Background/Purpose: Inflammatory subglottic stenosis may present with discomfort, stridor, and hoarseness. In these patients granulomatosis with angiitis needs to be ruled out.  

 A diagnosis using direct visualization and biopsy is made during videoendoscopy.  Biopsy is often combined with a dilatation procedure performed in the operating room under general anesthesia. Inflammatory subglottic stenosis appears to always recur after dilatation, but intervals between dilatations may possibly be lengthened due to the use of corticosteroid AND immunosuppressive and biologic response modifier medication.

 Methods: We describe 16 patients with inflammatory subglottic stenosis

Results: The patients were diagnosed and followed in a laryngology office. Mean age 35 (21-63). All patients presented with SOB and stridor. Ten also had nasal symptoms, 2 had  saddle nose deformities. 3 pts were positive for cANCA and 4 had pANCA. The patients did not have symptoms of cutaneous or systemic vasculitis, sinusitis, pulmonary or renal involvement or other features of granulomatosis with polyangiitis.  The patients all had biopsies showing acute and chronic inflammatory changes. Granulomas and vasculitis were not seen. All had dilation procedures. One had a cricotracheal resection. 3 pts had a tracheotomy. In cases of recurrent inflammatory subglottic stenosis, corticosteroids were very helpful in treatment. Five pts were treated with methotrexate, and one with cyclophosphamide. Almost all patients were negative for the presence of cytoplasmic antibodies.

Conclusion: Recurrent inflammatory subglottic stenosis is described in 16 patients.  All experienced shortness of breath and stridor.

Inflammatory subglottic stenosis appears to be a unique entity, not  related to granulomatosis with polyangiitis.

Treatment with corticosteroids was helpful, but did not avoid the eventual need for subsequent dilation. Methotrexate was moderately effective. One patient received cyclophosphamide. The use of TNF blockers as other biologic response modifiers is unknown.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/subglottic-stenosis-a-unique-inflammatory-disorder/