Achalasia-like syndrome in systemic sclerosis

Abstract

Two methods of quantifying oesophageal emptying for liquids have been used to assess the dysphagia of patients with systemic sclerosis: the oesophageal infusion scintiscan and the timed Gastrografin swallow. Upper gastrointestinal endoscopy and oesophageal manometry were also performed. Thirteen patients with oesophageal symptoms were studied. Eight had dysphagia, and all of these had endoscopies with no evidence of oesophagitis or stricture. Four of these eight subjects had gross delay of oesophageal emptying for fluids, and manometry showed absence of oesophageal peristalsis and incomplete relaxation of the lower oesophageal sphincter. This abnormality is similar to achalasia. Two of these four patients have benefited from pneumatic dilatation with improvement in their severe dysphagia. We believe that pneumatic dilatation should be considered in patients with systemic sclerosis and severe dysphagia where reflux oesophagitis is not apparent.