淀粉样变性病是由蛋白异常折叠后形成不溶性β－片层构型的淀粉样物发生异常沉积的一类代谢病，肾脏是最常受累的器官之一，诊断主要依赖肾穿刺病理活检。肾淀粉样变性病的临床及病理表现多样，病因学诊断及分型困难，无统一的病理学分级系统，临床缺乏有效的干预治疗。本文将对肾淀粉样变性病的病理表现、诊断及分型分级予以综述，以期为该疾病的诊断、治疗及预后评估提供依据。

Amyloidosis is a disorder caused by extracellular deposition of misfolded proteins in an insoluble beta-pleated physical format. The kidney is a frequent site of amyloid deposition. Diagnosis of renal amyloidosis is based on the renal pathology biopsy. As the clinical features and pathological manifestations of renal amyloidosis are diverse, the diagnosis and typing of renal amyloidosis can sometimes be problematic. There is not a unified pathological grading system and lacks effective clinical interventions for renal amyloidosis. In this manuscript, we described the pathological manifestations, diagnosis, typing, and grading of renal amyloidosis, so as to provide a basis for the diagnosis, treatment, and prognosis evaluation of the disease.