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AS: A 74-year-old male was transferred to the Royal Brompton hospital from a local hospital with respiratory failure and expectoration of bronchial casts (see figure 1A). The patient recalls recurrent chest infections with occasional haemoptysis in his teens with no prior history of asthma, TB or pertussis. Despite investigation and antibiotic treatment, his symptoms of intermittent, chronic recurrent bronchial cast expectoration continued. A fibreoptic bronchoscopy on the patient, aged 41, revealed erythematous bronchial mucosa in the right middle lobe, with a ventilation/perfusion scan revealing concomitant decreased ventilation. Bronchial washings were unremarkable, and given concern of a pathology originating from the right middle lobe, a right middle lobectomy was performed. Histology showed non-specific inflammatory fibrosis around the lobar bronchus.
His symptoms, however, did not resolve. Microbiological examination of his casts revealed no growth, and histological examination showed fibrin and mucus with some lymphocytes. Further trials of corticosteroids, nebulised N-acetylcysteine, hypertonic saline, prophylactic azithromycin, systemic antifungals and oral cyclophosphamide were unsuccessful. Extensive immunological and allergic tests were normal.
His symptoms remained stable till age …
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