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Published on: January 21, 2020
Infracordal Stenosis: A Glucocorticoid-Responsive Subtype of Autoimmune Laryngotracheal Stenosis
Brendan Denvir1, Bridget Burgess2, Kevin Motz2
1Department of Medicine, Division of Rheumatology, School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
Objective:
As our understanding of autoimmune laryngotracheal stenosis (LTS) evolves, distinguishing patients who may benefit from systemic immunosuppression versus those needing only local treatment is increasingly important. In this study, we identify a distinct subset of autoimmune LTS characterized by edema of the inferior true vocal folds that extends to the superior aspect of the cricoid cartilage, termed "infracordal stenosis." The objective of this study is to characterize the clinical presentation and treatment outcomes of infracordal stenosis and compare it to typical autoimmune-related subglottic stenosis (AI-SGS).
Methods:
We conducted a retrospective review of patients with autoimmune laryngotracheal stenosis evaluated by both rheumatology and otolaryngology at our institution to identify two groups: patients with infracordal stenosis and those with typical AI-SGS. Data on immunosuppressive treatments and airway dilation procedures were collected. Time to first dilation was compared between groups.
Results:
Among 49 patients with autoimmune LTS, 11 had infracordal involvement. Six patients had isolated infracordal stenosis while five had concomitant subglottic involvement. Kaplan-Meier analysis showed longer time to first dilation in patients with infracordal involvement (median 792 vs. 44 days; p = 0.048). Four out of six patients with isolated infracordal stenosis required no dilations during their entire follow-up period.
Conclusion:
Among autoimmune LTS patients referred to rheumatology, those with infracordal involvement experienced longer time to first dilation compared to those with typical AI-SGS. These findings suggest that infracordal stenosis may represent a distinct, glucocorticoid-responsive phenotype within autoimmune laryngotracheal stenosis, with implications for treatment selection and multidisciplinary care.
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