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Abstract: PO2340

Evaluating Nephrotic Syndrome Response to Rituximab in Children

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Williams, Anna E., Duke University, Durham, North Carolina, United States
  • Twombley, Katherine, Medical University of South Carolina, Charleston, South Carolina, United States
  • Chua, Annabelle N., Duke University, Durham, North Carolina, United States
  • Gbadegesin, Rasheed A., Duke University, Durham, North Carolina, United States
Background

Nephrotic syndrome (NS) is the most common cause of glomerular disease in the pediatric population. In children, 80% of cases are steroid sensitive (SSNS) & 20% are steroid resistant (SRNS). Rituximab (RTX) has been identified as a steroid-sparing therapy with minimal nephrotoxic side effects. The determinants of clinical response to rituximab is not completely known.

Methods

A retrospective review of patients aged 0-21 years with idiopathic NS who received at least 2 doses of RTX therapy over 6 years. Data collected included gender, race, ethnicity, age at diagnosis, steroid response, number of RTX doses, CD20 levels post therapy & outcomes. Outcome was defined as complete remission, CR (urine protein to creatinine ratio mg/mg: UPCR ≤ 0.2), partial remission, PR (UPCR 0.3-1.9) & no response, NR (UPCR ≥ 2). Data were compared by Fisher’s exact & Wilcoxon Rank Sum tests.

Results

48 patients met the inclusion criteria for the study comprising of 23(48%) with SSNS & 25(52%) with SRNS. There was no difference in race or age of onset between the patients with SSNS & SRNS. 18/29(62%) of patients who had CD20 lymphocyte levels measured following treatment achieved therapeutic end point of CD20 lymphocyte depletion. There was no difference in the proportion of patients who achieved this therapeutic end point between the patients with SSNS & SRNS (46% vs 72%). Overall, 72% of patients achieved partial or complete remission. The remission rate was significantly higher in the SSNS group compared with the SRNS group (87% versus 58%, p=0.001); however, there was no difference in remission rate between patients who achieved the therapeutic end point of CD20 lymphocyte depletion & those who did not in the entire cohort (56% vs 55%, p=1.0) as well as in subgroup of patients with SSNS & SRNS.

Conclusion

Children with both SSNS & SRNS achieved the desired therapeutic effect of CD20 lymphocyte depletion following treatment with RTX; however, disease remission rate was higher in children with SSNS. This data suggests that RTX can be administered at any phase of the disease (relapse or remission) without jeopardizing clinical response.