ESOPHAGEAL DYSFUNCTION IN SCLERODERMA - RELATIONSHIP WITH DISEASE SUBSETS

Objective. To investigate the relationship between esophageal function and the extent of disease in a nonselected group of scleroderma patie nts, and to study gastric and small bowel motility in a group of scler oderma patients with more severe clinical manifestations. Methods. Eso phageal function in 125 scleroderma patients was investigated by radio logic, endoscopic, manometric, and pa-metric techniques, Ten patients also underwent gastrointestinal (GI) manometric recording, both during fasting and after a standard meal. Results. Radiologic abnormalities of the esophagus were found in 55 of 81 patients (68%) and esophagitis in 45 of 125 (36%). No significant relationship was disclosed between GI symptoms, radiologic abnormalities, esophagitis grade, and the var ious disease subsets, However, the overall incidence of endoscopic eso phagitis (irrespective of the degree) was significantly (P < 0.05) cor related with the patient subgroups, with 100% incidence of esophagitis in those having the more severe cutaneous involvement (type III). Man ometric abnormalities were documented in 80% of patients, and patholog ic reflux in 78%. The severity of esophageal abnormalities on manometr y significantly correlated with the severity of the disease, whereas n o correlations were found with pH-metric data. Ninety percent of the 1 0 female patients undergoing antroduodenal manometry displayed abnorma l findings; of these, 60% showed neuropathic, and 30% myopathic, patte rns. The latter were recorded in patients with a more severe stage of the disease (type III). Conclusion. A direct relationship was observed between scleroderma subsets and the severity of esophageal (and, prob ably, more distal gut) motor involvement. Since no correlation was fou nd between esophageal symptoms and the severity of manometric abnormal ities, manometry should be considered the single most important GI tes t to document the severity of the ''esophageal'' disease. Gastric and small bowel manometry may also offer evidence of widespread gut involv ement, and provide a rationale for a more targeted therapeutic approac h.