Research

Original Investigation

Long-term Outcome of Airway Stenosis in Granulomatosis

With Polyangiitis (Wegener Granulomatosis)
An Observational Study
Marcos Martinez Del Pero, MD, FRCS (ORL-HNS); David Jayne, MD, FRCP; Afzal Chaudhry, PhD, FRCP;
Pasupathy Sivasothy, FRCP; Piyush Jani, FRCS (ORL-HNS)

IMPORTANCE Airway stenosis occurs in patients with granulomatosis with polyangiitis (GPA
or Wegener granulomatosis). It produces significant morbidity and contributes to mortality.

OBJECTIVE To investigate the frequency and distribution of airway stenoses in GPA and
evaluate the efficacy of local interventions in maintaining airway patency.

DESIGN, SETTING, AND PARTICIPANTS A retrospective single-center study of 44 patients with

GPA and airway stenosis assessed and treated as needed by a multidisciplinary team at a
university medical center between 1997 and 2012. The median duration of observation for
each patient from the time of diagnosis was 146 months.

INTERVENTIONS Patients who had critical stenoses underwent either dilatation or laser radial
cuts to the lesion. In some cases, intralesional administration of steroids or topical mitomycin
C was used.

MAIN OUTCOMES AND MEASURES The main outcome measure was airway patency for at least
12 months and the number of interventions required to achieve this end point. Details of
patients and interventions were recorded at baseline and at each treatment.

RESULTS The median age at diagnosis was 37.6 years; 73% of patients were women (n = 34).
The median follow-up after the initial intervention was 62.5 months. Subglottic stenosis was
found in 36 patients, lower airway stenosis in 30. There were 213 interventions in 39 patients,
including balloon and bougie dilatation and laser treatment. Adjuvant local treatment was
used in 71 interventions. A 12-month period of airway stability was achieved in 34 of 36 cases
(97%) (5 had no procedures and 3 had follow-up shorter than 12 months). The median
interval between procedures was 4.9 months, and after the last intervention recorded,
patients had at least 27 months of airway stability. Fourteen adverse events were recorded
(6.6%).

Author Affiliations: Department of

CONCLUSIONS AND RELEVANCE The frequency and distribution of airway stenoses in 44
Otolaryngology, Addenbrooke’s
patients with GPA has been described. In the 39 patients who required intervention, multiple Hospital, Cambridge University
procedures were required, but 97% then achieved a prolonged period of airway patency. The Hospitals NHS Foundation Trust,
procedures and adjuvant treatments were found to be safe. Our experience with a variety of Cambridge, England (Martinez Del
Pero, Jani); Department of
techniques in this rare presentation has permitted design of a structured approach and an Nephrology and Vasculitis,
algorithm to manage and evaluate airway stenosis in GPA. Addenbrooke’s Hospital, Cambridge
University Hospitals NHS Foundation
Trust, Cambridge, England (Jayne,
Chaudhry); Department of
Respiratory Medicine, Addenbrooke’s
Hospital, Cambridge University
Hospitals NHS Foundation Trust,
Cambridge, England (Sivasothy).
Corresponding Author: Marcos
Martinez Del Pero, MD,
FRCS (ORL-HNS), Department of
Otolaryngology, Box 48,
Addenbrooke’s Hospital, Hills Road,
JAMA Otolaryngol Head Neck Surg. 2014;140(11):1038-1044. doi:10.1001/jamaoto.2014.2430 Cambridge, CB2 0QQ, England
Published online October 16, 2014. (marcos@doctors.org.uk).

