Haas M, Meehan S M, Karrison T G, Spargo B H
Department of Pathology, The University of Chicago, IL 60637, USA.
Am J Kidney Dis. 1997 Nov;30(5):621-31. doi: 10.1016/s0272-6386(97)90485-6.
Data compiled during the 1970s and early 1980s indicated that during these periods, membranous nephropathy was the most common cause of unexplained nephrotic syndrome in adults, followed in order of frequency by minimal-change nephropathy and focal segmental glomerulosclerosis (FSGS). However, we and others recently reported an increase in the incidence of FSGS over the past two decades, and the number of cases of FSGS diagnosed by renal biopsies in these centers now exceeds the number of cases of membranous nephropathy. Nonetheless, as a substantial fraction of patients with FSGS do not have the nephrotic syndrome, it remained unclear as to what extent the relative frequencies of FSGS and other glomerulopathies as causes of the nephrotic syndrome have changed over this time. To address this concern, we reviewed data from 1,000 adult native kidney biopsies performed between January 1976 and April 1979 and from 1,000 biopsies performed between January 1995 and January 1997, identified all cases with a full-blown nephrotic syndrome of unknown etiology at the time of biopsy, and compared the relative frequencies with which specific diseases were diagnosed in these latter cases between the two time intervals. The main findings of this study were that, first, during the 1976 to 1979 period, the relative frequencies of membranous (36%) and minimal-change (23%) nephropathies and of FSGS (15%) as causes of unexplained nephrotic syndrome were similar to those observed in previous studies during the 1970s and early 1980s. In contrast, from 1995 to 1997, FSGS was the most common cause of this syndrome, accounting for 35% of cases compared with 33% for membranous nephropathy. Second, during the 1995 to 1997 period, FSGS accounted for more than 50% of cases of unexplained nephrotic syndrome in black adults and for 67% of such cases in black adults younger than 45 years. Third, although the relative frequency of nephrotic syndrome due to FSGS was two to three times higher in black than in white patients during both study periods, the frequency of FSGS increased similarly among both racial groups from the earlier to the later period. Fourth, the frequency of minimal-change nephrotic syndrome decreased from the earlier to the later study period in both black and white adults. Fifth, the relative frequency of membranoproliferative glomerulonephritis as a cause of the nephrotic syndrome declined from the 1976 to 1979 period to the 1995 to 1997 period, whereas that of immunoglobulin A nephropathy appeared to increase; the latter accounted for 14% of cases of unexplained nephrotic syndrome in white adults during the latter study period. Finally, 10% of nephrotic adults older than 44 years had AL amyloid nephropathy; none of these patients had multiple myeloma or a known paraprotein at the time of renal biopsy.
20世纪70年代和80年代初收集的数据表明，在这些时期，膜性肾病是成人不明原因肾病综合征最常见的病因，其次按频率依次为微小病变肾病和局灶节段性肾小球硬化（FSGS）。然而，我们和其他人最近报告称，在过去二十年中FSGS的发病率有所上升，这些中心通过肾活检诊断出的FSGS病例数现在超过了膜性肾病的病例数。尽管如此，由于相当一部分FSGS患者没有肾病综合征，目前尚不清楚在此期间FSGS和其他肾小球疾病作为肾病综合征病因的相对频率发生了多大变化。为了解决这一问题，我们回顾了1976年1月至1979年4月期间进行的1000例成人原发性肾活检数据以及1995年1月至1997年1月期间进行的1000例活检数据，确定了活检时病因不明的典型肾病综合征的所有病例，并比较了这两个时间段内在后一组病例中诊断出特定疾病的相对频率。这项研究的主要发现是，第一，在1976年至1979年期间，膜性肾病（36%）、微小病变肾病（23%）和FSGS（15%）作为不明原因肾病综合征病因的相对频率与20世纪70年代和80年代初之前的研究中观察到的相似。相比之下，1995年至1997年期间，FSGS是该综合征最常见的病因，占病例的35%，而膜性肾病为33%。第二，在1995年至1997年期间，FSGS占黑人成人不明原因肾病综合征病例的50%以上，占45岁以下黑人成人此类病例的67%。第三，尽管在两个研究期间，FSGS所致肾病综合征的相对频率在黑人中比白人高两到三倍，但从早期到后期，两个种族群体中FSGS的频率均有类似增加。第四，在黑人及白人成人中，从早期到后期的研究期间，微小病变肾病综合征的频率均有所下降。第五，膜增生性肾小球肾炎作为肾病综合征病因的相对频率从1976年至1979年期间到1995年至1997年期间有所下降，而免疫球蛋白A肾病的相对频率似乎有所增加；在后期研究期间，后者占白人成人不明原因肾病综合征病例的14%。最后，44岁以上的肾病成人中有10%患有AL淀粉样肾病；这些患者在肾活检时均无多发性骨髓瘤或已知的副蛋白。
