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Abstract: SA-OR059

Percentage of Glomerular Crescents Predicts Renal Outcomes in Children with IgA Vasculitis – A Midwest Pediatric Nephrology Consortium Study

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Wenderfer, Scott E., Baylor College of Medicine, Houston, Texas, United States
  • Rheault, Michelle N., University of Minnesota, Minneapolis, Minnesota, United States
  • Selewski, David T., University of Michigan, Ann Arbor, Michigan, United States
  • Tran, Cheryl L., Mayo Clinic, Rochester, Minnesota, United States
  • Pan, Cynthia G., Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Seamon, Meredith, The University of Utah, Salt Lake City, Utah, United States
  • Nelson, Raoul D., The University of Utah, Salt Lake City, Utah, United States
  • Twombley, Katherine, Medical University of South Carolina, Charleston, South Carolina, United States
  • Onder, Ali Mirza, University of Tennessee, Memphis, Tennessee, United States
  • Flynn, Joseph T., Seattle Children's Hospital, Seattle, Washington, United States
  • Maliakkal, Joseph George, Saint Louis University School of Medicine, St. Louis, Missouri, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Hidalgo, Guillermo, Eastern Carolina University, Greenville, North Carolina, United States
Background

While crescents on kidney biopsy predict poor outcome for children with IgA vasculitis, evidence-based thresholds for %crescents most predictive of renal risk are not established. The International Study of Kidney Disease in Children (ISKDC) used 50% and 75% thresholds to grade severity of crescentic glomerulonephritis. MEST-C scoring for IgA nephropathy has also been proposed for IgA vasculitis: 1 point for any glomerular crescent and 2 when crescents involve >25% of glomeruli.

Methods

To test the validity of 25, 50, and 75% thresholds for crescents in IgA vasculitis, we identified 62 patients (13 centers) in the IRB-approved Midwest Pediatric Nephrology Consortium Glomerulonephritis with Crescents Registry (18% of total). Enrollment included patients <21 yo with >1 crescentic glomerulus on biopsy from 2004-16. Primary outcome was end stage kidney disease (ESKD) at 1 year. Secondary outcomes included estimated glomerular filtration rate (eGFR) at 1 year and change in eGFR over time. Crescents were defined by local renal pathologist.

Results

The cohort was 47% female, 53% white, 3% black, 18% Hispanic, and 3% Asian. Median age at biopsy was 8 years (range 6-11) with 2.6 years median follow-up (IQR 1.7-4.8). A median of 36 glomeruli were sampled per biopsy (IQR 27-57). The median %crescents was 10.8 (IQR 7-18%, max 83%). Cellular crescents were seen in 95% of biopsies; fibrous crescents in 27%; both in 23%. Only 2% of children had crescents in >50% of glomeruli, and only one in >75% of glomeruli. One child with 13% cellular and 3% fibrous crescents advanced to ESKD at 26 months post-biopsy. Median change in eGFR was +2.8 mL/min/1.73m2/year (IQR -20 to +24) at 1 year and -1.5 (IQR -7 to +6) at latest follow-up. Change in eGFR was 2-fold greater for children with >25% crescents (-3.6) vs. those with <25% (-1.6).

Conclusion

Children with higher %crescents present with lower GFRs but most have significant recovery of eGFR at 1 year. Utility of the ISKDC thresholds for grading crescents in IgAV is limited, due to low prevalence of biopsies with crescents >50%. Long term, the 25% threshold for cellular crescents appears to predict worsening GFR decline.