Esophageal function scintigraphy as parameter for organ involvement of progressive systemic scleroderma

Involvement of internal organs such as lungs, heart and kidneys, is a life-threatening complication in progressive systemic scleroderma (PSS). However, the earliest and the most frequent internal manifestation of PSS is that of esophageal dysfunction. This study was undertaken to determine whether the new, sensitive and noninvasive esphageal function scintigraphy (EFS) enables us to identify patients likely to develop pulmonary interstitial fibrosis. 131 patients with PSS of different clinical types and courses underwent EFS. The esophageal clearance of a sup(99m)Tc-tagged 15 ml water bolus was measured. In normals, 91 +- 4.8% of the maximal rate were cleared from the ROI comprising the whole esophagus 10 sec after T max was reached. For comparison, chest X-rays and pulmonary function data were used. In the presence of interstitial lung disease EFS gave normal results in four patients only (sensitivity, 89%). All cases with either severely impaired lung function or signs of fibrosis on X-ray films showed esophageal clearance values below 40%. Moreover, esophageal motility disorders were seen in 32 among 43 patients with normal lung function. There was a correlationi between the stage and progression (reflected by unspecific signs of inflammation) and the incidence and severity of both lung function impairment and esophageal dysfunction. In conclusion, functional scintigraphy proved to be a very sensitive diagnostic procedure in the screening for internal manifestations of progressive systemic scleroderma. (Author)