We describe a case of limited Wegner’s Granulomatosis, without lung or renal involvement, with a negative ANCA who presented with subglottic stenosis.
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.
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.
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.
S. Mallick, None.