CLINICAL PRESENTATION, PATHOPHYSIOLOGY, D IAGNOSTIC PROCEDURES AND THERAPEUTIC STRATEGIES IN PROGRESSIVE-SYSTEMIC SCLEROSIS [PSS] WITH GASTROINTESTINAL INVOLVEMENT

About 50% of patients with progressive systemic sclerosis develop sign ificant clinical involvement of the alimentary tract. In decreasing or der of frequency esophagus (75%), anorectum (50-70%), small bowel (50% ), colon (40%) and stomach (40%) can exhibit characteristic morphologi cal or functional features. Typical symptoms of refluxesophagitis and severe constipation are often reported. Beside this, diarrhea, steator rhea and malnutrition are common complaints.Manometric and electrophys iological studies brought evidence of a neuropathy of the enteric nerv ous system in the early stages of the disease, resulting in disturbanc es 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 s mooth muscle layer of the bowel wall cause loss of function of sphinct ers 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 radionucl ide transit studies, H-2-breath tests, barium enemas, anorectal manome try and endoscopy. Therapeutic options include H-2-antagonists, proton -pump inhibitors, prokinetic drugs, octreotides and antibiotics. Nutri tional supplementation and surgical interventions are;often of limited therapeutic value. Finally in some cases long-term total parenteral n utrition is warranted.