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 Granulomatosis With Polyangiitis (Wegener’s)
G
ranulomatosis with polyangiitis (GPA or Wegener
granulomatosis) is a primary systemic vasculitis syn-
drome that predominantly affects the lungs, kidneys,
and otorhinolaryngologic (ENT) system. More than 70% of pa-
tients with GPA have ENT involvement at follow-up.1-4 Sub-
glottic stenosis (SGS) is a less common, but serious, manifes-
tation of ENT disease,5 and it is different from other ENT
manifestations in that it can present at any time regardless of
disease activity; it is the most common manifestation of ENT
disease in children6,7; and it follows a relapsing course often
refractory to standard medical therapy.
Subglottic stenosis can also present as part of more exten-
sive airway stenosis affecting the lower airways. Other pulmo-
nary manifestations of GPA include lung nodules, cavities, and
hemorrhagic alveolitis.8 In a large case series, Cordier et al9 found
that 17% of patients had endobronchial stenosis.
Traditionally, airway stenoses in GPA are treated with im-
munosuppression followed by local interventions for persis-
tent symptomatic stenoses. Some authors have suggested that
stenoses should be treated during active disease with dilata-
tion and intralesional glucocorticoids.10 Other local interven-
tions include dilatation with balloons or bougies 11 ; laser
resection,12 cryotherapy, or Argon-plasma coagulation. Topi-
cal mitomycin C has been used to reduce recurrence rates.13,14
Prompt medical therapy combined with local interventions has
reduced the need for tracheostomies and stents.10,15 Stents are
generally avoided because they contribute to persistent in-
flammation and disease reactivation.16 Sleeve excision of
the stenosis and end-to-end tracheal repair has also been
reported7,12,17 but is uncommonly performed.
The purpose of this study was to determine whether patients
with airway stenosis and GPA reach complete airway stability and
to use the data from the disease course as well as published data
to establish a strategy for the management of this condition.
Methods
Addenbrooke’s Hospital registered this retrospective cohort
study project in the research and development department and
decided that it did not require ethical approval.
Patients
The medical records were evaluated for all patients with air-
way stenosis and a diagnosis of GPA treated between 1997 and
May 2012 by a multidisciplinary team in our tertiary referral
center. The diagnosis of GPA was applied according to cur-
rent classification guidelines.18 The demographic data of the
patients were recorded, and a database was designed to record
the distribution of the lesions, intervention details, and pul-
monary function test results.
Data Collected
The data collected encompassed all interventions from diag-
nosis until May 2012. The general management of these pa-
tients is detailed in Table 1. The decision for active local inter-
vention was based on the patients’ clinical progress (ie,
breathlessness, reduced lung capacity or walking distance, and
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Original Investigation Research
infection distal to the stenosis) and the presence of active vas-
culitis. Medical treatment was attempted in all patients be-
fore local intervention unless they had critical stenoses.
Beginning in 2007, stenoses in the distal main bronchi
and their divisions were treated with balloon dilatation
under sedation, and laser and bougies were used for proxi-
mal stenoses (subglottis, trachea, and main bronchi). A
combination of general anesthesia or sedation was used
between 1997 and 2007. Currently, intralesional glucocorti-
coid administration is used as an adjuvant therapy in active
stenoses, and mitomycin C in fibrosed recurrent stenoses.
Active stenosis is diagnosed clinically in the presence of fri-
able irregular tissue, and scarred, pale, nonfriable lesions
are considered fibrosed stenoses.
Pulmonary function was assessed using the Empey in-
dex, calculated as the forced expiratory volume in 1 second
(FEV1) (milliliters per second) divided by the peak expiratory
flow rate (PEFR) (liters per minute), and the incremental shuttle
walk test. An Empey index greater than 10 is considered ab-
normal. The walking test assesses the distance a patient walks
with incremental speed between 2 fixed points, and an in-
crease of 47.5 m is considered symptomatically significant.19
The aim of therapy was to achieve a 12-month period of stable
airway patency defined by stability on direct visualization and
clinically.
Analysis
Continuous data are summarized by medians and interquar-
tile ranges (IQRs), while categorical and nominal data are sum-
marized using percentages. Stable airway patency was de-
fined as a period of 12 months or more of follow-up without
intervention. Patients were subcategorized according to the
sites affected: subglottic stenosis (SGS) only; main airways (SGS
and/or trachea and main bronchi); secondary bronchi only; and
widespread (main airways and secondary bronchi affected).
Despite the large sample size compared with previous
cohorts,10,15,20 the data were considered too heterogeneous for
meaningful statistical analysis of outcomes.
Results
Patient Characteristics and Disease Manifestations
Forty-four (17.5%) of 251 patients with GPA seen at our insti-
tution had airway stenosis. The median age at diagnosis of air-
way stenosis was 37.6 years, and 73% were women (n = 34)
(Table 2).21
Over half of the patients had localized disease (ENT and/or
lower airway stenosis only), and a quarter had renal involve-
ment. Four of 5 myeloperoxidase–antineutrophil cytoplas-
mic antibody (MPO-ANCA)–positive patients had localized dis-
ease and almost all ANCA-negative patients (10 of 11) had lower
airway involvement and constitutional symptoms without re-
nal disease. Only 1 of the ANCA-negative patients presented
with isolated SGS and constitutional symptoms, and the di-
agnosis was made by clinical assessment and biopsy.
The median time from diagnosis of GPA to diagnosis of en-
dobronchial disease was 23 months (IQR, 0-69 months), with
1039
 Research Original Investigation Granulomatosis With Polyangiitis (Wegener’s)

