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A fit 18 year old rugby player presented with a four month history of progressive exertional breathlessness, preceded by an unremarkable flu-like illness. There was no response to inhaled β2-agonist, oral theophylline, or inhaled corticosteroids. At assessment, he was breathless on minimal exertion with wheeze. He had never smoked cigarettes. There was no family history of chest disease. He had worked with fibreglass and hardener for one month and had an inhalation anaesthetic for a tonsillectomy, 18 months previously. On examination, his chest was hyperinflated with decreased expansion, hyper-resonant percussion note, reduced breath sounds, and diffuse polyphonic expiratory rhonchi.
Forced expiratory volume in one second was 1.3 litres (30% of predicted), forced vital capacity 4.2 litres (79%), with a ratio of 31%. Residual volume was 3.64 litres (290%) and total lung capacity 8.65 litres (131%). Single breath carbon monoxide transfer factor was 3.38 mmol/min/kPa (29%). Chest radiography (fig 1), high resolution computed tomography of the thorax at the level of the pulmonary trunk (fig 2), and open lung biopsy (fig 3) are shown.
- What are the clinical and pathological diagnoses?
- What aetiologies should be considered?
- What treatment options are available?