Thomas D B, Franceschini N, Hogan S L, Ten Holder S, Jennette C E, Falk R J, Jennette J C
UNC Nephropathology Laboratory, Department of Pathology and Laboratory Medicine, University of North Carolina, 409 Brinkhous-Bullitt Building, CB #7525, Chapel Hill, NC 27599, USA.
Kidney Int. 2006 Mar;69(5):920-6. doi: 10.1038/sj.ki.5000160.
Histologic variants of idiopathic focal segmental glomerulosclerosis (FSGS) may have prognostic value. A recent working classification system has distinguished five FSGS variants. We evaluated a cohort of adult patients with biopsy-proven FSGS diagnosed between March 1982 and July 2001 to determine if subtypes were associated with renal outcome. Renal biopsies were reviewed by two pathologists. Demographic and clinical data were obtained from charts. Outcomes were partial and complete remission of the nephrotic syndrome, and renal failure. The frequency of FSGS variants was: 3% cellular (N=6), 11% collapsing (N=22), 17% tip lesion (N=34), 26% perihilar (N=52), and 42% not otherwise specified (NOS) (N=83). Collapsing FSGS affected younger and more often black patients. Black race was uncommon in tip variant. Collapsing and tip variants had higher proteinuria and lower serum albumin than perihilar and NOS variants. Better renal function and less severe tubulointerstitial injury were observed in patients with tip variant. These patients were more likely to receive steroids and more often achieved complete remission (50%). After a median follow-up of 1.8 years, 23% of patients were on dialysis and 28% had renal failure. Collapsing FSGS had worse 1-year (74%) and 3-year (33%) renal survival compared to other variants (overall cohort renal survival at 1 and 3 years: 86 and 67%). Different histologic variants of FSGS have substantial differences in clinical features at the time of biopsy diagnosis and substantial differences in renal outcomes.
特发性局灶节段性肾小球硬化（FSGS）的组织学变异型可能具有预后价值。最近的一个工作分类系统已区分出五种FSGS变异型。我们评估了一组在1982年3月至2001年7月期间经活检证实为FSGS的成年患者，以确定各亚型是否与肾脏预后相关。两位病理学家对肾活检标本进行了复查。从病历中获取人口统计学和临床数据。结局指标为肾病综合征的部分和完全缓解以及肾衰竭。FSGS变异型的发生率分别为：细胞型3%（N = 6），塌陷型11%（N = 22），顶端病变型17%（N = 34），肾门周围型26%（N = 52），未另行指定型（NOS）42%（N = 83）。塌陷型FSGS患者年龄较轻，且黑人患者更为常见。顶端变异型中黑人种族并不常见。塌陷型和顶端变异型的蛋白尿水平高于肾门周围型和NOS变异型，血清白蛋白水平低于肾门周围型和NOS变异型。顶端变异型患者的肾功能较好，肾小管间质损伤较轻。这些患者更有可能接受类固醇治疗，且更常实现完全缓解（50%）。中位随访1.8年后，23%的患者接受透析治疗，28%的患者出现肾衰竭。与其他变异型相比，塌陷型FSGS的1年（74%）和3年（33%）肾脏生存率更差（整个队列1年和3年的肾脏生存率分别为86%和67%）。FSGS的不同组织学变异型在活检诊断时的临床特征以及肾脏预后方面存在显著差异。