Table 1. Overview of the Management Strategy for Airway Stenosis in Granulomatosis With Polyangiitisa

Local Interventions
Physical Medical Anti-infective Therapy Systemic Therapy
Balloon dilatation with Glucocorticoids Prolonged antibiotic therapy Standard therapy:
or without cutting for recurrent or persisting
Mitomycin C bacterial infections guided by Induction
Bougie antibiotic sensitivities Cyclophosphamide and
Alemtuzumab glucocorticoids
Laser Topical antibiotics if recurrent
crusting Maintenance
Cryotherapy Azathioprine and/or
Antifungal agents only if methotrexate and
Diathermy positive culture glucocorticoids

Argon-plasma Lavage Refractory

coagulation Rituximab and glucocorticoids a
All the treatments run in parallel.

ticularly among the 16 patients with only 1 lesion (n = 14).

Table 2. Characteristics of Patients With Airway Stenosis
The remaining 2 patients with single lesions had tracheal
Characteristic Measured Valuea and left upper lobe stenosis. Conversely, 14 of 15 patients
Women 32 (73)
with main bronchi involvement had more than 2 lesions,
Demography, median (IQR)
and 5 had bilateral stenoses of the main bronchi. There were
Age at GPA diagnosis, y 30.1 (19.9-43.2)
23 patients with lesions in the secondary bronchi: 9 were
Age at airway stenosis diagnosis, y 37.6 (26.6-46.8)
bilateral, and two-thirds of the lesions (15 of 23) presented
Disease duration, mo 146.1 (91.4-228.8)
in the left upper lobe. Eight patients had lesions in the sub-
Immunosuppressants tried, No. 4 (3-5)
glottis, with more severe lesions in the secondary bronchi
CYC exposure, mg/kg 140 (76-199)
Treated with antibiotics 18 (41)
sparing the main bronchi.
Distribution
Interventions
ENT system 43 (98)
During the follow-up period, 213 interventions were recorded
Lungs 25 (73)
(Table 3). The most common procedure overall was balloon
Kidneys 11 (34)
dilatation without adjuvant local treatment, but patients with
Other 16 (36)
SGS only were treated with carbon dioxide laser and mitomy-
Localizedb 23 (52)
cin C. Five (11.4%) did not require intervention, and 4 of these
Early systemicc 10 (23)
had only SGS (median follow-up, 69.1 months; IQR, 33.0-87.7
Generalizedd 10 (23)
Severee 1 (2)
months). Three-quarters of the lesions in the subglottis (26 of
Serologic findings 36), two-thirds of the lesions affecting the trachea or main bron-
PR3-ANCA positive 27 (61) chi (18 of 28), and half of the lesions in the secondary bronchi
MPO-ANCA positive 4 (9) (23 of 46) received local treatment.
PR3 and MPO-ANCA positive 1 (2) The median number of interventions per patient was 2,
ANCA negative 11 (25) and only 3 patients had a single procedure (Table 3). Forty-
three interventions (20.2%) were performed for critical ste-
Abbreviations: ANCA, antineutrophil cytoplasmic antibody;
CYC, cyclophosphamide; ENT, otorhinolaryngologic; GPA, granulomatosis with noses during active GPA, with a median interval before the
polyangiitis; IQR, interquartile range; MPO, myeloperoxidase; PR3, proteinase 3. next intervention of 4.4 months (IQR, 2.4-11.5 months), and
a
Unless otherwise indicated, data are reported as number (percentage) of the the median interval of those performed during remission of
44 included patients. GPA was 5.2 months (IQR, 2.4-14.4 months). Following the
b
Affecting the upper or lower airways. last planned intervention, the median follow-up period was
c
Any organ except renal or imminent vital organ failure. 27 months. Thirteen patients (30%) required tracheostomies
d
End-organ involvement, but creatinine level less than 500 μmol/L or other during the follow-up period, and 7 of these were subse-
vital organ failure.
e
quently decannulated. After the change in management
Renal disease (creatinine level >500 μmol/L).21
strategy in 2007, 1 patient had a temporary tracheostomy
and was decannulated within 22 days.
the longest being 239 months. Twelve patients had airway ste- All but 1 patient reached at least 1 period of airway sta-
