IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome

Pediatr Nephrol. 2020 Aug;35(8):1529-1561. doi: 10.1007/s00467-020-04519-1. Epub 2020 May 7.

Abstract

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

Keywords: Children; Chronic kidney disease; Genetics; Immunosuppressive treatment; Outcome; Pediatrics; Steroid-resistant nephrotic syndrome.

Publication types

  • Practice Guideline
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Drug Resistance
  • Female
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / adverse effects*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Infant, Newborn
  • Male
  • Nephrotic Syndrome / diagnosis
  • Nephrotic Syndrome / drug therapy*
  • Proteinuria / urine
  • Remission Induction / methods

Substances

  • Glucocorticoids
  • Immunosuppressive Agents