Woo Keng Thye, Chan Choong Meng, Foo Marjorie, Lim Cynthia, Choo Jason, Chin Yok Mooi, Teng Esther Wei Ling, Mok Irene, Kwek Jia Liang, Tan Hui Zhuan, Loh Alwin H L, Wong Jiunn, Kee Terence, Choong Hui Lin, Tan Han Khim, Wong Kok Seng, Tan Puay Hoon, Tan Chieh Suai
Clin Nephrol. 2023 Mar;99(3):128-140. doi: 10.5414/CN111022.
This is a study on the demographics and clinical outcomes including the response to therapy of patients with focal segmental glomerulosclerosis (FSGS) over the past decade.
All histologically proven FSGS cases diagnosed between 2008 and 2018 were analyzed for their clinical, laboratory, and histological characteristics including treatment that could influence the disease progression and renal outcome of these patients. We used the Columbia Classification for FSGS for the renal biopsy.
There were two subgroups of FSGS patients; those with nephrotic syndrome and those without nephrotic syndrome. Patients with FSGS with non-nephrotic syndrome had poorer survival rates compared to the nephrotic group. For those without nephrotic syndrome, the indices responsible for progression involved more tubular and blood vessel lesions in addition to glomerular pathology compared to those with nephrotic syndrome. Patients with FSGS with nephrotic syndrome responded to immunosuppressants more favorably compared to the non-nephrotic group, though both groups responded with decreasing proteinuria. The nephrotic group had a better 10-year long-term survival rate of 92 vs. 72% for the non-nephrotic group (log-rank 0.002). The 10-year survival for the whole group of FSGS patients was 64%.
Our data suggest that in FSGS, one of the significant components of the disease is the vascular and tubular damage, apart from the underlying glomerular pathology, resulting in varying responses to therapy, and the difference is reflected in inherently poorer response to immunosuppressant therapy in those without nephrotic syndrome as opposed to those with nephrotic syndrome, who responded to immunosuppressant therapy (IST) with stabilization of renal function and had less blood vessel and tubular lesions.
这是一项关于过去十年中局灶节段性肾小球硬化（FSGS）患者的人口统计学和临床结局（包括对治疗的反应）的研究。
分析2008年至2018年间所有经组织学证实的FSGS病例的临床、实验室和组织学特征，包括可能影响这些患者疾病进展和肾脏结局的治疗方法。我们使用FSGS的哥伦比亚分类法对肾活检进行分类。
FSGS患者分为两个亚组；患有肾病综合征的患者和未患有肾病综合征的患者。与肾病组相比，非肾病综合征的FSGS患者生存率较低。对于那些没有肾病综合征的患者，与肾病综合征患者相比，除了肾小球病理改变外，导致疾病进展的指标还涉及更多的肾小管和血管病变。与非肾病组相比，患有肾病综合征的FSGS患者对免疫抑制剂的反应更有利，尽管两组患者的蛋白尿均有所减少。肾病组的10年长期生存率较好，为92%，而非肾病组为72%（对数秩检验P = 0.002）。FSGS患者全组的10年生存率为64%。
我们的数据表明，在FSGS中，除了潜在的肾小球病理改变外，疾病的一个重要组成部分是血管和肾小管损伤，这导致了对治疗的不同反应，这种差异体现在无肾病综合征的患者对免疫抑制治疗的反应天生较差，而有肾病综合征的患者对免疫抑制治疗（IST）反应良好，肾功能稳定，血管和肾小管病变较少。
