[Clinical aspects, pathophysiology, diagnosis and therapy of gastrointestinal manifestations of progressive systemic scleroderma].
About 50% of patients with progressive systemic sclerosis develop significant clinical involvement of the alimentary tract. In decreasing order of frequency esophagus (75%), anorectum (50-70%), small bowel (50%), colon (40%) and stomach (40%) can exhibit characteristic morphological or functional features. Typical symptoms of reluxesophagitis and severe constipation are often reported. Beside this, diarrhea, steatorrhea and malnutrition are common complaints. Manometric and electrophysiological studies brought evidence of a neuropathy of the enteric nervous system in the early stages of the disease, resulting in disturbances of the digestive and interdigestive peristalsis and therefore e.g. leading to gastroparesis, bacterial overgrowth of the small intestine or constipation. In late PSS collagen deposition and atrophy of the smooth muscle layer of the bowel wall cause loss of function of sphincters as the lower esophageal sphincter or the anal sphincter and marked atony of parts of the intestine. The diagnostic procedures consist of esophageal manometry, 24-h pH-metry, esophageal and gastric radionuclide transit studies, H2-breath tests, barium enemas, anorectal manometry and endoscopy. Therapeutic options include H2-antagonists, proton-pump inhibitors, prokinetic drugs, octreotides and antibiotics. Nutritional supplementation and surgical interventions are often of limited therapeutic value. Finally in some cases long-term total parenteral nutrition is warranted.