The scope of treatment of pediatric IgA vasculitis nephritis and its outcome: a Pediatric Nephrology Research Consortium study

Mahmoud Kallash, Beth A. Vogt, Ahmed Zeid, Ei Khin, Mohammed Najjar, Ahmad Aldughiem, Elizabeth Benoit, Brian Stotter, Michelle Rheault, Jillian K. Warejko, Ankana Daga

Research output: Contribution to journalArticlepeer-review

Abstract

Background: IgA vasculitis (IgAV) is the most common type of vasculitis in children. There is a lack of consensus for management of significant IgAV nephritis (IgAVN). This study was designed to identify the most used treatment options and describe their efficacy. Methods: This is a multicenter retrospective study of children age 1–21 years with IgAVN who were managed for at least 6 months by a nephrologist. Subjects with at least microscopic hematuria and proteinuria and/or decreased kidney function were enrolled. Kidney outcome was assessed by eGFR and urine protein/creatinine (UPC) ratios at 2–4 weeks, 3, 6, and 12 months post-diagnosis. Results: A total of 128 subjects with median age of 7 years (range 2–18) were included. Of these, 69 subjects had kidney biopsy with crescents detected in 53%. AKI (P = 0.039), nephrosis (P = 0.038), and crescents on biopsy (P = 0.013) were more likely in older patients. Patients with UPC > 1 mg/mg were more likely to get a kidney biopsy (P < 0.001) and to be treated with steroids ± immunosuppressive (IS) agents (P = 0.001). Sixty-six percent of patients were treated with steroids and/or IS agents for variable durations. Anti-metabolite agents were the most common IS agents used with variability in dosing and duration. At 12 months, most subjects had a normal eGFR (79%) (median 123, range 68–207 mL/min/1.73 m2) and no proteinuria (median UPC 0.15, range 0.01–4.02 mg/mg). Conclusions: IS agents are frequently used in managing IgAVN associated with heavy proteinuria, nephrosis, and/or AKI. Prospective studies are needed to determine indications and needed duration of IS therapy. Graphical abstract: A higher resolution version of the Graphical abstract is available as Supplementary information. [Figure not available: see fulltext.]

Original languageEnglish
Pages (from-to)2687-2697
Number of pages11
JournalPediatric Nephrology
Volume37
Issue number11
DOIs
StatePublished - Nov 2022

Keywords

  • Children
  • HSP
  • IgA vasculitis
  • Nephritis
  • Proteinuria

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