[Motility disorders of the esophagus in progressive systemic scleroderma. Pathophysiology, diagnosis and therapy]

Gastrointestinal manifestations of collagen diseases are frequent. In progressive systemic sclerosis esophageal involvement is found in 60% of cases and is thus the main gastrointestinal complication. Atrophy of the smooth muscle and fibrotic degeneration of the distal esophagus result in progressive motility disorders which may cause severe reflux esophagitis with typical consequences, such as stenosis and strictures. Manometry and cinematography are basic diagnostic procedures. Esophagoscopy and long-term pH-monitoring are most useful for evaluating the degree of esophageal involvement. The severity of sclerodermatous motility disorders should be classified according to a modification of the Garrett scale, which is particularly recommended for determining the further prognosis and therapeutic approach. Esophageal involvement of grades I and II should be treated conservatively, whereas grade III is a clear indication for surgical therapy. The original Nissen type of fundoplication or distal gastric resection with Roux-en-Y anastonosis are the methods of choice.