The Laryngoscope
June 30, 2026 · via Europe PMC
Laryngeal IgG4-Related Disease: A Systematic Review of Clinical Features and Management.
Shinnawi S, Khoury M, Zhalka A, Elhija JA, Zahlaka M.
AI summary — for doctors, generated from the abstract only
Laryngeal IgG4-related disease is rare and often mimics idiopathic subglottic stenosis or a tumor, and may need deep biopsy since serum IgG4 can be normal.
What was studied
Systematic review of 27 publications describing 32 patients with histologically confirmed laryngeal IgG4-RD.
Key findings
- Most patients had hoarseness, dyspnea or stridor, and supraglottic and subglottic involvement predominated, with multilevel disease in about a third.
- Presentations were either fibrostenotic circumferential narrowing or mass-like lesions; superficial biopsy was often nondiagnostic and serum IgG4 often normal.
- Most received glucocorticoids, often with other immunosuppression and airway procedures; relapse and fixed fibrostenosis were common.
Clinical relevance
It reminds airway clinicians to consider IgG4-RD in unexplained laryngeal stenosis or submucosal masses.
Who should care
Limitations
Based on a small number of case reports and series, so reporting bias and no standardized outcomes limit conclusions.
Obtain deep or repeat biopsies with IgG4 staining in unexplained stenosis, and plan for long-term immunomodulation and airway management.
This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.
Abstract
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.
DOI
10.1002/lary.70714
PMID
42381230
Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.
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