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We examined the impact of various factors on the suc- cess of airway procedures within each group ( Table 5 and Table 6 ). The presence of gastroesophageal reflux disease (GERD) and the operative use of carbon dioxide laser were not found to have an impact on the rate of sur- gical utilization. Male patients withGPA-SGS had a shorter time until additional procedures were needed than fe- male patients with GPA-SGS, while the presence of a pre- vious tracheostomy showed a nonsignificant trend to- ward worse outcomes. When patients from both cohorts (GPA-SGS and iSGS) were pooled into a single group, none of these factors (sex and history of tracheostomy or GERD) had any significant impact on time until ad- ditional procedures were needed. We analyzed the utilization of systemic immuno- therapy within both groups. All patients with GPA-SGS and 50% of iSGS individuals received systemic immu- notherapy at some point throughout follow-up as part of disease management; all patients with iSGS received corticosteroids, while patients with GPA received a mix of corticosteroids (n = 13), methotrexate sodium (n = 11), and cyclophosphamide (n = 9). Use of immunosuppres- sive medicationwas not associated with longer procedure- free intervals. When traumatic causes are not readily identifiable by pa- tient history, determining the etiology of SGS can be di- agnostically challenging. While many patients with non- traumatic SGSmay have a systemic autoimmune condition such as GPA, many others will have an unrevealing au- toimmune workup. In the absence of any identifiable cause, these patients are considered to have iSGS, al- though there is some speculation that this may be due to some unknown autoimmune mechanism. This study was conducted to better define the similarities and dif- ferences in presentation and therapeutic management of iSGS and GPA-SGS. Previously, it has been demonstrated that GPA-SGS affects men and women equally. 2 In contrast, iSGS al- most exclusively affects women 3,9,10 ; it is thought to pre- dominantly affect women owing to estrogen-mediated al- terations to wound-healing responses in the subglottic airway. 11 Our study is consistent with these observa- tions, further confirming the tendency for iSGS to dis- proportionately affect men and for GPA-SGS to affect both men and women. Interestingly, we observed that male GPA-SGS patients underwent more frequent subglottic airway surgical procedures than female patients withGPA- SGS. This may be due, in part, to previous trends noting that male patients with GPA tend to develop a more se- vere form of the disease than female patients with GPA. 12 The median age of initial presentation in patients with GPA-SGS (36.3 years) was almost 9 years younger than that of the iSGS group (45.2 years). Although not statis- tically significant ( P = .24), this finding is broadly in line with that of previous research, and we speculate that had our cohort been larger, the observations would have been significant. A study of 52 patients with iSGS found the average age of initial presentation to be 43.5 years, 9 COMMENT

Table 2. Myer-Cotton Staging (MCS) at Time of Endoscopic Dilation

No. (%) GPA Idiopathic Combined

Characteristic

Patients undergoing endoscopic dilation. No. Dilations with known MCS, No.

MCS a 1

8 (22.2)

2 (4) 10 (11.6)

2 3 4

16 (44.4) 14 (28) 30 (34.9) 12 (33.3) 34 (68) 46 (53.5)

Abbreviation: GPA, granulomatosis with polyangiitis. a P = .02.

Table 3. Stenosis Location at Time of First Endoscopic Dilation

No. (%) GPA Idiopathic Combined

Characteristic

Patients undergoing endoscopic dilation, No. Dilations with known stenosis location at time of first endoscopic dilation, No.

Stenosis location a Anterior

1 (9.1) 1 (9.1) 9 (81.2)

8 (42.1) 2 (10.5) 9 (47.4)

9 (30) 3 (10)

Posterior

Circumferential

18 (60)

Abbreviation: GPA, granulomatosis with polyangiitis. a P = .42.

Surgical treatment of SGS consisted of both endo- scopic dilation and cricotracheal resection. Endoscopic dilation technique did not vary substantially between groups, aside from less frequent use of the carbon diox- ide laser in the GPA group ( Table 4 ). Patients with GPA underwent a mean of 3.53 surgical dilations per patient compared with 2.54 in those with iSGS ( P = .44). Seven patients with GPA-SGS (47%) required fewer than 2 air- way dilations compared with 11 of those with iSGS (46%) ( P .99). Definitive operative resection or reconstruction was attempted in both groups; 5 of those withGPA-SGS (33%), and 6 of those with iSGS (25%). While no patients with an open airway reconstruction in the iSGS group re- quired follow-up mechanical dilation, all patients with open airway reconstructions in the GPA-SGS group un- derwent more than 1 subsequent airway dilation ( P .01). Following open airway reconstruction, 1 pa- tient with GPA-SGS underwent subsequent trache- otomy. Open airway reconstruction led to permanent de- cannulation of 2 previously tracheotomy-dependent patients with GPA-SGS. Six patients with GPA-SGS (40%) underwent tracheotomy as a result of disease-related com- plications and 2 (13%) remained tracheotomy depen- dent at the date of last follow-up. No patients with iSGS required tracheotomy as a result of a disease-related com- plications ( P .01).

JAMA OTOLARYNGOL HEAD NECK SURG/VOL 139 (NO. 1), JAN 2013 WWW.JAMAOTO.COM

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