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Tracheobronchial Stenosis in Granulomatosis With Polyangiitis: Immunosuppressant Use and Airway Dilation Frequency
Brendan Denvir1, Ami A Shah1, Alexander T Hillel2
1Division of Rheumatology, Department of Medicine, Johns Hopkins University, Baltimore, Maryland.
Objective:
Tracheobronchial stenosis (TBS) occurs in 13% to 27% of patients with granulomatosis with polyangiitis (GPA) and may cause life-threatening airway compromise. Despite advances in treatment, TBS remains difficult to manage, with frequent relapses and high procedural burden. The objective of this study was to evaluate the relationship between immunosuppressant use and frequency of relapse in patients with TBS-GPA.
Methods:
We performed retrospective review of patients with TBS-GPA seen at Johns Hopkins Medical Institutions between 2013 and 2024. Baseline demographic and clinical characteristics, immunosuppressant exposure, and tracheal dilation procedure dates were abstracted. A multivariate mixed-effects Poisson regression model was used to assess the association between immunosuppressant exposures (rituximab, cyclophosphamide, methotrexate, azathioprine, leflunomide, and mycophenolate) and tracheal dilation incidence, adjusting for age, prednisone use, years since TBS diagnosis, antineutrophil cytoplasmic antibody status, GPA disease severity, and treatment with intralesional glucocorticoid injections.
Results:
A total of 56 patients with TBS-GPA were included in the analysis, with a mean follow-up duration of 9.9 years. In the adjusted mixed-effects Poisson model, patient-years while taking leflunomide were associated with a 68% lower incidence of tracheal dilations compared to periods not taking leflunomide (Incidence Rate Ratio 0.32, P < 0.001). No statistically significant associations were observed for the other immunosuppressants measured. Among other tested covariates, time since TBS diagnosis, nonsevere GPA, and intralesional glucocorticoid injections were associated with decreased dilation frequency.
Conclusion:
Leflunomide use was associated with a lower frequency of tracheal dilations in patients with TBS-GPA. These findings support the need for further evaluation of leflunomide as a treatment option in this population.
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