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Table of Contents
Year : 2019  |  Volume : 9  |  Issue : 2  |  Page : 57-59

Supraglottic stenosis: A rare presentation of granulomatosis with polyangiitis

1 Department of Laryngology, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India
2 Department of Pathology, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India

Date of Submission31-Dec-2018
Date of Acceptance03-Jun-2020
Date of Web Publication14-Aug-2020

Correspondence Address:
K C Arun
Department of Laryngology, Deenanath Mangeshkar Hospital, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jlv.JLV_27_18

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Granulomatosis with polyangiitis is a small-vessel vasculitis involving the airway, lungs, and kidney. Although subglottic involvement is common in the disease, we present a rare case of supraglottic involvement. The patient presented to us with a complaint of change in the voice for 3 months. Endoscopy showed concentric narrowing of supraglottis. Biopsy revealed small-artery vasculitis. c-ANCA was positive. The Patient responded well to medical therapy.

Keywords: c-ANCA, granulomatosis with polyangiitis, supraglottis

How to cite this article:
Gandhi SS, Arun K C, Puntambekar D. Supraglottic stenosis: A rare presentation of granulomatosis with polyangiitis. J Laryngol Voice 2019;9:57-9

How to cite this URL:
Gandhi SS, Arun K C, Puntambekar D. Supraglottic stenosis: A rare presentation of granulomatosis with polyangiitis. J Laryngol Voice [serial online] 2019 [cited 2023 Mar 20];9:57-9. Available from: https://www.laryngologyandvoice.org/text.asp?2019/9/2/57/291928

   Introduction Top

Granulomatosis with polyangiitis (Wegener's) (GPA) is a small-vessel necrotizing granulomatous vasculitis. The ear, nose, and throat, lung, and kidney are the most frequently involved organs (90%, 90%, and 80% patients, respectively).[1] Tracheobronchial stenosis is a potentially serious manifestation of GPA that usually respond poorly to corticosteroids and immunosuppressive agents. Tracheobronchial involvement, a less common GPA manifestation, comprises of stenosis of the tracheobronchial tree, which can lead to not only upper airway obstruction and potentially severe functional but also life-threatening consequences.[2] Subglottic stenosis is the most frequent tracheobronchial stenosis type that could be related to anatomic specificities and pathophysiological mechanisms.[3] We are discussing supraglottic stenosis in GPA which is a very rare entity.

   Case Report Top

A 36-year-old male came to Voice Clinic with chief complaints of change in the voice for 3 months and gradually, progressive difficulty in breathing of the same duration with no history of difficulty swallowing. None of the family members have a similar illness. There was no history of trauma, smoking, tobacco, or alcohol consumption in the past. He was diagnosed with retinal vasculitis and circinate balanitis in the past. The patient was treated with a provisional diagnosis of Behcet's disease.

Flexible fiberoptic videolaryngostroboscopy examination revealed bilateral roomy nasal cavity, fibrotic, concentric band over supraglottis involving bilateral false cords, arytenoids, and aryepiglottic folds with impaired bilateral vocal cord mobility [Figure 1]. No neck nodes were palpable and the rest of the head-and-neck examination was normal. The patient underwent direct laryngoscopy and biopsy under general anesthesia. Direct laryngoscopic findings confirmed the videolaryngoscopy findings with the firm, fibrotic, and concentric band over supraglottis extending up to glottis. Subglottis appeared normal with bilateral mobile cricoarytenoid joints on palpation. Biopsy from supraglottic tissue showed a granulomatous lesion with dense inflammatory cells with evidence of small-vessel vasculitis [Figure 2] without evidence of malignancy.
Figure 1: Supraglottic stenosis seen in videolaryngoscopy

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Figure 2: H and E stain showing small-vessel vasculitis in histopathological examination

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c-ANCA (42.5 AU/mL) and p-ANCA (38.1 AU/mL) both were positive with a raised erythrocyte sedimentation rate (ESR) (28) and negative C-reactive protein. Laboratory parameters revealed Hb 10.9 mg/dL, serum creatinine 0.7 mg %, estimated glomerular filtration rate (eGFR) 134.87 mL/min, fasting blood sugar 112 mg/dL, and postprandial blood sugar 175 mg/dL, total serum protein 7.43 g%, uric acid 3.67 mg%, and trace protein on urine analysis. High-resolution computed tomography of the chest showed normal lung parenchyma without any significant mediastinal lymph nodes. Ultrasound of the abdomen was normal. Rheumatologist confirmed the diagnosis of GPA.

