Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110, Larissa, Greece.
Division of Rheumatology, Department of Medicine, Faculty of Medicine, School of Health Sciences, University of Patras, Patras, Greece.
Dig Dis Sci. 2018 Apr;63(4):834-844. doi: 10.1007/s10620-018-4977-8. Epub 2018 Feb 21.
Systemic sclerosis (SSc) is a chronic systemic disease characterized by microvasculopathy, autoantibodies, and extensive fibrosis. Intestinal involvement is frequent in SSc and represents a significant cause of morbidity. The pathogenesis of intestinal involvement includes vascular damage, nerve dysfunction, smooth muscle atrophy, and fibrosis, causing hypomotility, which leads to small intestinal bacterial overgrowth (SIBO), malabsorption, malnutrition, diarrhea, pseudo-obstruction, constipation, pneumatosis intestinalis, and fecal incontinence. Manifestations are often troublesome and reduce quality of life and life expectancy. Assessment of intestinal involvement includes screening for small intestine hypomotility, malnutrition, SIBO, and anorectal dysfunction. Current management of intestinal manifestations is largely inadequate. Patients with diarrhea are managed with low-fat diet, medium-chain triglycerides, avoidance of lactulose and fructose, and control of bacterial overgrowth with antibiotics for SIBO. In diarrhea/malabsorption, bile acid sequestrant and pancreatic enzyme supplementation may help, and nutritional support is needed. General measures are applied for constipation, and intestine rest plus antibiotics for pseudo-obstruction. Fecal incontinence is managed with measures for associated SIBO, or constipation, and with behavioral therapies. Pneumatosis intestinalis is usually an incidental finding that does not require any specific treatment. Immunomoduation should be considered early in intestinal involvement. Multidisciplinary approach of intestinal manifestations in SSc by gastroenterologists and rheumatologists is required for optimum management.
系统性硬化症（SSc）是一种以微血管病变、自身抗体和广泛纤维化为特征的慢性系统性疾病。肠道受累在 SSc 中很常见，是发病率的重要原因。肠道受累的发病机制包括血管损伤、神经功能障碍、平滑肌萎缩和纤维化，导致运动功能障碍，从而导致小肠细菌过度生长（SIBO）、吸收不良、营养不良、腹泻、假性肠梗阻、便秘、肠积气和粪便失禁。这些表现常常令人困扰，降低生活质量和预期寿命。肠道受累的评估包括小肠运动功能障碍、营养不良、SIBO 和肛肠功能障碍的筛查。目前对肠道表现的治疗很大程度上是不够的。腹泻患者采用低脂饮食、中链甘油三酯、避免乳果糖和果糖，并使用抗生素治疗 SIBO 以控制细菌过度生长。在腹泻/吸收不良中，胆汁酸螯合剂和胰酶补充可能有帮助，需要营养支持。便秘采用一般措施，假性肠梗阻采用肠道休息加抗生素。粪便失禁采用与 SIBO、便秘相关的措施以及行为疗法进行治疗。肠积气通常是偶然发现，不需要任何特定治疗。免疫调节应在肠道受累早期考虑。需要胃肠病学家和风湿病学家对 SSc 的肠道表现进行多学科处理，以实现最佳管理。
