Non-immunosuppressive therapies for childhood IgA nephropathy

Abstract

IgA nephropathy (IgAN) is the most common chronic primary glomerulonephritis in both children and adults, and 20–30% of patients with persistent hematuria/proteinuria progress to kidney failure within 20 years. In Japan, most cases of childhood IgAN are detected by school screening programs during the early onset of the disease when hematuria/proteinuria are asymptomatic and kidney function is normal. Therefore, it is possible to follow the detailed clinical course and appropriate therapeutic interventions from early onset of the disease. Data on non-immunosuppressive therapies for children with IgAN are highly limited. The Japanese Pediatric IgA Nephropathy Treatment Study Group was organized in 1989 to conduct clinical trials and accumulate data on treatments for childhood IgAN. In this review, we focus on non-immunosuppressive therapies, notably with renin-angiotensin-aldosterone system (RAAS) inhibitors for childhood IgAN and related clinical trials conducted primarily in Japan. We also describe the anti-inflammatory and antiproteinuric effects of RAAS inhibitors in IgAN, differences in treatment regimens because of the acute and active pathological features of childhood IgAN, adverse events of RAAS inhibitors, other non-immunosuppressive treatment options, and future directions.

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Acknowledgments

We thank Richard Robins, PhD, from Edanz Group (https://en-author-services.edanzgroup.com/) for editing a draft of this manuscript.

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Correspondence to Yuko Shima.

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1. b; 2. c; 3. d; 4. e; 5. e

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Shima, Y., Nakanishi, K. & Yoshikawa, N. Non-immunosuppressive therapies for childhood IgA nephropathy. Pediatr Nephrol (2021). https://doi.org/10.1007/s00467-021-04954-8

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Keywords

  • Childhood IgA nephropathy
  • Non-immunosuppressive treatment
  • Clinical trials
  • Renin-angiotensin-aldosterone system inhibitors
  • ACE escape phenomenon