Dikman S H, Churg J, Kahn T
Hum Pathol. 1981 Feb;12(2):160-9. doi: 10.1016/s0046-8177(81)80103-7.
Morphologic studies and clinical correlations were undertaken in 59 patients with renal amyloidosis. Spicularly arranged amyloid deposits in the glomerular capillary wall were found in all clinical groups but were more frequent and more extensive in primary amyloidosis and multiple myeloma. The severity of proteinuria correlated with the presence of spicules and podocyte destruction rather than with the amount of amyloid in the glomerulus. The spicules were associated with morphologic and clinical evidence of rapid amyloid deposition and a fulminant clinical course. The absence of spicules and the presence of extensive new basement membrane material may produce basement membrane thickening, lamination, and double capillary wall contours, which are associated with mild proteinuria and, rarely, resolution of amyloidosis. Nodular or mixed nodular-diffuse patterns of glomerular amyloid deposits were more frequent in patients with secondary amyloidosis and a longer clinical course. Renal failure generally corresponded to severe glomerular amyloidosis and tubular atrophy. However, a relatively precipitous, usually irreversible decrease in renal function frequently occurred in patients with renal amyloidosis and did not always have a morphologic explanation. The duration of life from the time of biopsy no death in patients with primary amyloidosis (nine months) was markedly shorter than in those with secondary amyloidosis (more than 50 months).
对59例肾淀粉样变性患者进行了形态学研究及临床相关性分析。在所有临床组中均发现肾小球毛细血管壁有特殊排列的淀粉样沉积物，但在原发性淀粉样变性和多发性骨髓瘤中更常见且范围更广。蛋白尿的严重程度与针状结构的存在及足细胞破坏相关，而非与肾小球内淀粉样物质的量相关。针状结构与淀粉样物质快速沉积的形态学和临床证据以及暴发性临床病程相关。无针状结构且存在大量新的基底膜物质可能导致基底膜增厚、分层及双毛细血管壁轮廓，这与轻度蛋白尿相关，且淀粉样变性很少消退。继发性淀粉样变性且临床病程较长的患者中，肾小球淀粉样沉积物的结节状或混合结节 - 弥漫性模式更常见。肾衰竭通常与严重的肾小球淀粉样变性和肾小管萎缩相对应。然而，肾淀粉样变性患者肾功能常出现相对急剧且通常不可逆的下降，且并不总是能从形态学上解释。原发性淀粉样变性患者活检后存活时间（9个月）明显短于继发性淀粉样变性患者（超过50个月）。
