IgA nephropathy with presentation of nephrotic syndrome at onset in children

Pediatr Nephrol. 2017 Mar;32(3):457-465. doi: 10.1007/s00467-016-3502-6. Epub 2016 Oct 6.


Background: Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated.

Methods: We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN.

Results: Among 426 patients, 30 (7.0 %) had NS-IgAN. Logistic analyses showed that male sex (OR: 7.6, p = 0.0002), M1 (OR: 10.3, p = 0.002), and E1 (OR: 15.2, p = 0.0001) were significantly related to NS. The mean observation period was 6.2 ± 3.2 years. Although NS-IgAN was associated with significantly lower renal survival than non-NS-IgAN according to Kaplan-Meier analysis (p = 0.02), renal survival of NS-IgAN was good (92.4 % at 10 years). The most significant prognostic factor for renal survival was remission of proteinuria after treatment, and NS at onset is also a significant prognostic factor for renal survival after adjusting for remission of proteinuria. Twenty children with NS-IgAN were treated with prednisolone alone, or prednisolone and immunosuppressant. Remission of proteinuria occurred in 21 patients. Three cases of NS-IgAN progressed to stage III-V chronic kidney disease at the most recent observation. They all demonstrated heavy proteinuria after the 2-year initial treatment. The significant factor for persistent proteinuria at 5 years was S1 in NS-IgAN.

Conclusions: The most significant factor for renal survival was responsiveness to treatment, not NS itself. As modifiable acute lesions are the dominant pathological findings in NS-IgAN, histological improvements achieved by appropriate treatments can result in a favorable prognosis.

Keywords: End-stage renal disease; IgA nephropathy; Nephrotic syndrome; Remission of proteinuria.

MeSH terms

  • Age of Onset
  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Glomerulonephritis, IGA / complications*
  • Glomerulonephritis, IGA / drug therapy
  • Glomerulonephritis, IGA / pathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kaplan-Meier Estimate
  • Kidney / pathology
  • Kidney Failure, Chronic / etiology
  • Male
  • Nephrotic Syndrome / complications*
  • Nephrotic Syndrome / drug therapy
  • Nephrotic Syndrome / pathology
  • Prednisolone / therapeutic use
  • Prognosis
  • Proteinuria / drug therapy
  • Proteinuria / etiology
  • Renal Insufficiency, Chronic / drug therapy
  • Renal Insufficiency, Chronic / etiology
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Prednisolone