Abstract: FR-PO0821
A B-Cell-Targeted Approach to Steroid-Resistant Nephrotic Syndrome in Children
Session Information
- Glomerular Diseases: Practice and New Concepts Shaping Modern Care
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Moss, Haley Elizabeth, University of California San Diego, La Jolla, California, United States
- Ravi, Tanmaya, University of California San Diego, La Jolla, California, United States
- Athavale, Ambarish, University of California San Diego, La Jolla, California, United States
Background
Although 90% of childhood nephrotic syndrome (NS) patients respond to steroids, 60-90% will relapse. We report outcomes in patients with steroid sensitive and resistant childhood NS treated with rituximab.
Methods
We performed a retrospective chart review of pediatric patients with primary NS treated at Rady Children’s Health San Diego between March 2002 and March 2019.
Results
Population included 31 patients treated with rituximab. Mean age at diagnosis was 3 years (SD 3.9) with 22 (71%) identified as male at birth. All patients were diagnosed via biopsy, with 21 (68%) having Minimal Change Disease, 6 (19%) having FSGS, and 4 (13%) having IgM Nephropathy. 20 (65%) were steroid sensitive. Prior to rituximab infusion, all 31 patients received steroids, 25 (81%) tacrolimus, 19 (61%) mycophenolate mofetil, 9 (29%) cyclosporin, and 9 (29%) cyclophosphamide. Median serum creatinine prior to infusion was 0.4 (IQR 0.3, 0.52) mg/dL, with median eGFR of 115.6 (IQR 95.4, 141.05) mL/min/1.73m2 calculated using CKiD U25 Creatinine, and median urine protein: creatine ratio of 0.75 (IQR 0.29, 17.92). The median number of rituximab infusions was 8 (IQR 5,10) with 21 (68%) patients achieving complete remission, 7 (23%) having a partial response, and 3 (9%) having no response. All patients with no response to rituximab (3) were steroid resistant. 14 patients (44%) experienced an infusion reaction.
Conclusion
In a real-world cohort of pediatric patients with biopsy-proven podocytopathies, rituximab therapy was associated with high rates of remission, particularly in steroid-responsive disease, while nonresponse was confined to steroid-resistant patients. These findings suggest that B-cell-directed therapy may have an important role in selected pediatric nephrotic syndrome populations and provide a strong rationale for prospective, phenotype-stratified clinical trials.
| Total number of patients; n | 31 |
| Age in years at diagnosis; mean (SD) | 3 (3.9) |
| Sex; n (%) | Male: 22 (71%) |
| Ethnicity; n (%) | Hispanic or Latino: 15 (48%) Not Hispanic or Latino: 16 (52%) |
| Race; n (%) | White: 19 (61%) Other: 4 (13%) Hispanic: 4 (13%) African American: 3 (10%) Asian: 1 (3%) |
| Pathologic diagnosis; n (%) | Minimal Change Disease: 21 (68%) FSGS: 6 (19%) IgM Nephropathy: 4 (13%) |
| Serum Cr; median (IQR Q1, Q3) | 0.4 (IQR 0.3, 0.52) mg/dL |
| Urine protein:creatinine ratio; median (IQR Q1, Q3) | 0.75 (IQR 0.29, 17.92) |
| Steroid responsive (Y/N); n (%) | Yes: 20 (65%) |
| # of immunosuppressive medications prior to 1st rituximab infusion; mean (SD) | 2.88 of 5 options (0.89) |
| Response to rituximab (Complete remission, partial remission, no response); n (%) | Complete: 21 (68%) Partial: 7 (23%) No response: 3 (9%) |