nosis at the time of diagnosis, of whom 4 had renal disease, bility (≥12 months between treatments). The median time
and 2had ENT involvement only. Ten of these patients were patients spent without interventions was 27.6 months (IQR,
women. 17.2-46.3 months), and overall, patients spent 45.7% of the
follow-up time with a stable patent airway (IQR, 28.5-84.3
Distribution of Lesions months). Five patients without interventions and 3 with
Bronchoscopic examination of the 44 patients revealed 110 short follow-up were excluded from this calculation. Most
lesions with the distribution shown in Figure 1. The median of the patients who required further treatment after more
number of lesions per patient was 2 (IQR, 1-4). Subglottic than 2 years of disease control presented with airway diffi-
stenosis was the most prevalent lesion (36 of 44, 82%), par- culties but no evident reactivation of GPA (11 patients, 12 of

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 Granulomatosis With Polyangiitis (Wegener’s)
Figure 1. Distribution of Stenotic Lesions in the Airways of 44 Patients
36 (82)
7 (16)
8 (18)
6 (14)
9 (20)
4 (9)
3 (7)
13 procedures). In addition, in 6 of these 11 patients, the
lesion requiring further treatment changed over time. Four
of 5 patients who had 2 to 4 intralesional injections with
alemtuzumab in clinically fibrotic scars had a median
intervention-free period of 42.5 months.
The results for each subgroup are listed in Table 4.
Patients in the bronchi group had a more refractory course.
This is also seen in patients in the widespread group with a
stenotic lesion in each anatomical area of the bronchial tree
(n = 9; median time with airway stability, 55.7 months; IQR,
46.9-98.7 months) and in those with SGS and stenosis in the
secondary bronchi only (n = 8; median time with airway sta-
bility, 95.7 months; IQR, 83.8-98.2 months).
Pulmonary Function Tests
Twenty patients had pretreatment assessments, and 26 had
data available for comparison before and after treatment. Data
for pretreatment walking tests were present in 9 patients, and
there were data for progress assessment for 19 patients. Be-
fore interventions, the median percentage of predicted FEV1
was 74.5% (IQR, 52.6%-83.2%); the median percentage of pre-
dicted PEFR was 45.8% (IQR, 37.1%-76.4%); the median Empey
index was 9.89 (IQR, 8.0-11.4); and the median walking dis-
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Original Investigation Research
(n=44)
12 (28) 3 (7)
15 (34)
6 (14)
Data are reported as number
(percentage) of patients with lesions
in the indicated areas; numbers sum
to more than 100% because patients
presented with multiple lesions.
tance was 395 m (IQR, 285-445 m). After treatment, there was
improvement in all the values (Table 5). The greatest increase
in PEFR and greatest reduction in Empey index were seen in
patients with SGS. The median change in walking distance was
an improvement of 25 m (16.5%) in the 6 patients who had data
before and after interventions.
Adverse Events
Major adverse events included postoperative hemorrhages
in 2 patients and self-limiting perforation of the airway due
to trauma from the bougie dilatation in 2 patients (Table 6).
Two patients developed chest infections after intralesional
injections of alemtuzumab, although they were also receiv-
ing this medication systemically. One patient developed
polypoidal granulation tissue in the area treated with topi-
cal mitomycin C, which caused mild breathlessness and
required excision using a flexible bronchoscope. One
patient had stents inserted in the main bronchi in another
hospital, and these subsequently required surgical removal
after failed attempts at controlling restenosis with systemic
treatment. Another patient died 5 months after a dilatation
with no adjuvant treatment from respiratory and renal fail-
ure secondary to refractory active vasculitis. This patient
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 Research Original Investigation Granulomatosis With Polyangiitis (Wegener’s)

