Efficacy of abatacept treatment for focal segmental glomerulosclerosis and minimal change disease: A systematic review of case reports, case series, and observational studies

2020 ◽  
Vol 94 (3) ◽  
pp. 117-126
Author(s):  
Panupong Hansrivijit ◽  
Max M. Puthenpura ◽  
Nasrollah Ghahramani
Keyword(s):  
Systematic Review ◽  
Focal Segmental Glomerulosclerosis ◽  
Observational Studies ◽  
Minimal Change Disease ◽  
Case Reports ◽  
Case Series ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

scholarly journals Efficacy and safety of rituximab in adult frequent-relapsing or steroid-dependent minimal change disease or focal segmental glomerulosclerosis: a systematic review and meta-analysis

2020 ◽  
Author(s):  
Cheng Xue ◽  
Bo Yang ◽  
Jing Xu ◽  
Chenchen Zhou ◽  
Liming Zhang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Adverse Events ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Meta Analysis ◽  
Minimal Change ◽  
Efficacy And Safety ◽  
Segmental Glomerulosclerosis ◽  
Steroid Dependent

Abstract Background The efficacy and safety of rituximab (RTX) in adult frequent-relapsing (FR) or steroid-dependent (SD) nephrotic syndrome (NS), including minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS), are still inconclusive. Methods We performed a systematic review and meta-analysis registered in  PROSPERO (CRD42019148102) by pooling data of cohort studies or case series on adult patients with difficult-to-treat NS. Steroid-resistant NS was excluded. The primary outcomes were the complete remission (CR) rate and the relapse rate. Partial remission (PR) rate, no response (NR) rate and adverse events were the secondary outcomes. A random-effects model was performed for all the outcomes. Results We included 21 studies involving 382 adult MCD/FSGS subjects with a median follow-up duration from 12 to 43 months. RTX treatment induced a pooled 84.2% CR rate [95% confidence interval (CI): 67.7–96.3%], while MCD patients had a high 91.6% CR rate and FSGS patients a moderate 43% CR rate. However, 27.4% (95% CI 20.7–34.5%) of the patients relapsed during the follow-up. The pooled PR and NR rates were 5.8% (95% CI 1.2–12.5%) and 5.2% (95% CI 0.0–15.0%), respectively. RTX was associated with trivial adverse events and good tolerance. Conclusions In summary, by pooling results of current pilot studies, RTX may be an effective and relatively safe alternative for most adult FR or SD MCD/FSGS to displace calcineurin inhibitors or prednisone in the hierarchy of treatment. More clinical trials comparing RTX with other immunosuppressants and concerning the long-term adverse events are needed.

Rituximab Treatment for Relapsing Minimal Change Disease and Focal Segmental Glomerulosclerosis: A Systematic Review

2014 ◽  
Vol 39 (4) ◽  
pp. 322-330 ◽  
Author(s):  
Andreas Kronbichler ◽  
Julia Kerschbaum ◽  
Gema Fernandez-Fresnedo ◽  
Elion Hoxha ◽  
Christine E. Kurschat ◽  
...  
Keyword(s):  
Systematic Review ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

scholarly journals Case series: CTLA4-IgG1 therapy in minimal change disease and focal segmental glomerulosclerosis

2014 ◽  
Vol 30 (3) ◽  
pp. 469-477 ◽  
Author(s):  
Eduardo H. Garin ◽  
Jochen Reiser ◽  
Gabriel Cara-Fuentes ◽  
Changli Wei ◽  
Dany Matar ◽  
...  
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Case Series ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

Pathogenesis of proteinuria in minimal change disease and focal segmental glomerulosclerosis

2018 ◽  
Author(s):  
Patrick Niaudet ◽  
Alain Meyrier
Keyword(s):  
Serum Albumin ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Slit Diaphragm ◽  
Segmental Glomerulosclerosis ◽  
Podocyte Loss ◽  
Glomerular Filtrate

It is now well established that the podocyte, and in particular the slit diaphragm structure, are critical to the barrier to serum albumin entering glomerular filtrate in large quantities. In minimal change disease there is proteinuria without podocyte death, whereas in focal segmental glomerulosclerosis there is not only podocyte dysfunction but also podocyte loss.

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