Case history
A 44-yr-old male carpenter presented to the outpatient clinic complaining of progressive dyspnoea and dry cough for the last 7 yrs. The patient's symptoms had worsened in the last 2 yrs and at the time of presentation he had dyspnoea at rest. He used intravenous amphetamine but stopped 12 yrs before presentation. The patient denied smoking or other previous illnesses.
Physical examination revealed an increased anteroposterior chest diameter. Auscultation disclosed diminished breath sounds and fine crackles at the lower lung fields. Respiratory rate was 24 beats per minute and pulse oximetry in room air and at rest was 90%.
A chest radiograph (fig. 1⇓) and, later, a high-resolution computed tomography were performed (figs. 2a and b⇓). Pulmonary function tests showed: forced vital capacity (FVC) 1.23 L (25%), forced expiratory volume in one second (FEV1) 0.46 L (13%), FEV1/FVC 0.37 (46%), total lung capacity 7.56 L (110%), functional residual capacity 6.31 L (166%), residual volume 6.03 …