202 / 412
whereas the median age at which SGS was diagnosed in
a cohort of patients with GPA was 26 years.
2
Further-
more, patients with GPA and SGS are frequently diag-
nosed as having GPA at a very young age; in fact, up to
44% are diagnosed before the age of 20 years.
13
27% of
patients with GPA-SGS in our cohort were diagnosed as
having GPA when younger than 20 years.
GERD has been implicated in the development of SGS
and has been identified as a probable precipitant of
iSGS.
14-17
However, some question the existence of a di-
rect association.
9
GERD has also been explored as a pos-
sible cause of GPA-SGS, but evidence of a definitive link
has yet to be identified.
18
The most compelling data to
date come from a study by Blumin and Johnston
19
dem-
onstrating pepsin in the larynx and trachea in 59% of pa-
tients with iSGS, but none in matched control patients.
Half of our iSGS group either had a history of, or was em-
pirically treated for, GERD, which was statistically no dif-
ferent from the comparison GPA-SGS group. Further-
more, the rate of surgical utilization between those with
a diagnosis of GERD and those without was no different
in both groups. While our results fail to demonstrate a
difference in the rate of GERD and SGS in the iSGS and
GPA-SGS groups, understanding the impact of GERD on
the development of SGS will be best accomplished through
continued prospective studies.
Operative management strategies for subglottic ste-
nosis are focused on improving the airway, either via en-
doscopic dilation of the stenosis, excision of the steno-
sis with laryngotracheal reconstruction, or bypassing the
stenosis with tracheostomy. Carbon dioxide laser resec-
tion and/or intralesional corticosteroid injection are com-
mon adjuvant treatments to endoscopic dilation. Inter-
estingly, in our series we found that laser resection was
utilized more frequently in patients with iSGS than in
those with GPA-SGS. This may in part be explained by
practices of the operating surgeon or a reluctance to use
the carbon dioxide laser if there is a possibility of active
Table 6. Endoscopic Dilation Frequency
a
Based
on Myer-Cotton Staging (MCS) at Time
of Endoscopic Dilation
Characteristic
GPA Idiopathic
P
Value
Patients undergoing endoscopic
dilation, No.
14
23
NA
Dilations with known MCS
36
50
NA
Days between procedures based
on MCS, mean, No.
1
829
358
NA
b
2
562
602
.03
3
462
477
.23
4
NA
NA
NA
Abbreviations: GPA, granulomatosis with polyangiitis; NA, not applicable.
a
Excludes surgical procedures with less than 6 months’ postoperative
follow-up.
b
Only 1 dilation in the idiopathic MCS 1 group with more than 6 months’
postoperative follow-up.
Table 4. Operative Technique at Time of Endoscopic Dilation
Characteristic, No.
GPA
Idiopathic
Combined
P
Value
Patients undergoing endoscopic dilation
14
23
38
NA
Total dilations
48
55
103
NA
Operative use of laser
15
35
50
.01
Intraoperative injections
No injection
10
5
15
.10
Triamcinolone
31
39
70
.53
Mitomycin C
1
1
2
.99
Triamcinolone and mitomycin C
6
10
16
.59
Abbreviations: GPA, granulomatosis with polyangiitis; NA, not applicable.
Table 5. Therapeutic Airway Procedure Frequency
a
Characteristic
Granulomatosis With Polyangiitis, No.
Idiopathic, No.
Patients Procedures
P
Value DBP, Mean
P
Value Patients Procedures
P
Value DBP, Mean
P
Value
All patients
15
48
557
24
46
495
NA
GERD history
Yes
4
9
.74
565
.96
12
32
.35
506
.60
No
11
39
.74
555
.96
12
14
470
Lifetime tracheotomy history
Yes
6
33
.24
367
.11
NA
NA
NA
NA
NA
No
9
15
.24
975
.11
NA
NA
NA
NA
NA
Sex
Male
6
31
.24
318
.04
NA
NA
NA
NA
NA
Female
9
17
.24
994
.04
NA
NA
NA
NA
NA
Abbreviations: DBP, days between procedures; GERD, gastroesophageal reflux disease; NA, not applicable.
a
Excludes surgical procedures with less than 6 months’ postoperative follow-up.
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