Worsening anasarca on a child with severe steroid-dependent nephrotic syndrome without proteinuria: Answers

Objectives: Rituximab (RTX), a possible alternative treatment option, is recognized as a new therapeutic hope for the treatment of steroid-dependent nephrotic syndrome (SDNS) in children. However, the efficacy and safety of RTX in the treatment of childhood SDNS are still controversial. The objective of this study was to evaluate the efficacy and safety of RTX treatment in children with SDNS.Study Design: Six randomized controlled trials (RCTs) and one retrospective comparative control study data from studies, performed before January 2021 were collected, from PubMed, Cochrane Library, Embase, and Web of Science. The studies evaluating the efficacy and safety of RTX in childhood SDNS were included.Results: Six RCTs and one retrospective comparative control study were included in our analysis. Compared with the control group, the RTX treatment group achieved a higher complete remission rate (OR = 5.21; 95% CI, 3.18–8.54; p < 0.00001), and we found significant differences between the two groups on serum albumin level (MD = 0.88; 95% CI, 0.43–1.33; p = 0.0001) and estimated glomerular filtration rate (MD = 6.43; 95% CI, 2.68–10.19; p = 0.0008). However, RTX treatment did not significantly lower serum creatinine levels nor did it significantly reduce the occurrence of proteinuria. In addition, we found no advantages with RTX on treatment safety.Conclusions: RTX has shown satisfactory characteristics in terms of efficacy and may be a promising treatment method for SDNS in children. However, the long-term effects have not been fully evaluated and should be further studied through randomized clinical trials.

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A case report on usage of rituximab on complicated steroid-dependent nephrotic syndrome

International Journal of Basic & Clinical Pharmacology ◽

10.18203/2319-2003.ijbcp20205552 ◽

2020 ◽

Vol 10 (1) ◽

pp. 130

Author(s):

Shravan Jayachandran ◽

S. Suwitha ◽

A. Priya ◽

K. Arun Chander

Keyword(s):

Nephrotic Syndrome ◽

Treatment Duration ◽

Supportive Treatment ◽

Childhood Disease ◽

Steroid Dependent ◽

The Status ◽

Resistance Patterns ◽

History Of ◽

The Common ◽

Steroid Dependent Nephrotic Syndrome

Nephrotic syndrome (NS) is one of the common yet challenging childhood disease. Treating NS with immunosuppressants like corticosteroids is typically effective. However, there are significant evidences which displayed resistance patterns to the former drugs. Here in this case, a male patient came to the nephrology department with the history of steroid dependant nephrotic syndrome with complaint of its infrequent relapse. The condition was complicated since, the patient was on multiple steroids even after which there were recurrent episodes of syndrome. Considering the status, Mycophenolate mofetil was added as a supportive treatment for the management of the disease along with steroids, still the outcome was unremarkable. Hence, rituximab was ordered (four cycles). The outcome this time was appreciable, rituximab administration produced a positive result. No events were recorded during the treatment duration.

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A male patient with steroid-dependent nephrotic syndrome for 25 years and obesity-associated focal segmental glomerulosclerosis

Clinical and Experimental Nephrology ◽

10.1007/s10157-004-0279-x ◽

2004 ◽

Vol 8 (3) ◽

pp. 263-265 ◽

Cited By ~ 1

Author(s):

Osamu Motoyama ◽

Atsuhiko Muto ◽

Hidetaka Ogiwara ◽

Sonoo Mizuiri ◽

Sadao Kawamura ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Focal Segmental Glomerulosclerosis ◽

Male Patient ◽

Segmental Glomerulosclerosis ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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Risk factors for early B cell recovery following single-dose rituximab therapy in Japanese children with steroid-dependent nephrotic syndrome

Pediatric Nephrology ◽

10.1007/s00467-018-4075-3 ◽

2018 ◽

Vol 34 (2) ◽

pp. 359-360

Author(s):

Shuichiro Fujinaga ◽

Tomohiko Nishino

Keyword(s):

Risk Factors ◽

Nephrotic Syndrome ◽

Single Dose ◽

B Cell ◽

Cell Recovery ◽

Japanese Children ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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MO291RITUXIMAB IS EFFECTIVE AND SAFE IN ADULTS WITH STEROID-DEPENDENT NEPHROTIC SYNDROME: A LONG-TERM, SINGLE-CENTER EXPERIENCE

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0049 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Gemma Patella ◽

Alessandro Comi ◽

Giuseppe Coppolino ◽

Nicolino Comi ◽

Giorgio Fuiano ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Case Series ◽

Adult Patients ◽

Single Center ◽

Single Center Experience ◽

Steroid Dependent ◽

The Mean ◽

Steroid Dependent Nephrotic Syndrome

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p<0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p<0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.

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Mycophenolate Mofetil after Rituximab for Childhood-onset Complicated Frequently-relapsing or Steroid-dependent Nephrotic Syndrome

Journal of the American Society of Nephrology ◽

10.1681/asn.2021050643 ◽

2021 ◽

pp. ASN.2021050643

Author(s):

Kazumoto Iijima ◽

Mayumi Sako ◽

Mari Oba ◽

Seiji Tanaka ◽

Riku Hamada ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Mycophenolate Mofetil ◽

Treatment Failure ◽

Treatment Period ◽

Immunosuppressive Agents ◽

Steroid Resistance ◽

Rank Test ◽

Childhood Onset ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

Background. Rituximab is the standard therapy for childhood-onset complicated frequently-relapsing or steroid-dependent nephrotic syndrome (FRNS/SDNS). However, most patients redevelop FRNS/SDNS following peripheral B cell recovery. Methods. We conducted a multicenter, randomized, double-blind, placebo-controlled trial to examine whether mycophenolate mofetil (MMF) administration after rituximab can prevent treatment failure (FRNS, SDNS, steroid resistance, or use of immunosuppressive agents or rituximab). Thirty-nine patients (per group) were treated with rituximab, followed by either MMF or placebo until Day 505 (treatment period). The primary outcome was time to treatment failure (TTF) throughout the treatment and follow-up periods (until Day 505 for the last enrolled patient). Results. TTFs were clinically but not statistically significantly longer among patients given MMF after rituximab than among patients receiving rituximab monotherapy (median: 784.0 vs. 472.5 days, hazard ratio (HR): 0.593, 95% confidence interval (CI): 0.336-1.049, log-rank test: P=0.0694). Because most patients in the MMF group presented with treatment failure after MMF discontinuation, we performed a post-hoc analysis limited to the treatment period and found that MMF after rituximab prolonged the TTF and decreased the risk of treatment failure by 80% (HR: 0.202, 95% CI: 0.081-0.503). Moreover, MMF after rituximab reduced the relapse rate and daily steroid dose during the treatment period by 74% and 57%, respectively. The frequency and severity of adverse events were similar in both groups. Conclusions. Administration of MMF after rituximab may sufficiently prevent the development of treatment failure and is well tolerated, although the relapse-preventing effect disappears after MMF discontinuation.

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