肠道淀粉样变性的临床表现及诊断挑战

Clinical manifestations and diagnostic challenges of intestinal amyloidosis

淀粉样变性是一组异质性疾病,指异常纤维蛋白的细胞外沉积破坏了组织结构和功能。肠道受累是淀粉样变性的一种罕见表现,其临床表现不一,包括体重下降、慢性腹泻、腹痛、肠道出血或假性梗阻。内镜下的特点包括细颗粒状外观、糜烂、溃疡、黏膜脆性、结节样隆起或肿瘤样病变。偏光显微镜下结肠黏膜固有层中淀粉样沉积物的刚果红染色呈绿色双折射是淀粉样变性的诊断特征。该综述旨在全面讨论目前有关淀粉样变性的文献,以阐明肠道淀粉样变性的临床表现、诊断及诊断上的挑战。

Amyloidosis is a group of heterogeneous diseases in which the extracellular deposition of abnormal fibrin disrupts tissue structure and function.Intestinal involvement is a rare manifestation of amyloidosis,with clinical manifestations ranging from weight loss,chronic diarrhea,abdominal pain,intestinal bleeding,or pseudo-obstruction.Endoscopic features include fine-grained appearance,erosion,ulcers,brittle mucosa,nodular eminence,or tumor-like lesions.The Congo red staining of amyloid deposits in the lamina propria of colon mucosa under polarizing microscope shows green birefringence,which is a diagnostic feature of amyloidosis.This review aims to provide a comprehensive review of the current literature on amyloidosis in order to clarify the clinical manifestations,diagnosis,and diagnostic challenges of intestinal amyloidosis.