The patient was started with oral prednisolone, azathioprine, and sulfamethoxazole-trimethoprim combination under rheumatologist guidance. The patient was on prednisolone 5 mg once daily, azathioprine 50 mg twice daily, and sulphamethoxazole-trimethoprim double strength to be clarified. The patient responded well to medication with acceptable voice and no difficulty in breathing. The patient is followed up every 3 months with complete blood count, ESR, serum glutamic-pyruvic transaminase, serum creatinine with GFR, and urine analysis report.

   Discussion Top

GPA is a systemic disease involving the upper and lower airway, kidney, and lungs. When multiple systems are involved, though rare, need to rule out common connective tissue disease. Subglottic stenosis is the most common manifestation of GPA in the larynx, but supraglottic stenosis is very rare with only one case reported in the literature.[4] Supraglottic stenosis is an unusual subset of laryngotracheal stenosis having different causes, associated symptoms, and treatment options. Isolated supraglottic stenosis usually found following trauma (surgical or nonsurgical), ingestion of corrosive, burns, and irradiation. Autoimmune diseases such as sarcoidosis, erosive lichen planus, and cicatricial pemphigoid are well-known causes of supraglottic stenosis.[5] The biopsy helps to identify granuloma and inflammatory process and rule out malignancy and fungal infection. Laboratory test such as c-ANCA which is very sensitive and specific to GPA aids in the diagnosis. Sometimes, c-ANCA may be negative or can become positive in the later part of the disease. The diagnosis is challenging as in our case where there is an overlap between different connective tissue diseases. Initially, he had a provisional diagnosis of Behcet's disease but later on confirmed as GPA. Treatment is basically a medical therapy with steroids and immunosuppressive drugs. Intralesional corticosteroid injections, radiate lesion cuts, and dilatation can be done to treat the majority of new stenosis.[6] Tracheostomies and stents can be done for a patient with stridor but can lead to airway complications that are difficult to treat.[7]

   Conclusion Top

Despite its rarity, the clinician must be acquainted with the atypical presentation of GPA and treat accordingly.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Jennette JC, Falk RJ. Review small vessel vasculitis. N Engl J Med 1997;337:1512-2.  Back to cited text no. 1
Daum TE, Specks U, Colby TV, Edell ES, Brutinel MW, Prakash UB, et al. Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med 1995;151 (2 Pt 1):522-6.  Back to cited text no. 2
Eliachar I, Chan J, Akst L. New approaches to the management of subglottic stenosis in Wegener's granulomatosis. Cleve Clin J Med 2002;69 Suppl 2:SII149-51.  Back to cited text no. 3
Belloso A, Estrach C, Keith AO. Supraglottic stenosis in localized Wegener granulomatosis. Ear Nose Throat J 2008;87:E11-4.  Back to cited text no. 4
Stevens MS, Chang A, Simpson CB. Supraglottic stenosis: Etiology and treatment of a rare condition. Ann Otol Rhinol Laryngol 2013;122:205-9.  Back to cited text no. 5
Nouraei SA, Obholzer R, Ind PW, Salama AD, Pusey CD, Porter F, et al. Results of endoscopic surgery and intralesional steroids for airway compromise due to tracheobronchial Wegner's granulomatosis. Thorax 2008;63:49-52.  Back to cited text no. 6
McCaffrey TV. Management of laryngotracheal stenosis on the basis of site and severity. Otolaryngol Head Neck Surg 1993;109:468-73.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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