sources:
Systematic searches of PubMed/MEDLINE, Embase, Scopus, Web of Science, and the Cochrane Library were performed through January 1, 2026, limited to English-language human studies combining IgG4-RD with laryngeal and upper-airway terms.
Review Methods:
Eligible studies reported histopathologic or clinicopathologic confirmation of IgG4-RD involving the supraglottic, glottic, or subglottic larynx with data on presentation, diagnosis, treatment, or outcomes. Two reviewers independently screened records and extracted data.
Results:
Twenty-seven publications met inclusion criteria, yielding 32 patients. Most were middle-aged with slight female predominance and presented with hoarseness, dyspnea, or stridor. Supraglottic and subglottic involvement predominated; multilevel airway disease occurred in about one third of cases. Endoscopy and imaging showed two main patterns: fibrostenotic circumferential narrowing resembling idiopathic subglottic stenosis and mass-like or polypoid lesions mimicking neoplasia. Histologic confirmation often required deep submucosal or repeat biopsy after nondiagnostic superficial sampling, and serum IgG4 levels were frequently normal despite biopsy-proven disease. Most patients received systemic glucocorticoids, often with additional immunosuppressive therapy and endoscopic dilation or airway surgery; relapse and progression to fixed fibrostenosis were common.
Conclusion:
Laryngeal IgG4-RD is an uncommon but underrecognized cause of airway obstruction and voice disturbance. Clinicians should include it in the differential diagnosis of unexplained laryngeal stenosis or submucosal masses and obtain adequately deep biopsies alongside long-term immunomodulatory and airway-preserving surgical strategies.
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