Department of Pathology, University of Utah, Salt Lake City, UT.
Department of Pathology, University of Chicago, Chicago, IL.
Am J Kidney Dis. 2018 Oct;72(4):612-616. doi: 10.1053/j.ajkd.2018.04.009. Epub 2018 Jun 19.
Amyloid nephropathy is an uncommon disease that frequently presents with reduced kidney function and proteinuria and, in developed nations, is most often associated with underlying paraproteinemia. The histologic appearance of glomerular amyloid deposition includes mesangial and capillary wall infiltration by an amorphous eosinophilic material, and features of endo- or extracapillary proliferation are not typically seen. Rare cases of crescentic injury have been reported in a subset of patients with amyloid nephropathy, particularly those with amyloid derived from serum amyloid A protein. Collapsing glomerulopathy, which like crescentic injury is associated with an extracapillary proliferation, has not to our knowledge been reported in the setting of amyloid nephropathy. We report 2 patients presenting with acute kidney injury and nephrotic syndrome found to have amyloid nephropathy with prominent epithelial cell hyperplasia and glomerular collapse on biopsy. This injury is likely multifactorial and related to direct podocyte injury and vascular compromise and expands further the spectrum of paraprotein-associated renal injury.
淀粉样变肾病是一种不常见的疾病,常表现为肾功能下降和蛋白尿,在发达国家,常与潜在的副蛋白血症有关。肾小球淀粉样沉积的组织学表现包括系膜和毛细血管壁的无定形嗜酸性物质浸润,通常没有内或外毛细血管增殖的特征。在一部分淀粉样变肾病患者中,特别是那些由血清淀粉样 A 蛋白衍生的淀粉样物质的患者中,已经报道了少数新月体损伤的病例。塌陷性肾小球病与外毛细血管增殖有关,如新月体损伤,据我们所知,在淀粉样变肾病中尚未有报道。我们报告了 2 例以急性肾损伤和肾病综合征为表现的患者,经活检发现存在明显的上皮细胞增生和肾小球塌陷的淀粉样变肾病。这种损伤可能是多因素的,与直接的足细胞损伤和血管损伤有关,并进一步扩展了副蛋白相关肾损伤的范围。