Clinical outcomes of subglottic stenosis in granulomatosis with polyangiitis: results of an international multicenter observational study - PubMed

5 hours ago

Subglottic stenosis (SGS) is a challenging manifestation of granulomatosis with polyangiitis (GPA), often relapsing and poorly responsive to immunosuppressive treatment.
The study aimed to identify features of SGS associated with a more aggressive course and assess the efficacy of available treatments in preventing relapses.
Eighty-nine patients were included, with 54% classified as higher relapsers (≥2 flares).
Systemic immunosuppressive therapy was associated with fewer relapses compared to local treatments alone.
Glucocorticoids in induction regimens reduced relapse risk, but glucocorticoid monotherapy was associated with higher relapse rates.
Cyclophosphamide (CYC) was linked to the longest relapse-free survival and reduced 5-year relapse risk.
No specific maintenance regimen was significantly protective, though rituximab and DMARDs showed a trend toward improved outcomes.
The study concludes that systemic immunosuppressive therapy, particularly CYC-based induction, is associated with fewer SGS relapses and prolonged relapse-free survival.

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