Use of EuroLupus Cyclophosphamide Dosing for the Treatment of Lupus Nephritis in Childhood-Onset Systemic Lupus Erythematosus in North America

Laura Cannon¹, Scott Wenderfer², Laura Lewandowski³, Jennifer Cooper⁴, Beatrice Goilav⁵, Andrea Knight⁶, Aimee Hersh⁷, Stacy Ardoin⁸ and Rebecca Sadun¹, ¹Duke University, Durham, NC, ²Baylor College of Medicine, Houston, TX, ³NIAMS, NIH, Rockville, MD, ⁴University of Colorado/Children's Hospital Colorado, Denver, CO, ⁵Children's Hospital at Montefiore, Bronx, NY, ⁶Hospital for Sick Children, Toronto, ON, Canada, ⁷University of Utah, Salt Lake City, UT, ⁸Nationwide Children's Hospital, Columbus, OH

Meeting: ACR Convergence 2021

Keywords: Lupus nephritis, Pediatric rheumatology, Surveys, Systemic lupus erythematosus (SLE)

Session Information

Date: Sunday, November 7, 2021

Session Title: Pediatric Rheumatology – Clinical Poster II: SLE, JDM, & Juvenile Scleroderma (0764–0785)

Session Type: Poster Session B

Session Time: 8:30AM-10:30AM

Background/Purpose: Childhood-onset systemic lupus erythematosus (cSLE) has higher rates of lupus nephritis (LN) than adult-onset SLE, often requiring intensive immunosuppression. This study examined North American practices and preferences for the low-dose EuroLupus cyclophosphamide protocol, as compared to the high-dose National Institute of Health (NIH) cyclophosphamide protocol, to treat LN in cSLE.

Methods: A 35-item web-based survey was distributed to Childhood Arthritis and Rheumatology Research Alliance (CARRA) and Pediatric Nephrology Research Consortium (PNRC) providers. The survey assessed participant demographics, cyclophosphamide prescribing practices, perceptions of EuroLupus protocol, and LN vignette treatment decisions; one vignette was taken from a 2009 CARRA survey and responses were compared. Multivariable logistic regression analyzed provider factors associated with practices and preference for low- versus high-dose cyclophosphamide.

Results: Responses were provided by 185/421 (44%) CARRA physicians (Table 1). Among respondents who prescribed cyclophosphamide for pediatric LN over the past year (n=135), half reported using EuroLupus protocol. When presented the same vignette about an adolescent with class IV LN, 32% of pediatric rheumatologists chose EuroLupus dosing in 2020, versus only 6% in 2009 (Figure 1). Provider factors associated with choosing the low-dose regimen were familiarity with the protocol (OR 4.2, p=0.006) and greater perceived benefit (OR 1.6, p< 0.0001) (Table 2). Forty pediatric nephrologists had similar responses to the pediatric rheumatology providers. Overall, 78% of respondents perceived EuroLupus protocol efficacy to be equivalent to the high-dose protocol in cSLE LN.

Conclusion: Of pediatric specialists who prescribe cyclophosphamide for cSLE LN, more use low-dose cyclophosphamide compared to a decade prior. Nevertheless, familiarity with EuroLupus dosing remains low.

Table 1. Demographics of survey respondents.
CARRA=Childhood Arthritis and Rheumatology Research Alliance
PNRC=Pediatric Nephrology Research Consortium
IV=intravenous
LN=lupus nephritis

Figure 1. Change over a decade in pediatric rheumatologist preferences for first-line treatment of lupus nephritis in childhood-onset systemic lupus erythematosus. Survey respondents from the Childhood Arthritis and Rheumatology Research Alliance membership (n=134) were presented with the same clinical vignette that had been posed in 2009 (n=71) regarding first-line therapy for newly diagnosed SLE with class IV lupus nephritis in a 14-year-old girl.
CYC=cyclophosphamide
MMF=mycophenolate mofetil
EL= EuroLupus
NIH= National Institute of Health

Table 2. Provider factors associated with use of EuroLupus cyclophosphamide dosing based on results of logistic regression models.
Shown are the results from a logistic regression model comparing provider factors for those selecting EuroLupus dosing vs NIH dosing who had prescribed cyclophosphamide for pediatric LN over the past 12 months (n=135).
NIH= National Institute of Health
LN= lupus nephritis

Disclosures: L. Cannon, None; S. Wenderfer, Bristol-Myers Squibb, 2; L. Lewandowski, None; J. Cooper, None; B. Goilav, None; A. Knight, None; A. Hersh, None; S. Ardoin, Aurinia, 2, American Board of Pediatrics, 4, Childhood Arthritis and Rheumatology Research Alliance, 4; R. Sadun, None.

To cite this abstract in AMA style:

Cannon L, Wenderfer S, Lewandowski L, Cooper J, Goilav B, Knight A, Hersh A, Ardoin S, Sadun R. Use of EuroLupus Cyclophosphamide Dosing for the Treatment of Lupus Nephritis in Childhood-Onset Systemic Lupus Erythematosus in North America [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/use-of-eurolupus-cyclophosphamide-dosing-for-the-treatment-of-lupus-nephritis-in-childhood-onset-systemic-lupus-erythematosus-in-north-america/. Accessed December 5, 2022.

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