Session Title: Vasculitis
Session Type: Abstract Submissions (ACR)
Tracheobronchial stenosis (TBS) is noted in 12 to 23% in patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis (SGS) and bronchial stenosis. The optimal systemic treatments and endoscopic interventions providing the best efficacy, and the best timing for such interventions, remain unclear, explaining why, in the 2010s, TBS remains a therapeutic challenge in the management of GPA patients.
To analyze the endoscopic management of TBS in GPA and to identify factors associated with the efficacy of endoscopic interventions, we conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS. We also compared characteristics of GPA patients with TBS with GPA patients without TBS included in the French Vasculitis Study Group.
Compared to patients without TBS, those with TBS were younger, more frequently female, and had less frequent kidney, ocular and gastrointestinal involvement and mononeuritis multiplex.
173 procedures were performed in 47 patients. Endoscopic procedures included 137 tracheal and 50 bronchial interventions, mainly endoscopic dilatation, local steroid injection and conservative laser surgery, and less frequently stenting. Per-endoscopic events were noted in only 5/173 cases (2.9%). After the first endoscopic procedure, cumulative incidence of endoscopic treatment failure was 49% at 1 year, 70% at 2 years and 80% at 5 years.
Factors significantly associated with a higher cumulative incidence of treatment failure were a shorter time from GPA diagnosis to endoscopic procedure [HR 1.08 (1.01-1.14); P=0.01] and a bronchial stenosis [HR 1.96 (1.28-3.00); P=0.002]. A prednisone dose ≥30 mg/day at the time of the procedure was associated with a lower cumulative incidence of treatment failure [HR 0.53 (0.31-0.89); P=0.02]. No difference was observed according to the immunosuppressive agents used.
Finally, 13 patients (28%) experienced recurrent bacterial bronchopulmonary infections as late complications of tracheobronchial involvement, fatal in 2 cases (4%).
TBS represent severe and refractory manifestations with high rate of restenosis. High-dose systemic corticosteroids at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than SGS.
J. E. Kahn,
G. Le Guenno,
L. Guillevin for the French Vasculitis Study Group,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-wegeners-endoscopic-management-of-tracheobronchial-stenosis-results-from-a-multicenter-experience-in-47-patients/