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Abstract: PO2341

Clinical Course and Management in Children with Henoch Schönlein Purpura (HSP) Nephritis

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Stone, Hillarey, Cincinnati Children's Hospital Medical Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, US, Cincinnati, Ohio, United States
  • Nehus, Edward, Cincinnati Children's Hospital Medical Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, US, Cincinnati, Ohio, United States
  • Dickinson, Kimberley, The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Burrows, Evanette Kenvis, The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Luna, Ingrid Y., The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Gluck, Caroline A., Nemours Children's Hospital, Wilmington, Delaware, United States
  • Dixon, Bradley P., Children's Hospital Colorado, Aurora, Colorado, United States
  • Dharnidharka, Vikas R., St Louis Children's Hospital, St. Louis, MO, US, St. Louis, Missouri, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, OH, US, Columbus, Ohio, United States
  • Somers, Michael J., Boston Children's Hospital, Boston Children's Hospital, Boston, MA, US, Boston, Massachusetts, United States
  • Flynn, Joseph T., Seattle University, Seattle University, Seattle, WA, US, Seattle, Washington, United States
  • Neu, Alicia, Johns Hopkins University, Baltimore, Maryland, United States
  • Furth, Susan L., The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Forrest, Christopher B., The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Denburg, Michelle, The Children's Hospital of Philadelphia, The Children's Hospital of Philadelphia, Philadelphia, PA, US, Philadelphia, Pennsylvania, United States
  • Mitsnefes, Mark, Cincinnati Children's Hospital Medical Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, US, Cincinnati, Ohio, United States
Background

HSP can lead to serious complications including HSP nephritis (HSPN). Controversy exists regarding outcomes of HSPN, varying between benign, self-limited disease and 2-19% risk of severe chronic kidney disease (CKD). Given this risk, treatment with immunosuppression may be warranted. Unfortunately, evidence-based guidelines for management of HSPN are lacking. The goals of this study were to identify children with HSP and describe the clinical course and management in those with HSPN using a large, multicenter electronic health record (EHR) database.

Methods

Children with HSP were identified using PEDSnet, a network of pediatric health systems with EHR data standardized in a common data model. In patients with HSP, contact with nephrology was used as an indicator of HSPN. Demographics and clinical data among those seen by nephrology were compared to those not seen by nephrology. Outcomes of hypertension, dialysis, and transplant were identified using standard terminologies (SNOMED and CPT4). Among those with HSPN, treatment and outcomes are described.

Results

From 2009 to 2020, 5,360 patients with HSP were identified, including 1,217 (23%) with HSPN. Average length of follow up was 2.7 years. Patients with HSPN were older (7.9 vs 6.2 years, p<0.001), with no difference in sex or race/ethnicity. Among those with HSPN, 128 (10%) had kidney biopsy. Treatment included renin-angiotensin system (RAS) blockade in 166 (14%), corticosteroids in 427 (35%), and other immunosuppressive agents (mostly azathioprine or mycophenolate mofetil) in 97 (8%). Hypertension was diagnosed in 224 (18%) within one year of HSP diagnosis. By the end of follow up, 26 (3%) had estimated glomerular filtration rate <60 mL/min/1.73m2, including 11 (1%) requiring dialysis or kidney transplant.

Conclusion

In this large study of >5,000 children with HSP, 23% developed HSPN with the majority having mild disease. Management of HSPN by pediatric nephrologists most commonly included observation, steroids, and/or RAS blockade. Children with HSPN in this study had an excellent short-term prognosis, with only 3% with advanced CKD or end stage renal disease at last follow up.

Funding

  • Other NIH Support