Systemic immunosuppression may be effective in preventing the recurrence of subglottic stenosis in patients with granulomatosis with polyangiitis (GPA), a type of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), according to an article published in the European Journal of Internal Medicine.
For this monocentric retrospective observational study, the researchers reviewed the medical charts of 105 patients with GPA referred to a tertiary care center between 2008 and 2021. The prevalence of subglottic stenosis in this cohort was 20%, as it was detected in 21 participants.
According to the results, patients with subglottic stenosis and GPA had an earlier disease onset (mean age 30.2 versus 47.3 years) and a lower Birmingham Vasculitis Activity Score (mean, 10.5 vs 13.5) compared with GPA patients without subglottic stenosis.
Patients who received systemic immunosuppressive treatment had a relapse rate of 44%, whereas 5 who were not administered immunosuppressants all experienced a relapse after the first subglottic stenosis dilation procedure.
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Moreover, the researchers found rituximab and cyclophosphamide reduced the need for a subsequent dilation procedure, as opposed to no medical treatment.
In patients with subglottic stenosis and generalized disease who received rituximab or cyclophosphamide-based induction treatment and higher cumulative doses of glucocorticoids, the median time to subglottic stenosis relapse was significantly extended (36 compared with 12 months).
“Our analysis also hints that early immunosuppressors retain their efficacy, but they tend to lose it as the number of procedures increases. One possible explanation might be that during follow-up fibrosis increases at subglottic stenosis level as a result of both persistent/recurrent inflammation and repeated post-dilation scars,” Moroni and colleagues added.
“However, it was not possible to identify a single regimen that was undoubtedly associated with definite remission of subglottic stenosis, nor superior to all other treatments.”
Subglottic stenosis, a potentially life-threatening manifestation of GPA, is often treated with endoscopic dilation. Although this procedure is often followed by relapses, the utility of immunosuppressive treatment for subglottic stenosis management remains unclear.
Reference
Moroni L, Giudice L, Lanzillotta M, et al. Role of systemic immunosuppression on subglottic stenosis in granulomatosis with polyangiitis: analysis of a single-centre cohort. Eur J Intern Med. Published online May 6, 2023. doi:10.1016/j.ejim.2023.05.006
