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Abstract: PO1982

Renal Activity Index in Lupus (RAIL) Score Distinguishes Responder and Non-Responder in Pediatric Lupus Nephritis

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Cody, Ellen, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Wenderfer, Scott E., Baylor College of Medicine, Houston, Texas, United States
  • Ma, Qing, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Merritt, Angela, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Devarajan, Prasad, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Brunner, Hermine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
Background

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). 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. 15 patients were included, 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-responders had no difference or an increase in RAIL score, and a change scoreis >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.

Funding

  • NIDDK Support