An unusual case of rapid radiological progression of bullous emphysema secondary to severe α1-antitrypsin deficiency

A 68-year-old ex-smoker with known emphysema secondary to severe α1-antitrypsin deficiency (A1ATD) PiZZ phenotype presented with an abrupt onset of worsening breathlessness. His emphysema had shown only mild progression over the years with a forced expiratory volume in 1 s of 1.36 L (38.2% predicted) in 2007 compared with 1.23 L (37.2% predicted) in 2012, and KCO of 0.73 (54.1% predicted) in 2007 vs 0.61 (46.7% predicted) in 2012. His condition had rarely been exacerbated and he was able to function independently until the episode. CT of the chest on admission demonstrated the known pan-acinar emphysema and no evidence of a pulmonary embolism. Strikingly, however, there …