Uzma Ahmad, Ijaz Ahmad, Rani Sophia, Waseem Ahmad Bashir.
Wegener`s Granulomatosis presenting with stridor.
Ann King Edward Med Uni Jan ;8(3):238-9.
A 45-year-old lady presented in causality with intermittent breathing difficulty for last 7-8 months, which had gone worse for few days. In the past she had been under physicians for this symptom and was treated with various forms of inhalers. There was no previous admission or any medical illness. On examination she was afebrile, pale, looked exhausted with marked supraclvaicular recession and alar flaring. She was unable to lie flat. She had biphasic stridor with initial Oxygon saturation of 85 % . Systemic examination was unremarkable. A chest and lateral soft tissue neck X-rays were unremarkable. Initial clinical impression was of an upper airway obstruction. She was immediately given 100 mg Hydrocortisone and continuos Oxygon inhalation. ENT opinion was requested. In ENT history she denied any hoarseness of voice, dysphagia or loss of weight. She was non-smoker with no previous surgery to upper aerodigestive tract or trauma. Examination with felxible nasendoscope revealed normal and mobile vocal cords. Rest of the head and neck examination was normal. Initial clinical diagnosis of subglottic obstruction with possibility of tumour. A coronal laryngotracheo-tomogram showed significant narrowing of upper tracheal lumen. Blood tests showed white cell count 11/dl, Hb 10 g/dl, ESR 60 and CRP 45. Urinalysis and renal and liver function tests were normal: For further assessment she underwent laryngo-bronchoscopy under general anesthetic. This revealed granular mass extending from just below the subglottis area of larynx cords to upper 2-3 cm of trachea. Biopsy of this tissue showed chronic inflammatory cells with granuloma formation and blood vessels with narcotizing fubrinoid vasculitis, features consistent with WG. She was refereed to clinical immunologist. At this stage anti-neutrophil cytoplasm antibody assay (ANCA) was positive cytoplasmic pattern i.e. c-ANCA. Treatment was started as cyclophosphamide 50 mg TDS and prednisilone 30 mg a day. She showed significant improvement in her breathing. Repeat CRP and ANCA titres also followed the clinical improvement. Prednisilone was stopped after 2 mnoths and cyclophospamide after 18 months.
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