Early changes in oesophageal function in progressive systemic sclerosis: a comparison of manometry and radiology

Abstract

The characteristics of oesophageal dysfunction were studied with manometry and cine radiography in a recumbent position in 21 patients with typical progressive systemic sclerosis (PSS). Manometry was also performed in a matched control group. Only one patient had a completely normal manometry. Mean resting pressure in both the upper and lower oesophageal sphincters were significantly decreased in PSS. Twelve patients had no detectable peristalsis in the lower oesophagus. In the upper oesophagus, the mean pressure amplitude of the peristalitic wave was found to be lower than normal in all patients with detectable peristalsis. In some patients, the only feature of oesophageal dysfunction observed was an increased speed of the peristaltic wave in the middle and lower oesophagus. This is interpreted as an impaired coordination of the propulsive peristalsis. Neuromuscular dysfunction of the oesophagus in its full length was thus clearly demonstrated. At cine radiography, three patients were judged as normal, and 13 patients had severe impairment of the peristaltic function in the distal two-thirds of oesophagus. Oesophageal scoring based on manometry correlated well to scoring based on radiography. Cine radiography of the recumbent patient gives adequate information for clinical purposes. Detection of early changes in the amplitude and speed of the propagation wave requires manometry.

Esophageal Involvement in Scleroderma: Clinical, Endoscopic, and Manometric Features

“…Even if, for most authors, there is no correlation between presence of esophageal symptoms and presence of EMDs [13, 21], a link was, however, established between their severity and presence of EMDs [6, 7, 22]. In this study, symptoms were significantly more frequent in presence of esophageal dysmotility and are, therefore, a simple warning sign that should prompt search of EMDs by manometry.…”

Oesophageal dysfunction in familial amyloidosis with polyneuropathy

“…Amyloidosis might have caused autonomic denervation of the oesophagus-just as it may cause autonomic denervation of blood vessels and the urinary bladder (Andersson & Bjerle, 1975,1976. Abundant amyloid deposits in the oesophageal wall (Gilat & Spiro, 1968) may stiffen the wall and inhibit muscular contractions and propulsive pressure waves in a manner analogous to the fibrosis that occurs in progressive systemic sclerosis (Blom-Bulow et al, 1984). However, our oesophageal compliance studies indicated that the oesophageal wall of FAP patients was not stiffened.…”

A Case of Chronic Intestinal Pseudo-Obstruction

“…Manometry studies in early scleroderma show increased velocity of the peristaltic wave, discordance of the peristaltic wave with LES relaxation, and failure of the LES to relax to baseline. Later findings include decreased amplitude of peristalsis and low LES resting pressure progressing to aperistalsis in the smooth muscle portion of the esophagus with absent LES resting pressure 14,15 . Gastroesophageal reflux disease (GERD) is the most significant clinical condition.…”