A 64-Year-Old Man With Rapidly Progressive Respiratory Failure and Pneumomediastinum

A 64-year-old man presented for consideration for lung transplant. He had a history of previous tobacco use, OSA on CPAP therapy, and gastroesophageal reflux disease. He worked as a design engineer. The patient had a 4-year history of dyspnea on exertion, followed with periodic CT scan of the chest. Nine months prior to his evaluation for lung transplant, the patient developed worsening of dyspnea, dry cough, poor appetite, and weight loss. At times, the cough was violent and associated with chest pressure. He was prescribed systemic corticosteroids and antibiotics without improvement. Four months later, the patient noted sudden onset of severe chest pain and worsening dyspnea. A CT scan of the chest demonstrated extensive pneumomediastinum in addition to changes consistent with pulmonary fibrosis. An esophagogram showed thickening of the distal esophagus, but no signs of perforation. He was prescribed supplemental oxygen and advised to stop the use of CPAP. The patient sought a second opinion. A CT scan of the chest showed improvement of the pneumomediastinum and extensive fibrotic lung disease. Pulmonary function tests (PFTs) were consistent with a restrictive pattern, decreased diffusing capacity (Dlco), and a preserved residual volume over total lung capacity ratio. The patient was prescribed systemic corticosteroids with no improvement of his symptoms. Repeat PFTs showed further decline of Dlco, and he was referred for lung transplant evaluation.