Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Faculty of Medicine, Kidney and Vascular Pathology, University of Tsukuba, Tsukuba, Japan.
PLoS One. 2021 Jan 5;16(1):e0244677. doi: 10.1371/journal.pone.0244677. eCollection 2021.
The Columbia classification is widely used for diagnosis of focal segmental glomerulosclerosis (FSGS). In practice, we occasionally encounter segmental glomerular lesions unclassified as Columbia classification. We analyzed the clinical implication of unclassified segmental lesions comparing with Columbia-classified FSGS.
A retrospective cohort study from 13 local hospitals in Japan. From 172 biopsy cases diagnosed with FSGS or minimal change disease (MCD)/FSGS spectrum with unclassified segmental lesions, adult patients with nephrotic syndrome who received immunosuppressive therapies were included. The cases are classified by pathology, i.e., typical FSGS lesions sufficiently classified into subgroups of Columbia classification: collapsing (COL), tip (TIP), cellular (CEL), perihilar (PH), and not otherwise specified (NOS), and unclassified by the Columbia classification into three subgroups: "endothelial damage,"; "simple attachment,"; and "minor cellular lesion,". The response to immunosuppressive treatment and 30% decline of eGFR were compared.
Among 48 eligible cases, all were Japanese, 34 were typical FSGS; 13 TIP, 15 CEL, 6 NOS, and no COL or PH cases. Fourteen were unclassified cases: endothelial damage (n = 6), simple attachment (n = 5), and minor cellular lesion (n = 3). The median age of overall patients was 60 years old and the median of eGFR and urinary protein creatinine ratio was 51.5 mL/min/1.73m2 and 7.35, respectively. They received similar therapeutic regimen. Kaplan-Meier analysis revealed no significant difference in treatment response between typical FSGS and unclassified cases. Evaluating among the subgroups, endothelial damage, simple attachment and minor cellular lesion showed similar treatment response to TIP or CEL. No significant difference was also observed in the 30% decline of eGFR.
Japanese adult patients with nephrotic syndrome showing unclassified segmental lesions as Columbia classification may be equivalent clinical impact as Columbia classification of FSGS.
哥伦比亚分类法被广泛用于局灶节段性肾小球硬化症（FSGS）的诊断。在实际工作中，我们偶尔会遇到无法归入哥伦比亚分类法的节段性肾小球病变。我们分析了未分类节段性病变与哥伦比亚分类法 FSGS 的临床意义。
这是一项来自日本 13 家医院的回顾性队列研究。纳入了 172 例活检诊断为 FSGS 或微小病变病（MCD）/FSGS 谱伴有未分类节段性病变的患者，这些患者为接受免疫抑制治疗的肾病综合征成年患者。通过病理将病例进行分类，即充分归入哥伦比亚分类法亚组的典型 FSGS 病变：塌陷（COL）、尖端（TIP）、细胞性（CEL）、近旁（PH）和未特指（NOS），以及未归入哥伦比亚分类法的三个亚组：“内皮损伤”；“单纯附着”；和“轻微细胞病变”。比较了对免疫抑制治疗的反应和 eGFR 下降 30%的情况。
在 48 例符合条件的病例中，所有患者均为日本人，34 例为典型 FSGS；13 例 TIP、15 例 CEL、6 例 NOS，无 COL 或 PH 病例。14 例为未分类病例：内皮损伤（n=6）、单纯附着（n=5）和轻微细胞病变（n=3）。所有患者的中位年龄为 60 岁，eGFR 和尿蛋白/肌酐比的中位数分别为 51.5 mL/min/1.73m2和 7.35。他们接受了类似的治疗方案。Kaplan-Meier 分析显示，典型 FSGS 和未分类病例的治疗反应无显著差异。在亚组之间评估时，内皮损伤、单纯附着和轻微细胞病变与 TIP 或 CEL 显示出相似的治疗反应。eGFR 下降 30%也无显著差异。
日本肾病综合征成年患者出现未归入哥伦比亚分类法的节段性病变可能与哥伦比亚分类法 FSGS 的临床影响相当。