had had a limited response to the dilatation, which suggests (28%-62.5%)10,17,22 and may reflect the focus on SGS in other
that there was alveolar disease. series. As in previous reports, patients with airway stenosis pre-
sented at an earlier mean age (37.6 years) (published range,
26-40 years10,12,15,17) than the general GPA population (pub-
lished range, 53-55 years23,24). However, the female predomi-
Discussion nance (73%) in our cohort is mirrored only by McDonald et al.4
This medical record review is one of the largest published se- The area most affected was the subglottis (36 of 44, 81.8%),
ries describing the airway management in GPA. The preva- and most patients with a single lesion had SGS (14 of 16). It is
lence of airway stenosis among patients with GPA in our in- not clear whether stenoses with the appearance of mature scar
stitution was 17.5% (44 of 251), similar to that reported in other tissue in GPA are fibrotic or whether there is an underlying in-
publications (12%-50%).2-4,9,15,22 Lower-airway involvement (30 flammatory process.15,25 Our results with alemtuzumab sug-
of 44, 68.2%) was higher than figures quoted in the literature gest that even in fibrotic-looking lesions there may be an un-
derlying inflammatory process.
Table 3. Characteristics of Procedures The proportion of patients who required only 1 proce-
dure was at the lower end of published data (11%-35%).10,12,15
Characteristic Measured Valuea
Continuous data, median No. (IQR) This may be explained by the long follow-up period in this study
Lesions per patient 2 (1-4) and the refractory nature of the condition. The number of pro-
Interventions per patient 3 (1-8) cedures performed (n = 213) and the fact that patients spent
Interval between procedures (median-IQR), mo 4.9 (2.3-14.1) just under half (45.7%) of their follow-up time with a stable pat-
Follow-up duration after last procedure, mo 27 (6.2-47.5) ent airway illustrates the refractory nature of the condition.
Procedures during active endobronchial diseaseb 43 (20.1) Patients with lower airway stenoses had the most refractory
Procedures during lung infectionb 66 (30.4) course (Table 4).
Methods of dilatation (n = 213)c,d The median interval between procedures was the same
Balloon dilatation 130 (60.8) whether patients were treated during disease activity or not.
Bougie dilatation 34 (15.9) Topical mitomycin C showed some benefit in patients treated
Laser dissection 24 (11.2) with thermal techniques or persistent stenoses. Our experi-
Diathermy dissection 5 (2.34) ence using carbon dioxide laser was favorable and without
Argon-plasma coagulation 5 (2.34) long-lasting adverse events. Although there are no good com-
Cryotherapy 9 (4.21)
parative trials, most authors advocate the use of mechanical
Adjuvant therapy
techniques of dilatations and/or radial incisions with intral-
Intralesional glucocorticoids 24 (9.3)
esional corticosteroid or topical mitomycin C.11,13,14,26 Initial
Topical mitomycin C 38 (14.7)
pulmonary function test results were consistent with airway
Intralesional alemtuzumab 9 (3.5)
obstruction, but 3 patients requiring treatment for symptoms
None 142 (55.0)
had normal pulmonary function findings. Pulmonary func-
Abbreviation: IQR, interquartile range. tion test results showed an improvement over time in objec-
a
Unless otherwise indicated, data are reported as number (percentage) of tive and functional measures, but more consistent data are re-
procedures.
b
quired to confirm these findings.
Infection was defined as positive cultures of bronchial lavage or sputum.
c
The incidence of adverse events was 9%, and the inci-
Six patients treated with only glucocorticoids or alemtuzumab.
d
dence of adverse events directly related to endoscopy was 5%.
A total of 53 dilatations (24.8%) were performed under general anesthesia.
The rate of airway perforations (1.3%) was comparable to other

