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primary care respiratory journal (2008); 17(2): 114-116
copyright gpiag - reproduction..during the quiescent phase of the disease, independent
of an active..progressive dyspnoea and was found to have
an unusual, and often...
Primary Care Respiratory Journal
(2008);
17(2):
114-116
Copyright GPIAG - Reproduction prohibited
CASE REPORT
Subglottic stenosis as a complication of Wegener’s
granulomatosis
*Allen J Blaivas
a
, Walter Strauss
b,c
, Michael Yudd
b,d
a
b
c
d
Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System, 385
Tremont Avenue, East Orange, NJ 07018, USA
Assistant Professor of Medicine, UMDNJ - New Jersey Medical School
Chief, Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System
Medical Director, Dialysis Unit, Division of Nephrology and Hypertension, Department of Veterans Affairs, VA New Jersey Health Care
System
Received 18th January 2007; accepted 23rd October 2007
Summary
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Introduction
© 2008 General Practice Airways Group. All rights reserved.
AJ Blaivas
et al. Prim Care Resp J
2008;
17(2):
114-116.
doi:10.3132/pcrj.2008.00002
Wegener’s granulomatosis (WG) is a relatively uncommon collagen vascular disease that can lead to both upper and lower airway disease.
Subglottic stenosis is one manifestation of the airway disease and can occur even during the quiescent phase of the disease, independent
of an active inflammatory response. A high index of suspicion is necessary for this complication in patients with known WG who
complain of dyspnoea, and WG should be high on the differential diagnosis in those found to have “idiopathic” subglottic stenosis. We
report the case of a patient who presented with this problem, and we review the prevalence, diagnosis, symptoms, and treatment.
Keywords
Wegener’s granulomatosis, dyspnoea, stridor, subglottic stenosis, diagnosis
Wegener’s granulomatosis (WG) is an uncommon necrotising
vasculitis that affects the airways, sinuses, and kidneys. We
present a patient with WG on immunosuppressive therapy
who developed progressive dyspnoea and was found to have
an unusual, and often overlooked, airway complication of this
disease.
Case report
A 55 year-old white male with a past medical history of
Wegener’s granulomatosis was admitted to our hospital with
complaints of progressive dyspnoea on exertion, and stridor
that occurred only on forceful breathing. His WG had been
diagnosed approximately nine months previously, and his
presentation at diagnosis was significant for recurrent
rhinosinusitis, arthralgias, active urine sediment, and
ulcerating tracheitis. Kidney biopsy revealed crescenteric
glomerulonephritis and C-ANCA on diagnosis was 1:640. He
had been started on treatment with cyclophosphamide and
prednisone, and was still on this treatment when admitted
nine months later.
On admission, the patient denied any arthralgia or active
otorhinolaryngological manifestations of the disease. Urine
sediment was bland, and C-ANCA was only 1:80. However, in
view of the previously noted tracheitis caused by WG at
diagnosis, upper airway obstruction was suspected. Variable
extrathoracic obstruction was discovered on flow volume
loop (see Figure 1), and CT scan revealed critical narrowing in
the subglottic region extending for almost 15 mm, with the
thinnest diameter being approximately 6 x 5 mm (Figure 2).
Bronchoscopy confirmed the critical stenosis immediately
below the vocal cords. The stenotic area did not appear
inflamed on bronchoscopic examination.
The patient underwent tracheostomy to secure the airway
and he has been undergoing combination treatment with
intralesional glucocorticoid therapy and serial passage of
blunt dilators to enlarge the stenotic airway.
* Corresponding author:
Tel: (973) 676 1000 ext 1920 Fax: (973) 395 7034 E-mail: allen.blaivas@va.gov
PRIMARY CARE RESPIRATORY JOURNAL
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doi:10.3132/pcrj.2008.00002
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Subglottic stenosis as a complication of Wegener’s granulomatosis
Copyright GPIAG - Reproduction prohibited
Figure 1. Flow volume loop demonstrating variable
extrathoracic obstruction, as evidenced by blunted
inspiratory limb.
