Jafry Nazarul Hassan, Manan Shumaila, Rashid Rahma, Mubarak Muhammed
Department of Nephrology, Sindh Institute of Urology and Transplantation, Sindh, Karachi 74200, Pakistan.
Department of Pathology, Sindh Institute of Urology and Transplantation, Sindh, Karachi 74200, Pakistan.
World J Nephrol. 2024 Mar 25;13(1):88028. doi: 10.5527/wjn.v13.i1.88028.
The Columbia classification identified five histological variants of focal segmental glomerulosclerosis (FSGS). The prognostic significance of these variants remains controversial.
To evaluate the relative frequency, clinicopathologic characteristics, and medium-term outcomes of FSGS variants at a single center in Pakistan.
This retrospective study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan on all consecutive adults (≥ 16 years) with biopsy-proven primary FSGS from January 1995 to December 2017. Studied subjects were treated with steroids as a first-line therapy. The response rates, doubling of serum creatinine, and kidney failure (KF) with replacement therapy were compared between histological variants using ANOVA or Kruskal Wallis, and Chi-square tests as appropriate. Data were analyzed by SPSS version 22.0. -value ≤ 0.05 was considered significant.
A total of 401 patients were diagnosed with primary FSGS during the study period. Among these, 352 (87.7%) had a designated histological variant. The not otherwise specified (NOS) variant was the commonest, being found in 185 (53.9%) patients, followed by the tip variant in 100 (29.1%) patients. Collapsing (COL), cellular (CEL), and perihilar (PHI) variants were seen in 58 (16.9%), 6 (1.5%), and 3 (0.7%) patients, respectively. CEL and PHI variants were excluded from further analysis due to small patient numbers. The mean follow-up period was 36.5 ± 29.2 months. Regarding response rates of variants, patients with TIP lesions achieved remission more frequently (59.5%) than patients with NOS (41.8%) and COL (24.52%) variants ( < 0.001). The hazard ratio of complete response among patients with the COL variant was 0.163 [95% confidence interval (CI): 0.039-0.67] as compared to patients with NOS. The TIP variant showed a hazard ratio of 2.5 (95%CI: 1.61-3.89) for complete remission compared to the NOS variant. Overall, progressive KF was observed more frequently in patients with the COL variant, 43.4% ( < 0.001). Among these, 24.53% of patients required kidney replacement therapy ( < 0.001). The hazard ratio of doubling of serum creatinine among patients with the COL variant was 14.57 (95%CI: 1.87-113.49) as compared to patients with the TIP variant.
In conclusion, histological variants of FSGS are predictive of response to treatment with immunosuppressants and progressive KF in adults in our setup.
哥伦比亚分类法确定了局灶节段性肾小球硬化（FSGS）的五种组织学变异型。这些变异型的预后意义仍存在争议。
评估巴基斯坦某单一中心FSGS变异型的相对频率、临床病理特征及中期结局。
本回顾性研究于巴基斯坦卡拉奇信德泌尿与移植研究所肾脏病科对1995年1月至2017年12月期间所有经活检证实为原发性FSGS的连续成年患者（≥16岁）进行。研究对象接受类固醇作为一线治疗。使用方差分析或克鲁斯卡尔 - 沃利斯检验以及适当的卡方检验比较各组织学变异型之间的缓解率、血清肌酐翻倍情况及接受替代治疗的肾衰竭（KF）情况。数据采用SPSS 22.0版进行分析。P值≤0.05被认为具有统计学意义。
研究期间共401例患者被诊断为原发性FSGS。其中，352例（87.7%）有指定的组织学变异型。未另作说明（NOS）变异型最为常见，见于185例（53.9%）患者，其次是顶端变异型，见于100例（29.1%）患者。塌陷型（COL）、细胞型（CEL）和系膜旁型（PHI）变异型分别见于58例（16.9%）、6例（1.5%）和3例（0.7%）患者。由于患者数量少，CEL和PHI变异型被排除在进一步分析之外。平均随访期为36.5±29.2个月。关于变异型的缓解率，顶端病变患者的缓解频率（59.5%）高于NOS（41.8%）和COL（24.52%）变异型患者（P<0.001）。与NOS变异型患者相比，COL变异型患者完全缓解的风险比为0.163[95%置信区间（CI）：0.039 - 0.67]。与NOS变异型相比，顶端变异型完全缓解的风险比为2.5（95%CI：1.61 - 3.89）。总体而言，COL变异型患者中进行性KF更为常见，为43.4%（P<0.001）。其中，24.53%的患者需要肾脏替代治疗（P<0.001）。与顶端变异型患者相比，COL变异型患者血清肌酐翻倍的风险比为14.57（95%CI：1.87 - 113.49）。
总之，在我们的研究中，FSGS的组织学变异型可预测成人免疫抑制剂治疗反应及进行性KF。
