Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis

Abstract

Objectives/Hypothesis: Idiopathic subglottic stenosis (iSGS) is a rare and potentially life-threatening disease marked by recurrent and progressive airway obstruction frequently requiring repeated surgery to stabilize the airway. Unknown etiology and low disease prevalence have limited the ability to characterize the natural history of iSGS and resulted in variability in surgical management. It is uncertain how this variation relates to clinical outcomes. Study Design: Medical record abstraction. Methods: Utilizing an international, multi-institutional collaborative, we collected retrospective data on patient characteristics, treatment, and clinical outcomes. We investigated variation between and within open and endoscopic treatment approaches and assessed therapeutic outcomes; specifically, disease recurrence and need for tracheostomy at last follow-up. Results: Strikingly, 479 iSGS patients across 10 participating centers were nearly exclusively female (98%, 95% confidence interval [CI], 96.1–99.6), Caucasian (95%, 95% CI, 92.2–98.8), and otherwise healthy (mean age-adjusted Charlson Comorbidity Index 1.5; 95% CI, 1.44–1.69). The patients presented at a mean age of 50 years (95% CI, 48.8–51.1). A total of 80.2% were managed endoscopically, whereas 19.8% underwent open reconstruction. Endoscopic surgery had a significantly higher rate of disease recurrence than the open approach (chi2 = 4.09, P = 0.043). Tracheostomy was avoided in 97% of patients irrespective of surgical approach (95% CI, 94.5–99.8). Interestingly, there were outliers in rates of disease recurrence between centers using similar treatment approaches. Conclusion: Idiopathic subglottic stenosis patients are surprisingly homogeneous. The heterogeneity of treatment approaches and the observed outliers in disease recurrence rates between centers raises the potential for improved clinical outcomes through a detailed understanding of the processes of care.

Idiopathic subglottic stenosis: an epidemiological single-center study

“…As in other studies [1–3, 8, 11–14], this study demonstrated homogeneity among ISS patients, who were mainly healthy females of European descent, diagnosed in their 50 s and requiring multiple procedures. The pathophysiology of the condition is unclear, but the age at diagnosis of approximately 50 years coincides with onset of menopause, supporting the hypothesis that hormones influence the etiologic process [1, 4, 11, 15].…”

Outcomes after cricotracheal resection for idiopathic subglottic stenosis

“…Regardless of etiology, the treatment goals center around creating an open and lasting airway and are accomplished surgically. Despite the relative homogeneity of the disease presentation and population, the treatment of iSGS remains quite variable on a spectrum from endoscopic management with balloon dilation to cricotracheal resection (CTR) . Despite CTR having less recurrence than endoscopic management, there can be significant morbidity associated with undergoing the open procedure.…”

“…Such a rare disease has proven difficult to ascertain meaningful answers to research questions in the past. Recently, the North American Airway Collaborative (NoAAC) established a multi‐institutional collection of data for iSGS to help elucidate answers . Interestingly, there is significant institutional variability with treatment choice and criteria for selecting patients to undergo CTR versus endoscopic excision with balloon dilation .…”

Hormone receptors analysis in idiopathic progressive subglottic stenosis

“…An intense episode of coughing, the presence of mycobacterial infections, and gastroesophageal reflux (GERD) laryngitis in fact have been proposed as possible triggers . Although presence of such purported triggers is inconstant, the prevalence of clinical GERD in the IPSS series has been reported to be similar to the one of the general population . Yet, the presence of pepsin was identified in the larynx of nearly two‐thirds of a further series .…”