RAIL Distinguishes Responder and Non-Responder in Pediatric Lupus Nephritis

Ellen Cody¹, Scott Wenderfer², Qing Ma¹, Angela Merritt¹, Prasad Devarajan¹ and Hermine Brunner¹, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Baylor College of Medicine, Houston, TX

Meeting: ACR Convergence 2021

Keywords: Biomarkers, Lupus nephritis, Pediatric rheumatology

Session Information

Date: Saturday, November 6, 2021

Session Title: SLE – Diagnosis, Manifestations, & Outcomes Poster I: Diagnosis (0323–0356)

Session Type: Poster Session A

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

Background/Purpose: Systemic Lupus Erythematosus (SLE) is a diagnostic and therapeutic challenge, particularly lupus nephritis (LN). We described a composite score, the Renal Activity Index for Lupus (RAIL), consisting of neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), monocyte chemotactic protein 1 (MCP-1), adiponectin, hemopexin and ceruloplasmin, where higher scores reflect more active inflammation on biopsy. We hypothesize that when followed longitudinally during induction therapy, a change in RAIL score distinguishes clinical responders from non-responders.

Methods: Pediatric patients (< 18 years) diagnosed with LN were included (IRB #2008-0635), N=15. Diagnosis was made according to ACR criteria for SLE with renal biopsy confirmation of LN. Urine was collected at diagnosis and end of induction. Responders were defined by urine protein to creatinine ratio < 0.2 mg/mg, absence of hematuria, and normal glomerular filtration rate. Response also defined as improved activity index on follow up biopsy. There were 10 responders, 5 non-responders. Analysis by T-test, as well as sensitivity and specificity for no change in RAIL score.

Results: RAIL score in the responder group pre and post therapy was significantly different, p-value 0.015. T-Test between non-responder and responder difference scores showed trend towards significance, p-value 0.081 (Fig 1). Most responders had a difference of at least 0.5 during induction, whereas most non-responder had no difference or an increase in RAIL score, and a change score >0 identified responders with 90% sensitivity.

Conclusion: A change in RAIL during induction therapy is promising for predicting responders vs non-responders, with average decrease of 1 compared to no change. To further evaluate, more samples are needed, which is on-going.

Figure 1: Change in RAIL Score in Clinical Non-Responders vs Responders

Disclosures: E. Cody, None; S. Wenderfer, Bristol-Myers Squibb, 2; Q. Ma, None; A. Merritt, None; P. Devarajan, BioPorto Inc, 1; H. Brunner, Novartis, 6, Pfizer, 6, Roche, 6, GlaxoSmithKline, 6, Abbvie, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Biogen, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, AstraZeneca-Mediimmune, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Boehringer, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, BMS, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Celgene, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Eli Lilly, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, EMD Serono, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Idorsia, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Cerocor, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, F.Hoffman-La Roche, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Merck, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Novartis, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Sanofi, 12, Contributions to employer (Cincinatti Children's Hospital) *NOTE: This funding has been reinvested for the research activities of the hospital in a fully independent manner without any commitment to third parties, Aurina, 2.

To cite this abstract in AMA style:

Cody E, Wenderfer S, Ma Q, Merritt A, Devarajan P, Brunner H. RAIL Distinguishes Responder and Non-Responder in Pediatric Lupus Nephritis [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/rail-distinguishes-responder-and-non-responder-in-pediatric-lupus-nephritis/. Accessed February 3, 2023.

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