Research progress in pathological diagnosis, typing and grading of renal amyloidosis

doi:10.3877/cma.j.issn.2095-3216.2020.02.008

Abstract

Abstract:

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.

Key words: Renal amyloidosis, Pathological diagnosis, Typing, Pathological grading

Siyu Chen, Weixia Han, Chen Wang. Research progress in pathological diagnosis, typing and grading of renal amyloidosis[J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2020, 09(02): 86-89.

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Figures/Tables 3

References 14

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类型	临床表现	病理特征
类型	临床表现	肾脏沉积区域	免疫病理	电镜
AL型	浆细胞疾病，NS	肾脏任何区域	可分型	纤维丝
AH型	浆细胞疾病，NS	肾脏任何区域	可分型	纤维丝
AA型	慢性炎症、感染、肿瘤、家族遗传疾病，尿Pr＋＋～＋＋＋，CKD	肾脏任何区域	可分型	纤维丝
ALECT2型	CKD，尿Pr－/＋	肾脏任何部位，肾小球及肾间质为主	可分型	纤维丝
AFib型	尿Pr＋/＋＋/＋＋＋，CKD	肾小球	可分型	纤维丝
AApoAI型	尿Pr－/＋，CKD	肾髓质	可分型	纤维丝
AApoAII型	尿Pr＋＋～＋＋＋，CKD	肾小球、肾血管	可分型	纤维丝
AApoAIV型	尿Pr－，CKD	肾髓质	不可分型	纤维丝
AApoCII型	CKD＋/－，尿Pr＋＋～＋＋＋	肾脏任何区域，以肾小球沉积为主	不可分型	纤维丝
Agel型	可有神经受累，尿Pr＋/＋＋/＋＋＋	局限于肾小球，部分见间质轻微沉积	可分型	波状/层状
ALys型	干燥综合征、胃肠道症状，尿Pr＋/＋＋/＋＋＋	肾小球、肾血管、肾髓质	可分型	纤维丝
ATTR型	尿Pr＋/＋＋/＋＋＋，CKD	肾脏任何部位，肾小球及肾血管为主	可分型	纤维丝

类型	临床表现	病理特征
类型	临床表现	肾脏沉积区域	免疫病理	电镜
AL型	浆细胞疾病，NS	肾脏任何区域	可分型	纤维丝
AH型	浆细胞疾病，NS	肾脏任何区域	可分型	纤维丝
AA型	慢性炎症、感染、肿瘤、家族遗传疾病，尿Pr＋＋～＋＋＋，CKD	肾脏任何区域	可分型	纤维丝
ALECT2型	CKD，尿Pr－/＋	肾脏任何部位，肾小球及肾间质为主	可分型	纤维丝
AFib型	尿Pr＋/＋＋/＋＋＋，CKD	肾小球	可分型	纤维丝
AApoAI型	尿Pr－/＋，CKD	肾髓质	可分型	纤维丝
AApoAII型	尿Pr＋＋～＋＋＋，CKD	肾小球、肾血管	可分型	纤维丝
AApoAIV型	尿Pr－，CKD	肾髓质	不可分型	纤维丝
AApoCII型	CKD＋/－，尿Pr＋＋～＋＋＋	肾脏任何区域，以肾小球沉积为主	不可分型	纤维丝
Agel型	可有神经受累，尿Pr＋/＋＋/＋＋＋	局限于肾小球，部分见间质轻微沉积	可分型	波状/层状
ALys型	干燥综合征、胃肠道症状，尿Pr＋/＋＋/＋＋＋	肾小球、肾血管、肾髓质	可分型	纤维丝
ATTR型	尿Pr＋/＋＋/＋＋＋，CKD	肾脏任何部位，肾小球及肾血管为主	可分型	纤维丝

指标	缩写	定义	评分
淀粉样蛋白沉积的类型	GAP	无，血管周，轻微系膜区，节段系膜区，系膜毛细血管，膜性，肾小球硬化	0～6分
淀粉样蛋白沉积百分比	GA%	0，1%～10%，11%～25%，26%～50%，51%～75%，76%～100%	0～5分
血管的淀粉样蛋白沉积	VA	无，微小，局灶，中等，严重的淀粉样蛋白沉积	0～4分
间质的淀粉样蛋白沉积	IA	无，微小，局灶，中等，严重的淀粉样蛋白沉积	0～4分
间质纤维化和小管萎缩	Ifib	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分
间质的炎症性浸润	Linf	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分
肾小球硬化	GS	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分

指标	缩写	定义	评分
淀粉样蛋白沉积的类型	GAP	无，血管周，轻微系膜区，节段系膜区，系膜毛细血管，膜性，肾小球硬化	0～6分
淀粉样蛋白沉积百分比	GA%	0，1%～10%，11%～25%，26%～50%，51%～75%，76%～100%	0～5分
血管的淀粉样蛋白沉积	VA	无，微小，局灶，中等，严重的淀粉样蛋白沉积	0～4分
间质的淀粉样蛋白沉积	IA	无，微小，局灶，中等，严重的淀粉样蛋白沉积	0～4分
间质纤维化和小管萎缩	Ifib	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分
间质的炎症性浸润	Linf	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分
肾小球硬化	GS	0，1%～10%，11%～25%，26%～50%，51%～100%	0～4分

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