The effectiveness of cyclosporine A for patients with steroid-resistant nephrotic syndrome

Introduction. Congenital nephrotic syndrome (CNF) is manifested at birth or within the first three months of life. The Finnish-type of CNF is caused by the mutation of the NPHS1 gene, which encodes nephrin in the podocyte slit diaphragm. It is a very severe disease, for which immunosuppressive therapy is not advised. Here we describe a patient with CNF who responded to CsA by partial remission. Case outline. A girl aged 2.5 months presented with severe non-syndromic steroid-resistant nephrotic syndrome. She needed aggressive support including daily albumin infusions and diuretics. Substitution of vitamin D, thyroxin, and anticoagulants were regularly administered. She was also treated with angiotensin converting enzyme inhibitor, without clear benefits regarding proteinuria. In addition, she received intravenous gamma-globulin replacement therapy and antibiotics during frequent infections. While waiting for the results of genetic analyses and faced with many problems related to daily albumin infusions, infections, and thromboembolic complications, cyclosporine A (CsA) was introduced as an alternative to early nephrectomy and consequent renal failure. The patient responded by partial remission and CsA treatment continued at home without the albumin infusions. After almost five years since the beginning of the treatment, the patient?s renal function remains unreduced. Conclusion. Our case demonstrates that CsA can induce partial remission in patients with genetic forms of steroid-resistant nephrotic syndrome without influencing the glomerular filtration rate. However, its long-term effect and safety should carefully be monitored.

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Efficacy and safety of cyclosporine a for patients with steroid-resistant nephrotic syndrome: a meta-analysis

BMC Nephrology ◽

10.1186/s12882-019-1575-8 ◽

2019 ◽

Vol 20 (1) ◽

Author(s):

Hong-Yan Li ◽

Xialan Zhang ◽

Tianbiao Zhou ◽

Zhiqing Zhong ◽

Hongzhen Zhong

Keyword(s):

Nephrotic Syndrome ◽

Serum Creatinine ◽

Cyclosporine A ◽

Plasma Cholesterol ◽

Meta Analysis ◽

Cochrane Library ◽

Efficacy And Safety ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome ◽

The Cochrane Library

Abstract Background The purpose of this study was to determine efficacy and safety of cyclosporine A (CsA) for patients with steroid-resistant nephrotic syndrome (SRNS). Methods The Cochrane Library and PubMed were searched to extract the associated studies on Oct 10, 2018, and the meta-analysis method was used to pool and analyze the applicable investigations included in this study. The P(opulation) I(ntervention) C(omparison) O(utcome) of the study were defined as follows: P: Patients with SRNS; I: treated with CsA, cyclophosphamide (CYC), tacrolimus (TAC) or placebo/not treatment (P/NT); C: CsA vs. placebo/nontreatment (P/NT), CsA vs. CYC, CsA vs. TAC; O: complete remission (CR), total remission (TR; complete or partial remission (PR)), urine erythrocyte number, proteinuria levels, albumin, proteinuria, serum creatinine, and plasma cholesterol, etc. Data were extracted and pooled using RevMan 5.3. Results In the therapeutic regimen of CsA vs. placebo/nontreatment (P/NT), the results indicated that the CsA group had high values of CR, TR, and low values of proteinuria, serum creatinine, and plasma cholesterol when compared with those in the placebo group. In comparing CsA vs. cyclophosphamide (CYC), the results indicated that the CsA group had higher TR than the CYC group. In comparing CsA vs. tacrolimus (TAC), the results revealed insignificant differences in CR, and TR between the CsA and TAC groups. The safety of CsA was also assessed. The incidence of gum hyperplasia in CsA group was higher than that in the P/NT group, with no differences in incidence of infections or hypertension between CsA and P/NT groups. There was no difference in the incidence of hypertension between the CsA and TAC groups. Conclusions CsA is an effective and safe agent in the therapy of patients with SRNS.

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Cyclosporine A Effect on Hearing in Children with Steroid Dependent Nephrotic Syndrome and Steroid Resistant Nephrotic Syndrome.

GEGET ◽

10.21608/geget.2021.187499 ◽

2021 ◽

Vol 16 (1) ◽

pp. 14-24

Author(s):

Mohamed El Farsy ◽

Maged Ashraf Abd El Fattah ◽

Fathy Fatouh ◽

Ahmed Abd El Fattah

Keyword(s):

Nephrotic Syndrome ◽

Cyclosporine A ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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Cyclosporine A and steroid therapy in childhood steroid-resistant nephrotic syndrome

International Journal of Nephrology and Renovascular Disease ◽

10.2147/ijnrd.s10168 ◽

2010 ◽

pp. 117 ◽

Cited By ~ 1

Author(s):

Gargah Tahar

Keyword(s):

Nephrotic Syndrome ◽

Steroid Therapy ◽

Cyclosporine A ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome

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The Cyclosporine-A Treatment does not have Harmful Effect on the Linear Growth of Pediatric Patients with Steroid-dependent and Steroid-resistant Nephrotic Syndrome

Childhood Kidney Diseases ◽

10.3339/jkspn.2016.20.2.45 ◽

2016 ◽

Vol 20 (2) ◽

pp. 45-49

Author(s):

Sang Soo Lee ◽

Ji Hoon Kim ◽

Chung Ho Kim ◽

Byoung-Soo Cho ◽

Deog Yoon Kim ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Cyclosporine A ◽

Linear Growth ◽

Pediatric Patients ◽

Harmful Effect ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome ◽

Steroid Dependent

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Cyclophosphamide versus cyclosporine A therapy in steroid-resistant nephrotic syndrome: a retrospective study with a mean 5-year follow-up

Journal of International Medical Research ◽

10.1177/0300060518782017 ◽

2018 ◽

Vol 46 (11) ◽

pp. 4506-4517 ◽

Cited By ~ 3

Author(s):

Yanwei Liu ◽

Ruikun Yang ◽

Chen Yang ◽

Shuhong Dong ◽

Ying Zhu ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Relapse Rate ◽

Cyclosporine A ◽

Effective Rate ◽

Peroral Administration ◽

Primary Analysis ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome ◽

Delayed Time

Objective To compare the clinical efficacy of cyclophosphamide (CTX) and cyclosporine A (CSA) in initial treatment of children with steroid-resistant nephrotic syndrome (SRNS). Methods Prospectively maintained databases were reviewed to retrospectively compare two cohorts with SRNS that received peroral administration of 2 to 2.5 mg/kg/d CTX for 3 to 6 months or 1 to 5 mg/kg/d CSA for 2 years until the primary analysis cut-off date during 2007 to 2011. The time to first on-study relapse of SRNS was the primary endpoint. The effective rate was the second endpoint. Results A total of 127 children with SRNS were included (CTX-treated cohort: n = 62; CSA-treated cohort: n = 65), with a mean 5-year follow-up. CTX-treated children showed a significantly delayed time to first on-study relapse of SRNS compared with CSA-treated children (hazard ratio 0.66, 95% confidence interval 0.32–1.75). The relapse rate (rate/year) in CTX-treated children (1.1 ± 0.1) at the 24-month follow-up was significantly higher than that with CSA (0.4 ± 0.2). This difference persisted until the final follow-up. Conclusions CSA is associated with a significantly lower relapse rate and significantly higher effective rate compared with CTX, especially in children with minimal change disease.

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