硬皮病消化道受累临床表现及诊治进展.doc

硬皮病消化道受累临床表现及诊治进展 Manifestations and Management Progress of Gastrointestinal Involvement in Scleroderma 胥魏 南京医科大学附属无锡市人民医院 Xu Wei Wuxi People’s Hospital Affiliated to Nanjing Medical University 通讯作者：张缪佳 南京医科大学附属江苏省人民医院Corresponding Author：Zhang Miao Jia Jiangsu Province Hospital Affiliated to Nanjing Medical University 【摘要】：约90%的硬皮病患者可出现不同程度的消化道受累，其发病机制可能与胶原蛋白沉积、自身抗体作用、平滑肌细胞炎症及萎缩、特征性纤维化有关。消化道受累的常见临床表现包括胃食管反流病、Barrett食管、胃轻瘫、假性肠梗阻、小肠细菌过度生长、便秘。早期诊断和治疗尤为关键。硬皮病患者需接受PPI治疗，可预防胃肠道反流的相关并发症，可能可以预防肺间质性疾病的发生或减慢进程。促动力剂在疾病早期更有效。越来越多的研究结果显示了抗生素治疗对于胃肠道并发症的益处。硬皮病相关的严重营养不良导致的死亡预计达到20%。 [Abstract]: It is estimated that approximately 90% of patients with scleroderma have gastrointestinal tract involvement. The pathophysiology may due to collagen deposition, autoantibodies, smooth muscle cell inflammation and atrophy, and the characteristic fibrosis. Major manifestations include gastroesophageal reflux disease(GERD), Barrett’s esophagus, gastroparesis, intestinal pseudo-obstruction, small intestinal bacterial overgrowth(SIBO), and constipation. Early recognition and treatment are important. Patients with scleroderma should receive proton pump inhibitors(PPI) treatment to prevent GER complications, and also possibly to prevent or slow the progression of interstitial lung disease(ILD). Prokinetics are more effective in the early stages of the disease. More and more research demonstrated the benefits of antibiotic therapy for gastrointestinal complications. Mortality due to significant malnutrition has been estimated to be 20%. 【关键词】：硬皮病 消化道 胃食管反流病 Barrett食管 假性肠梗阻 小肠细菌过度生长 营养不良 [Keywords]: Scleroderma Gastrointestinal tract Gastroesophageal reflux disease(GERD) Barrett’s esophagus Intestinal pseudo-obstruction Small intestinal bacterial overgrowth(SIBO) Malnutrition 硬皮病是一种以局限性或弥漫性皮肤增厚和纤维化为特征的全身性自身免疫病。病变特点为皮肤纤维增生及血管洋葱皮样改变，最终导致皮肤硬化、血管缺血。临床上，除皮肤受累外，也可影响内脏，消化道是硬皮病最常见的受累的内脏系统。大约有90%硬皮病患者，无论是局限性硬皮病和弥漫性硬皮病，均可出现胃肠道纤维化的一些表现。硬皮病累及胃肠道的发病机制与其他系统受累的机制类似。疾病早期即出现肌间神经功能异常，是由于胶原蛋白沉积和/或自身抗体的作用，随后平