Steroid-Dependent Nephrotic Syndrome
Conditions
Brief summary
Idiopathic Nephrotic Syndrome (INS) is the first glomerulopathy in children and 60% of the patients develop Steroid-Dependant Nephrotic Syndrome (SDNS). Recently, rituximab (RTX), a humanized anti-CD20 antibody depleting B cells demonstrated the ability to increase relapse free survival and to decrease the number of relapse and the need of other immunosuppressive drugs. However, the remission rate after 2 years is only 30 to 40%. The aim of the study is to study the ability of intravenous Immunoglobulin to improve remission rate in SDNS when added associated with Rituximab compared to a treatment by Rituximab alone.
Detailed description
Idiopathic Nephrotic Syndrome (INS) is the first glomerulopathy in children and 60% of the patients develop Steroid-Dependant Nephrotic Syndrome (SDNS). Depleting B cells demonstrated the ability to increase relapse free survival and to decrease the number of relapse and the need of other immunosuppressive drugs. However, the remission rate after 2 years is only 30 to 40%. The aim of the study is to study the ability of intravenous Immunoglobulin to improve remission rate in SDNS when added associated with Rituximab compared to a treatment by Rituximab alone.
Interventions
DRUGimmunoglobulin IV
5 injections of immunoglobulin IV once a month during 5 months (2g/kg at M1, 1.5g/kg at M2 to M5, maximal dose 100g)
Sponsors
Assistance Publique - Hôpitaux de Paris
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Masking description
No masking
Intervention model description
2 Arms : * Rituximab (375 mg/m2) * Rituximab (375 mg/m2) followed by 5 injections of immunoglobulin IV once a month during 5 months (2g/kg at M1, 1.5g/kg at M2 to M5, maximal dise 100g)
Eligibility
Sex/Gender
ALL
Age
2 Years to 25 Years
Healthy volunteers
No
Inclusion criteria
* Childhood onset nephrotic syndrome (first flair \<18 years) * ≥ 2 years old at inclusion * Steroid-dependent: * Patient with at least 2 relapses confirmed during the decay of corticosteroids or within 2 weeks following steroids discontinuation. * Patient with at least 2 relapses including one under steroidsparing agent (MMF, Calcineurin inhibitors, cyclophosphamide, Levamisole) or within 6 months of treatment withdrawal. * or with frequent relapses: · 2 or more relapses within 6 months after initial remission or 4 or more relapses within any 12-month period. * with a relapse within 3 months prior to inclusion * In remission: Protein-over-creatinine ratio ≤ 0.2g/g (≤ 0.02g/mmol)
Exclusion criteria
* Patients with steroid-resistant nephrotic syndrome; * Patients with genetic nephrotic syndrome; * Patients previously treated with rituximab; * Patients with no affiliation to a social security scheme (beneficiary or legal); * Prior Hepatitis B, Hepatitis C or HIV infection; * Pregnancy or breastfeeding. * Patients with hyperprolinaemia, * Known hypersensitivity to one of the study medication, * Scheduled and not postponable injection of live attenuated vaccine * Protected adults * Patients with neutrophils \< 1.5 G/L and/or platelets \< 75 G/L
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| The occurrence of the first relapse | 24 months | Relapse is defined as a protein to creatinine ratio of 2g/g of creatinine (0.2 g/mmol) or higher |
Secondary
| Measure | Time frame |
|---|---|
| Time to first relapse | 24 months |
| Number of relapse over a 24 months follow-up | 24 months |
| Cumulative amount of corticosteroid over a 24 months follow-up | 24 months |
| Adverse events in each arm | 24 months |
Countries
France
Outcome results
None listed