ESOPHAGEAL FUNCTION IN SYSTEMIC-SCLEROSIS - A PROSPECTIVE EVALUATION OF MOTILITY AND ACID REFLUX IN 36 PATIENTS

Systemic sclerosis (SSc) is a connective tissue disorder which frequen tly involves the esophagus, with severe gastroesophageal reflux (GER) and dysphagia as clinical consequences of esophageal dysmotility. The relationship between the severity and extent of esophageal acid exposu re and the specific manometric disturbances has received little attent ion. Similarly, a paucity of manometric data exists regarding pharynge al/upper esophageal sphincter (UES) function in SSc patients. We prosp ectively studied 36 SSc patients using computerized solid-state manome tric and ambulatory dual-pH (upper and lower esophageal) monitoring, t o define further the relationship between esophageal dysmotility and s everity of GER in these patients. Patients were separated for analysis into two subgroups based on the absence (group 1, N = 25) or presence (group 2, N = 11) of distal esophageal peristalsis. SSc disease varia nt (diffuse vs. limited) and duration of illness were inaccurate predi ctors of the presence and severity of esophageal involvement. The mean lower esophageal sphincter (LES) pressure for the SSc patients (15.8 +/- 1.2 mm Hg, mean +/- SE) was significantly lower (p < 0.01) than th at for a control group (26.0 +/-2.1 mm Hg). There was no significant d ifference between the mean LES pressure for group 1 (15.0 +/- 1.6 mm H g) and group 2 (17.5 +/- 1.6 mm Hg) patients. Although distal esophage al aperistalsis was noted in 70% of patients, normal proximal esophage al contraction pressures were documented in all cases. Mean UES pressu re was significantly (p < 0.01) lower in group 1 (52.5 +/- 4.6 mm Hg) than in group 2 (80.5 +/- 10.6 mm Hg). The mean duration of UES relaxa tion and the mean time interval between the onset of UES relaxation an d onset of pharyngeal contraction were significantly (p < 0.05) shorte r for group 1 than group 2 patients. Pharyngeal pressures, peristalsis , and other aspects of pharyngeal/UES coordination were normal. Excess ive distal esophageal acid exposure was often seen in patients in both subgroups, but it was significantly (p < 0.01) greater in group I. Ex cessive proximal esophageal acid exposure was documented only in patie nts with absent distal peristalsis. Linear regression analysis reveale d a poor correlation between the severity of esophageal acid exposure and the LES pressure. Thus, the severity and extent of GER in SSc is m ost closely related to the integrity of distal esophageal peristalsis.