Abstract：
Gastrointestinal involvement in systemic sclerosis can be severe, reaching the critical point of chronic intestinal pseudo-obstruction, secondary to major disorders of small bowel motility. It is associated with some clinical and biological characteristics, in particular the positivity of anti-fibrillarin/U3RNP antibodies. Chronic intestinal pseudo-obstruction (CIPO) is complicated by a small intestinal bacterial overgrowth that requires cyclic antibiotic therapy. CIPO leads to a reduction of the food intake, due to painful symptoms, nausea and vomiting caused by meals, and ultimately to severe malnutrition. Meal splitting is often transiently effective and patients require exogenous nutritional support, mostly parenteral. Systemic sclerosis is not an obstacle to initiation and long-term continuation of parenteral nutrition and central venous catheter implantation is not associated with an increased risk of cutaneous or infectious complications. However, continuation of long-term parenteral nutrition requires monitoring in an expert nutrition center in order to adapt nutritional volumes and intakes and to limit potentially fatal cardiac and hepatobiliary complications. In addition to nutrition, prokinetic treatments, whose side effects must be known, can be associated. Invasive procedures, whose risk-benefit ratio must be carefully assessed, can also be used to treat symptoms exclusively.
摘要：
系统性硬化症的胃肠道受累可能很严重，达到慢性假性肠梗阻的临界点，继发于小肠运动的主要障碍。它与一些临床和生物学特征有关，特别是抗纤维蛋白/U3RNP抗体的阳性。慢性肠道假性梗阻（CIPO）并发小肠细菌过度生长，需要循环抗生素治疗。CIPO导致食物摄入量的减少，由于痛苦的症状，由膳食引起的恶心和呕吐，最终导致严重的营养不良。膳食分裂通常是暂时有效的，患者需要外源性营养支持，主要是肠胃外。系统性硬化症并不是开始和长期持续肠外营养的障碍，中心静脉导管植入与皮肤或感染性并发症的风险增加无关。然而,长期肠外营养的延续需要在专家营养中心进行监测,以适应营养量和摄入量,并限制潜在致命性心脏和肝胆并发症.除了营养，促动力治疗，必须知道其副作用，可以关联。侵入性程序，必须仔细评估其风险收益比，也可以专门用于治疗症状。
