A 41-year-old woman presented with a 4-month history of shortness of breath on exertion, with no associated cough or systemic symptoms. Her previous medical history included Raynaud’s phenomenon, and 18 months dysphagia and dyspepsia. A recent barium swallow demonstrated severe oesophageal dysmotility and inco-ordination of the gastro-oesophageal junction. She smoked (20-pack year smoking history), and was overweight (body mass index 43). Examination was normal, apart from skin changes of hands and mouth (Fig. 3.1). Pulmonary function tests (Table 3.1), a CT chest (Fig. 3.2) and an...

A 41-year-old woman presented with a 4-month history of shortness of breath on exertion, with no associated cough or systemic symptoms. Her previous medical history included Raynaud’s phenomenon, and 18 months dysphagia and dyspepsia. A recent barium swallow demonstrated severe oesophageal dysmotility and inco-ordination of the gastro-oesophageal junction. She smoked (20-pack year smoking history), and was overweight (body mass index 43). Examination was normal, apart from skin changes of hands and mouth (Fig. 3.1). Pulmonary function tests (Table 3.1), a CT chest (Fig. 3.2) and an echocardiogram were performed. She had a modestly elevated pulmonary arterial pressure at 35mmHg.