儿童原发性免疫缺陷病与炎症性肠病

Primary immunodeficiency disease and inflammatory bowel disease in children

炎症性肠病(IBD)在儿童相对少见,其发生率在原发性免疫缺陷病(PID)患者显著增高。IBD易发生于PID中的免疫失调性疾病、吞噬细胞缺陷、自身炎症性疾病,在联合免疫缺陷病、伴典型表现的联合免疫缺陷综合征中也有报道。PID伴IBD患者发病年龄早,临床表现重,内镜下表现不典型,对传统治疗方案反应差。文章仅就PID合并IBD的机制、临床表现及治疗进行综述。

The morbility of inflammatory bowel disease(IBD)is low in normal children,but is relatively high in some kinds of primary immunodeficiency diseases(PID).Some kinds of PID are easy to combine with IBD,for example,diseases of immune dysregulation,congenital defects of phagocytes,autoinflammatory disease,combined immunodeficiencies,and combined immunodeficiency syndrome with typical features.PID combined with IBD has different clinical features,for example,the small onset age,severe clinical manifestations,atypical manifestations in endoscopy,and resistance to traditional treatment.In this article we reviewed the mechanisms as well as the clinical features and treatment of PID combined with IBD.