Table 4. Patient Phenotype and Outcome According to Disease Distribution

Patients, No. (%) Local Median Proportion

Localized Disease PR3-ANCA–Positive Interventions, Follow-up, of Time With
Lesion Distributiona (n = 23) (n = 27) Median No. mo Stable Airway, %a
SGS only 4 (17.4) 11 (78.6) 1 38.8 87.1b
(n = 14)
SGS, trachea, and/or main bronchi 5 (21.7) 5 (71.4) 10 88.1 78.3
(n = 7)
Bronchi only 3 (13.0) 3 (50.0) 3.5 44.9 36.7c
(n = 6)
Widespread diseased 11(47.8) 3 (33.3) 4 83.9 94.7
(n = 17)
c
Abbreviations: ANCA, antineutrophil cytoplasmic antibody; PR3, proteinase 3; Two patients excluded: 1 had no interventions, and 1 had follow-up shorter
SGS, subglottic stenosis. than 12 months.
a d
Calculated from the sum of months with airway stability over total follow-up Any combination of main airways and secondary bronchi.
period.
b
Six patients excluded: 4 had no interventions, and 2 had follow-up shorter
than 12 months.

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 Granulomatosis With Polyangiitis (Wegener’s) Original Investigation Research

reports (0%-4%).10,11,15,26 One patient had a complication di-

Table 5. Pulmonary Function Testsa
rectly attributable to mitomycin C (1 of 213, 0.5%) and re-
quired further intervention. Mitomycin C carries a risk of Difference From
Predicted Change, % Change
scarring,10,13,27-29 but our results show that the risks can be re- in Empey
Lesion Distribution FEV1 PEFR Index, %
duced when this treatment is used with caution, ie, no more SGS only 8.5 64.31 −28.72
than 4 applications of 0.5 mg each for less than 60 seconds. (n = 8)
Seven of 13 patients who required tracheostomies were de- Main airways 5.67 10.16 −11.30
(n = 6)
cannulated. This compares favorably with other published co-
SGS, trachea, and/or 29.32 21.00 −13.38
horts where the tracheostomy rates vary from 0% to 42%, of main bronchi
which 2.3% to 100% are permanent.10,12,15,26 (n = 4)
The long follow-up in the present study allows the Widespread diseaseb 4.11 15.17 −13.53
(n = 12)
authors to draw useful conclusions on the course of the dis-
Abbreviations: FEV1, forced expiratory volume in 1 second; IQR, interquartile
ease. However, a larger sample to enable comparison
range; PEFR, peak expiratory flow rate.
between treatment groups would have been advantageous, a
Change measured as initial test result minus the last test result. The median
and more complete data on pulmonary function tests to (IQR) time from procedure was 13.57 (IQR, 3.07-43.6) months.
assess functional outcomes would be desirable. The man- b
Any combination of main airways and secondary bronchi.
agement and outcome analysis of airway stenosis in GPA is
complex, as demonstrated by the fact that recurrence of
Table 6. Adverse Events and Outcomes
stenosis is independent of disease activity and that over
time the site of stenosis requiring treatment may change. Adverse Event Outcome
Different outcome measures such as airway diameter, func- Infection Treated successfully; 1 mucus plug removed
(n = 5) endoscopically; 1 lung infection after
tional and symptomatic assessments, and intervals between alemtuzumab treatment; 3 chronic lung
procedures are not entirely satisfactory. Airway diameter infections following several courses of treatment
and functional and symptomatic assessments lack consis- Polyps after treatment Excised with flexible bronchoscope
(n = 1)
tency, and interprocedure interval measurements are Bleeding Self-limiting
blurred in those patients who have staged procedures (eg, (n = 2)
the overall median interval in the present study was 4.9 Perforation Healed spontaneously
(n = 2)
months, while the median intervention-free period after the
Allergy to fentanyl No severe effects
last planned procedure was 27 months). Using 12-month air- (n = 1)
way stability as the outcome seemed sensible for this data Stent complications Both stents were removed; 1 of the patients later
set, but the addition of a patient-reported outcome measure (granulation and developed bronchopleural fistula
displacement)
would be useful. Using our data and those of previous (n = 2)
reports,10,14,15,30 we developed an algorithm for the assess- Death Patient died of pneumonia and active pulmonary
(n = 1) vasculitis 5 months after last intervention
ment and management of these patients (Figure 2).

