Evaluation of the function of the tubular esophagus in patients with progressive systemic scleroderma based on a standardized symptom score

Abstract

The oesophagus is the internal organ most often affected in progressive systemic scleroderma (PSS). Suitable methods for demonstrating oesophageal involvement are available in only a few centres. Aim of this study was to validate a standardized symptom score for assessing oesophageal function in patients with PSS. Oesophageal symptoms of dysphagia, odynophagia (pain on swallowing), heartburn and regurgitation were assessed in 27 consecutive patients with PSS. Intensity (0 to 3 points) and frequency (1 to 4 points) of these symptoms were quantified using a standardized system (after de Dombal and Hall): points per symptom (0 to 12) and points per patient (0 to 48). Results were compared with long-term manometric recordings as reference. The control group consisted of 20 healthy volunteers matched for age and sex. Healthy controls and patients with PSS had significantly different oesophageal motility: 22 of 27 patients fulfilled the criteria of oesophageal hypomotility. Using a total symptoms score of 8 points (points per patient) as limit of normal, the sensitivity of the scoring for the diagnosis of abnormal oesophageal function was 68%, with a specificity of 100%, positive predictive value of 100% and a negative predictive value of 42%. The reported method of symptom scoring provides a valid means of positive prediction of (abnormal) oesophageal function, making manometric investigation unnecessary. Absence or an only mild degree of oesophageal symptoms does not exclude abnormal motility so that manometry must be performed in such patients.