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Pharyngoesophageal dysphagia: an under recognised, potentially fatal, but very treatable feature of systemic sclerosis

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Dysfunction of upper pharyngoesophageal region in systemic sclerosis (SSc) is infrequent, poorly recognised and poorly documented in the literature. Yet, it can have very distressing and even fatal consequences that may yet be very responsive to appropriate management. This paper documents the findings and outcome of management of a series of five patients with SSc who had pharyngoesophageal dysphagia to demonstrate the above. Following the documentation of a patient with SSc that had severe pharyngoesophageal dysphagia in 1981, this paper reports the findings in five patients with SSc presenting thereafter, having the same manifestations. Patients 1–4 who had barium swallow and fluoroscopy, demonstrated pharyngoesophageal dysfunction as basis of their symptoms. Patient 1 had fatal outcome from pulmonary haemorrhage following repeated bouts of aspiration pneumonia. Patients 2 and 3 had a long history of SSc and were on appropriate medical treatment. They developed a short history of dysphagia that resolved with additional improvements in swallowing techniques. Patient 4 had a short history of scleroderma, but severe and very distressing dysphagia that failed to respond to improved swallowing techniques alone, but responded well to the addition of medical treatment for SSc, over a 14–24 months period. Patient 5 had a short history of SSc and dysphagia that responded well to medical treatment over 6–12months. We conclude that pharyngoesophageal dysphagia in SSc, is a rare, very distressing and potentially fatal manifestation that can have a very favourable outcome with appropriate management.
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Keywords: aspiration pneumonia; pharyngoesophageal dysphagia; scleroderma

Document Type: Research Article

Publication date: November 1, 2016

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