8
Figure 2. Representative CT scan slices demonstrating
severe stenosis in the subglottic region.
6
4
2
Flow (litres/sec)
0
Discussion
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-4
-6
-1
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2
3
4
Volume (litres)
Wegener’s granulomatosis most commonly affects the upper
and lower respiratory tract, although any organ system may
be involved. Airway and pulmonary complications of WG
include subglottic stenosis, tracheal and endobronchial
inflammation and stenoses, granulomatous pulmonary
nodules, and alveolar and cavitary infiltrates.
1
As was the case
in our patient, the upper respiratory tract disease commonly
presents as rhinosinusitis. Also noted at initial diagnosis was a
lower airway manifestation of WG, ulcerating tracheitis.
Subglottic stenosis (SGS) occurs in approximately 16-23%
of patients with WG sometime during the course of disease
and results from circumferential inflammation, edema, and
fibrosis that typically extends for 3 to 4 cm below the vocal
cords. The subglottic region of the trachea is particularly
susceptible to symptomatic narrowing due to the complete
ring that comprises the cricoid cartilage – in contrast to the
lower trachea which has a soft fibromembranous posterior
wall. The normal subglottic area in the adult patient has an
internal diameter of 23 mm laterally and an anteroposterior
diameter of 18mm.
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Dyspnoea on exertion and stridor are the most common
presenting complaints – 81.5% and 29.6%, respectively.
However, depending on the degree of narrowing, patients
may be asymptomatic.
2
In our case the relationship to WG
was obvious, but the differential diagnosis of SGS can include
trauma (90% of which is due to endotracheal intubation),
infections such as tuberculosis, diphtheria, or syphilis,
inflammation due to sarcoidosis or relapsing polychondritis,
chemical irritation, and obstruction by a foreign body.
3
SGS is
often seen on the flow-volume loop, with the characteristic
finding of fixed airway obstruction.
1
Our patient had the less
common finding of variable exthoracic obstruction
demonstrated on the inspiratory curve. When noted,
bronchoscopy and/or CT scan of the neck, particularly with
virtual bronchoscopy technology, may be useful in confirming
the diagnosis.
4
As seen in our patient, SGS – which is not necessarily
associated with acute inflammation – may occur during the
quiescent phase of the illness, and is caused by mature
subglottic scarring.
4
In our patient all other manifestations of
the disease were relatively controlled when the patient began
115
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AJ Blaivas
et al.
Copyright GPIAG - Reproduction prohibited
Learning Points
1.
Subglottic stenosis is a common cause of dyspnoea in
patients with Wegener’s Granulomatosis and occurs even
when systemic inflammation is well controlled.
Diagnosis can be obtained with CT scan of the neck,
preferably with virtual bronchoscopy, flow-volume loop,
and endoscopic visualisation.
Intratracheal dilatation, often with intralesional
glucocorticoids, is usually necessary when a symptomatic
narrowing is present.
Conclusion
Though uncommon, clinicians should strongly consider a
diagnosis of SGS in any patient with a known history of WG who
presents with dyspnoea. Conversely, WG must be ruled out in
any patient that is found to have SGS.
Conflict of interest declaration
None declared.
2.
3.
References
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Clinical features and therapeutic
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Summers RM, Aggarwal NR, Sneller MC, Cowan MJ, Wood BJ, Langford CA,
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Lebovics RS, Hoffman GS, Leavitt RY,
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102:1341-5.
Laryngyscope
1992;
developing the complaint of dyspnoea that was subsequently
revealed to be caused by SGS.
Although some patients respond to systemic
immunosuppressive therapy, manual dilation with
intralesional corticosteroids, often preceded by tracheostomy,
is required in most symptomatic patients. Laser resection is
not beneficial, since it usually leads to extensive scarring and
stenosis.
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