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Systemic Immunosuppression to Reduce Surgical Intervention in ANCA-Negative Subglottic Stenosis
Guy Benshetrit1,2, Stephen McAdoo2,3, Romana Kuchai1,2
1National Centre for Airway Reconstruction, Charing Cross Hospital, Imperial College Healthcare NHS Trust, London, UK.
Systemic immunosuppression combined with surgery may improve outcomes for patients with aggressive idiopathic subglottic stenosis (iSGS) and granulomatosis with polyangiitis (GPA-SGS). This approach can prolong disease-free intervals, offering new hope for managing this challenging airway condition.
Area of Science:
- Otolaryngology
- Immunology
- Pulmonology
Background:
- Idiopathic subglottic stenosis (iSGS) presents a clinical challenge due to its unclear etiology and recurrent nature.
- Current management primarily relies on surgical dilatation, often yielding suboptimal long-term results for a subset of patients.
- Aggressive forms of subglottic stenosis (SGS) necessitate exploring adjunctive therapies beyond surgery.
Purpose of the Study:
- To evaluate the efficacy of systemic immunosuppression as an adjunct to surgical management in patients with aggressive iSGS and granulomatosis with polyangiitis SGS (GPA-SGS).
- To assess the impact of immunosuppressive therapy on disease activity and inter-dilation intervals (IDI) in these patient cohorts.
Main Methods:
- Retrospective analysis of patients with isolated SGS at a tertiary airway center.
- Cohort classification into iSGS, GPA-SGS, and a sub-cohort of aggressive iSGS termed 'atypical-SGS'.
- Systemic immunosuppression was administered to atypical-SGS and GPA-SGS cohorts alongside surgery; disease activity was monitored via IDI before and after treatment.
Main Results:
- The study included 60 patients: 33 iSGS, 20 GPA-SGS, and 7 atypical SGS.
- Idiopathic SGS patients showed an indolent course without immunosuppression (median IDI: 17.6 months).
- GPA-SGS and atypical SGS cohorts demonstrated significantly prolonged IDI after immunosuppression (26.0 and 27.8 months, respectively) compared to pre-treatment intervals (p<0.05).
- No significant adverse events were reported with immunosuppressive therapy.
Conclusions:
- Atypical SGS represents a distinct, aggressive subtype of SGS requiring tailored management.
- Systemic immunosuppression, typically used for GPA, effectively extends disease-free intervals in both GPA-SGS and atypical SGS.
- Findings support considering immunotherapy for frequently recurring, ANCA-negative SGS, emphasizing multidisciplinary care.
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