October 2004, Vol 126, No.

4_MeetingAbstracts
Abstract: Case Reports | October 2004

Limited Wegner’s Granulomatosis A Case

Report of Diagnosis and Successful
Treatment of Subglottic Stenosis
Srikumar Mallick, MBBS*; Amar Shimel, MRCP; Ricky Jones, FRCP; Sudipta Roy, MBChB

Warwick General Hospital, Warwick, England

Chest

Chest. 2004;126(4_MeetingAbstracts):1001S-1002S.
doi:10.1378/chest.126.4_MeetingAbstracts.1001S

Abstract
INTRODUCTION: We describe a case of limited Wegner’s Granulomatosis, without lung or
renal involvement, with a negative ANCA who presented with subglottic stenosis.

CASE PRESENTATION: A 37 year old non-smoking male machine operator who used to be a
active sportsman presented with increasing breathlessness for over a year and intermittent
epistaxis for two years. Breathlessness was associated with noisy breathing. There was no history
of rhinitis, oral ulcers, joint pain or urinary symptoms. His peak flow six months ago was
300litres/s and was diagnosed with asthma. He was started on bronchodilators with no
improvement in his symptoms. His PEFR dropped progressively and irreversibly down to
250litres/s. One month earlier he reported to A&E department for increased breathlessness and
was treated as exacerbation of asthma and was discharged on a course of steroids and
antibiotics.As his symptoms worsened he was referred to the respiratory clinic. On physical
examination, he was found to have nasal crusting and ulceration. Chest auscultation revealed
inspiratory stridor. There was no oral ulcer or skin lesion suggestive of vasculitis or erythema
nodosum. Systemic examination was unremarkable. Flow volume loop was consistent with fixed
airway obstruction (picture). He was referred to otorhinolaryngology team. Nasoendoscopy
showed ulcerated lesions with crusting and bleeding in the nose and subglottic stenosis (picture ).
Initial investigation showed normal FBC, U&E, LFT’s and CRP. His urine analysis was negative
for casts or red cells. Rheumatoid factor, ANA, ANCA were negative. Serum ACE level was
within normal range. Chest x-ray was normal. CT scan of upper airway and neck showed
localised soft-tissue thickening at the level of vocal cords causing moderate irregular narrowing
of the airway. No abnormality was demonstrated on HRCT scan of thorax. For protection of the
airway and to alleviate symptoms urgent surgical tracheostomy was done under local anaesthesia
followed by laryngoscopy and biopsy. Biopsy appearances showed mixed acute and chronic
inflammation with poorly formed granuloma and involvement of blood vessels. The appearances
were keeping with Wegener’s granulomatosis From the history and biopsy findings the patient
was diagnosed with limited Wegener’s granulomatosis. He was started on oral prednisolone 60
mg daily and intravenous Cyclophosphamide. Within five days he started feeling a lot better.
Repeat laryngoscopy on 7th day showed significant improvement. Finally he was decannulated
on the 15th day post admission. He was discharged on reducing dose of prednisolone and
monthly cyclophosphamide infusion.

DISCUSSIONS: Causes of subglottic stenosis are intubation and blunt external trauma. Other
non-traumatic and non-neoplastic causes include infection, WG, relapsing polychondritis,
laryngeal sarcoidosis, gastro-oesophageal reflux1 and amyloidosis. WG is a disease manifested
by necrotising granuloma of the upper respiratory tract(60–80%), Lung (>90% of cases),renal
involvement (70% of cases). Isolated laryngotracheal disease is rare2. Most patients present in
their 30s and 40s. In the past WG was almost always fatal,mean survival being 5 months.
Treatment with steroids and cyclophosphamide has resulted in a 5 year survival rate approaching
95%. Subglottic stenosis from WG is managed with medical therapy. Surgical management (in
those who remain symptomatic) includes intralesional steroid, manual dialatations, CO2 laser
excision and open laryngotracheoplasty.

CONCLUSION: Not all noisy breathing is asthma. Stridor is a critical sign which needs urgent
attention. Limited WG can present without lung or renal involvement and with a negative ANCA
test. Early diagnosis is important to prevent complications like subglottic stenosis which can be
life threatening3.

DISCLOSURE: S. Mallick, None.

Wednesday, October 27, 2004

2:00 PM - 3:30 PM

References
1 Bain WM, Harrington JW, et al. Head and neck manifestations of gastrooesophageal
reflux.Laryngoscope1983;94:516–519.

2 Mcdonald TJ, Neel HB, et al. WG of the subglottis and the upper portion of the
trachea.AnnOtolRhinolLaryngol.1982Nov-Dec;91(6pt-1)588–92.

3 Wojciechowski I, Piotrowski S, et al. The case of WG of the nose larynx and

ear.OtolaryngolPol.2001;55(3):323–6.