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Case based discussions
Recurrent pneumothoraces in a 17-year-old man with mixed connective tissue disease
  1. Claire McBrien1,
  2. Andrea Penketh1,
  3. Hilary Johnson2,
  4. Andrew G Nicholson3,
  5. Athol Wells4
  1. 1Department of Respiratory Medicine, Gloucestershire Hospitals NHS Foundation Trust, Cheltenham, Gloucestershire, UK
  2. 2Department of Rheumatology, Gloucestershire Hospitals NHS Foundation Trust, Cheltenham, Gloucestershire, UK
  3. 3Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  4. 4Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  1. Correspondence to Dr Claire McBrien, Department of Respiratory Medicine, Cheltenham General Hospital, Sandford Road, Cheltenham, GL53 7AN, UK; c.n.mcbrien{at}

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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).

Figure 1

CT chest, December 2011.

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 …

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