Rituximab for Refractory Lupus Nephritis and Flare: Pediatric Rheumatologists vs Nephrologists

Girl in hospital bed attended by mother and doctor
Using a web-based survey, study authors assessed the current practices of pediatric nephrologists and rheumatologists in North America with regard to the treatment of refractory proliferative lupus nephritis.

For patients with childhood-onset systemic lupus erythematosus (SLE), rituximab (RTX) use among pediatric nephrologists and rheumatologists for the treatment of proliferative lupus nephritis (LN) refractory to induction therapy for LN flare following remission vary widely, according to findings from a survey published in Pediatric Rheumatology Online Journal.

Study authors sought to assess the current practices among pediatric rheumatologists and nephrologists in North America regarding the treatment of refractory proliferative LN and flare.

In November 2015, a survey was distributed to members of the American Society for Pediatric Nephrology (ASPN) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) who had completed fellowship programs in pediatric nephrology and pediatric rheumatology, respectively, to evaluate treatment choices. The survey presented 2 cases for assessment: refractory LN after induction therapy with corticosteroid and cyclophosphamide (CYC) therapy; and LN flare following initial response to treatment. Survey respondents selected treatments for 3 follow-up scenarios for each case that differed according to severity of the presentation. Therapeutic options included CYC, mycophenolate mofetil (MMF), RTX, and other agents, alone or in combination.

Representing approximately 15% of the eligible members from each of the 2 organizations, 76 respondents from ASPN and 41 respondents from CARRA completed the survey. Overall, 52% of pediatric nephrologists reported that they managed less than 25 patients with SLE, while half of all pediatric rheumatologists reported that they managed between 25 and 100 patients with SLE. Further, only 51% of pediatric nephrologists and 24% of pediatric rheumatologists reported that they followed a standard protocol for the treatment of patients with LN.

Treatment choices of pediatric nephrologists and rheumatologists varied greatly, with more than a 50% agreement regarding an individual treatment selection in only 2 of 6 follow-up scenarios: 59% of pediatric nephrologists and 38% of pediatric rheumatologists selected increasing dose of MMF in LN refractory to induction therapy with proteinuria, hematuria, and improved serum creatinine.

In a follow-up scenario that demonstrated severe renal flare after attaining remission with induction therapy, 58% of pediatric rheumatologists vs 43% of pediatric nephrologists selected CYC plus RTX; pediatric nephrologists chose CYC alone as their preferred choice for treatment.

Overall, pediatric rheumatologists vs pediatric nephrologists selected significantly more therapeutic options that included RTX in all of the follow-up scenarios except one (P <.05).

Study authors concluded, “Further investigation is necessary to delineate the reasons behind [these findings]. This study highlights the importance of collaborative efforts in developing consensus treatment plans for pediatric LN.”


Gilbert M, Goilav B, Hsu JJ, et al; Pediatric Nephrology and Rheumatology Collaborative Group, the Childhood Arthritis and Rheumatology Research Alliance, and the American Society for Pediatric Nephrology. Differences in rituximab use between pediatric rheumatologists and nephrologists for the treatment of refractory lupus nephritis and renal flare in childhood-onset SLE. Pediatr Rheumatol Online J. 2021;19(1):137. doi:10.1186/s12969-021-00627-w