Figure 2. Algorithm for Managing Patients With Granulomatosis With Polyangiitis

and Suspected Airway Stenosis

Assessment
Symptoms and disease activity
Dynamic spirometry and incremental shuttle test
Nasendoscopy and bronchoscopy
Microbiology of nose, throat, BAL, and brushings
Chest CT scan

Local intervention Antimicrobial therapy Systemic vasculitis therapy

Subglottic, tracheal, or Secondary bronchi or

severe and/or recurrent moderate and/or unilateral
main bronchial stenosis main bronchial stenosis

Laser and/or bougie Balloon dilatation

dilatation cryotherapy

Chronic and/or fibrotic Active lesion,

lesion, topical mitomycin C intralesional glucocorticoid

No Yes
Reassess Improvement Periodic reassessment
BAL indicates bronchoalveolar
lavage; CT, computed tomography.

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 Research Original Investigation
Conclusions
The frequency, distribution, and management of subglottic,
tracheal, and bronchial stenoses occurring in patients with
GPA has been described. Patients underwent various inter-
ventions, and multiple procedures were needed before a
period of stable airway patency could be achieved. Pro-
ARTICLE INFORMATION
Submitted for Publication: March 31, 2014; final
revision received June 9, 2014; accepted August
29, 2014.
Published Online: October 16, 2014.
doi:10.1001/jamaoto.2014.2430.
Author Contributions: Dr Martinez Del Pero had
full access to all of the data in the study and takes
responsibility for the integrity of the data and the
accuracy of the data analysis.
Study concept and design: Martinez Del Pero, Jayne,
Sivasothy, Jani.
Acquisition, analysis, or interpretation of data:
Martinez Del Pero, Chaudhry, Sivasothy, Jani.
Drafting of the manuscript: Martinez Del Pero,
Jayne, Sivasothy.
Critical revision of the manuscript for important
intellectual content: Chaudhry, Sivasothy, Jani.
Statistical analysis: Martinez Del Pero, Jayne,
Chaudhry, Sivasothy.
Administrative, technical, or material support:
Sivasothy, Jani.
Study supervision: Chaudhry, Sivasothy, Jani.
Conflict of Interest Disclosures: None reported.
Funding/Support: This project was supported by
the Cambridge Biomedical Research Centre.
Role of the Sponsor: The Cambridge Biomedical
Research Centre had no role in the design and
conduct of the study; collection, management,
analysis, and interpretation of the data;
preparation, review, or approval of the manuscript;
and decision to submit the manuscript for
publication.
Previous Presentation: Data reported herein were
presented as an oral presentation at the
Laryngology and Rhinology Free Paper Session of
the Royal Society of Medicine; February 1, 2013;
London, England.
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