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

• Published in: Pediatric nephrology (Berlin, West) Vol. 37; no. 11; pp. 2687 - 2697
• Main Authors: Kallash, Mahmoud, Vogt, Beth A., Zeid, Ahmed, Khin, Ei, Najjar, Mohammed, Aldughiem, Ahmad, Benoit, Elizabeth, Stotter, Brian, Rheault, Michelle, Warejko, Jillian K., Daga, Ankana
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.11.2022; Springer Nature B.V
• Subjects: Acute Kidney Injury; Adolescent; Adult; Aged; Biopsy; Child; Child, Preschool; Creatinine; Epidermal growth factor receptors; Hematuria; Humans; IgA Vasculitis - complications; IgA Vasculitis - diagnosis; IgA Vasculitis - drug therapy; Immunoglobulin A; Immunosuppressive Agents - therapeutic use; Infant; Kidneys; Medicine; Medicine & Public Health; Nephritis; Nephritis - pathology; Nephrology; Nephrotic Syndrome; Original Article; Patients; Pediatrics; Proteinuria; Proteinuria - etiology; Proteinuria - pathology; Retrospective Studies; Steroid hormones; Steroids; Urology; Vasculitis; Young Adult; Nephritis; Children; HSP; IgA vasculitis; Proteinuria
• ISSN: 0931-041X; 1432-198X; 1432-198X
• DOI: 10.1007/s00467-022-05496-3

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 m 2 ) 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 .