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ORIGINAL ARTICLE

Clinical Manifestations and Treatment

of Idiopathic and Wegener

Granulomatosis–Associated Subglottic Stenosis

Stanford C. Taylor, BS; Daniel R. Clayburgh, MD, PhD; James T. Rosenbaum, MD; Joshua S. Schindler, MD

Objective

To compare and contrast the manifesta-

tions and surgical management of subglottic stenosis in

patients with airway obstruction attributed to granulo-

matosis with polyangiitis (GPA), previously known as

Wegener granulomatosis, and those with idiopathic sub-

glottic stenosis (iSGS).

Design

Retrospective medical chart review. Review of

subglottic stenosis cases seen in the otolaryngology de-

partment of an academic medical center from 2005

through 2010. Data were obtained on disease presenta-

tion, operative management. and findings.

Setting

Tertiary referral center.

Participants

A total of 24 patients with iSGS and 15

patients with GPA-associated subglottic stenosis (GPA-

SGS).

Results

All individuals with iSGS were female, and 40%

of patients with GPA-SGS were male (

.01). Patients

with iSGS tended to have a higher Myer-Cotton stenosis

grade at the time of dilation than those with GPA-SGS

(

=.02). Individuals with GPA-SGS were more likely to

undergo tracheotomy as a result of disease-related com-

plications than individuals with iSGS (

.01). No pa-

tients with an open airway reconstruction in the iSGS

group required follow-up mechanical dilation. In con-

trast, all patients with open airway reconstructions in the

GPA-SGS group underwent more than 1 subsequent air-

way dilation (

.01).

Conclusions

While surgical utilization is the mainstay

of treatment in iSGS and GPA-SGS, iSGS occurs almost

exclusively in females and presents with a greater de-

gree of stenosis at the time of endoscopic dilation. In con-

trast, GPA-SGS is associated with greater rates of trache-

otomy. Open airway reconstruction may be used in the

treatment of iSGS and GPA-SGS but is much more ef-

fective in iSGS.

JAMA Otolaryngol Head Neck Surg. 2013;139(1):76-81

CQUIRED SUBGLOTTIC STE

nosis (SGS) describes air-

way narrowing in the area

of the cricoid and is asso-

ciated with prolonged in-

tubation or external trauma. Other pa-

tients acquire SGS from a systemic

autoimmune etiology, such as granulo-

matosis with polyangiitis (GPA), previ-

ously known as Wegener granulomato-

sis. However, in about 20% of cases there

is no identifiable precipitant, and pa-

tients are therefore classified as having id-

iopathic subglottic stenosis (iSGS).

While

posttraumatic SGS is often evident based

on patient history, discriminating be-

tween iSGS and GPA-SGS can be diagnos-

tically challenging, especially in cases in

which SGS is the presenting symptom of

GPA. Presenting symptoms of dyspnea,

noisy breathing, and voice changes can oc-

cur in both groups.

2,3

Anatomically, the co-

horts appear different, as scar formation

in patients with GPA-SGS and iSGS typi-

cally involves soft tissue (membranous ste-

nosis), but intubation and trauma-

related SGS typically leads to firm,

cartilaginous scar tissue.

GPA-SGS is di-

agnosed based on the presence of autoan-

tibodies or characteristic findings on bi-

opsy, but negative results cannot reliably

rule out autoimmune-mediated SGS, es-

pecially when the disease has limited or-

gan involvement.

5,6

Indeed, given themany

similarities between patients with iSGS and

those with GPA-SGS, some have sug-

gested that iSGS may represent a spec-

trum of autoimmune-mediated SGS that

is not yet fully understood and that GPA-

SGS is but a part of this autoimmune SGS

spectrum.

Despite the similarities noted between

iSGS and GPA-SGS, there is a paucity of

data in the literature directly comparing

these entities, specifically with regard to

therapeutic and surgical outcomes. Given

Author Af

Medicine (

Departme

Otolaryng

Surgery (D

Schindler)

Arthritis a

Diseases,

Medicine (

Oregon He

University,

Author Affiliations:

School of

Medicine (Mr Taylor),

Department of

Otolaryngology–Head and Neck

Surgery (Drs Clayburgh and

Schindler), and Division of

Arthritis and Rheumatic

Diseases, Department of

Medicine (Dr Rosenbaum),

Oregon Health and Science

University, Portland.

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

WWW.JAMAOTO.COM

Reprinted by permission of JAMA Otolaryngol Head Neck Surg. 2013; 139(1):76-81.

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