CM: A 17-year-old man presented to the Emergency Department with chest tightness, cough and left-sided chest pain. He had been diagnosed with mixed connective disease as a child, having had Raynaud's syndrome associated with a positive antinuclear antibody with a speckled pattern and a positive ribonucleoprotein (RNP) antibody. He had never experienced respiratory symptoms or exercise limitation before. He had never smoked tobacco or cannabis, or been exposed to industrial dusts or chemicals. A chest radiograph revealed a spontaneous left-sided pneumothorax. A chest drain was inserted, with rapid resolution of the pneumothorax. Eleven days later, he developed a spontaneous right-sided pneumothorax, which failed to resolve with a 12Ch seldinger drain. A CT scan of the chest was performed (figure 1).

AP: The CT chest shows a large residual pneumothorax with mediastinal shift, drain in situ, and extensive, mainly subpleural, cystic change in both lungs, upper and lower lobes. This suggests advanced emphysema. I see that the α-1 anti-trypsin level is normal, therefore a lung biopsy should be obtained to further characterise his disease and search for a cause.

CM: The patient underwent a right video-assisted thoracoscopic surgery (VATS) bullectomy and pleurectomy, with wedge lung biopsy. Three days after discharge, he …