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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 (

= .44). Seven

patients with GPA-SGS (47%) required fewer than 2 air-

way dilations compared with 11 of those with iSGS (46%)

(

.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

(

.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 (

.01).

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.

COMMENT

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.

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.

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.

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 (

= .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,

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

of Endoscopic Dilation

Characteristic

No. (%)

GPA Idiopathic Combined

Patients undergoing endoscopic

dilation. No.

Dilations with known MCS, No.

MCS

8 (22.2)

2 (4) 10 (11.6)

16 (44.4) 14 (28) 30 (34.9)

12 (33.3) 34 (68) 46 (53.5)

Abbreviation: GPA, granulomatosis with polyangiitis.

= .02.

Table 3. Stenosis Location at Time

of First Endoscopic Dilation

Characteristic

No. (%)

GPA Idiopathic Combined

Patients undergoing endoscopic

dilation, No.

Dilations with known stenosis

location at time of first

endoscopic dilation, No.

Stenosis location

1 (9.1)

8 (42.1)

9 (30)

Posterior

1 (9.1)

2 (10.5)

3 (10)

Circumferential

9 (81.2)

9 (47.4)

18 (60)

Abbreviation: GPA, granulomatosis with polyangiitis.

= .42.

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

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