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Background <p>Patients with IgA nephropathy (IgAN) occasionally present with nephrotic range proteinuria (NRP), but the clinical features and long-term renal prognosis of patients with IgAN-related NRP (IgAN-NRP) with or without nephrotic syndrome (NS) remain elusive.</p> Methods <p>A retrospective, multicenter, cohort study enrolled 788 patients with IgAN from 42 medical centers in 2002–2004. Patients were divided into NRP (group I, n = 39) and non-NRP (group II, n = 749) groups. Group I patients were subdivided into NRP with NS (I-A, n = 14) and NRP without NS (I-B, n = 25) groups. The primary outcome (PO) was a 1.5-fold increase in serum creatinine from baseline. Clinical remission (CR) was defined as both hematuria and proteinuria remission on ≥3 consecutive tests over at least 6&#xa0;months.</p> Results <p>Compared with group II, group I had significantly more severe renal dysfunction and received steroid therapy more frequently. During a median follow-up of 90.0&#xa0;months, more patients reached the PO in group I (38.5%) than in group II (11.0%). Multivariate analysis of all enrolled patients showed that NRP was a significant independent risk factor for the PO. However, in group I, 10 of 11 patients who achieved CR had a favorable renal prognosis, and corticosteroid therapy significantly attenuated the risk for the PO. When comparing groups I-A and I-B, baseline renal impairment and cumulative probabilities of the PO were comparable.</p> Conclusion <p>Regardless of the presence of NS, IgAN-NRP is a critical factor leading to a poor prognosis, unless CR is achieved. Intensive treatment might be vital for IgAN-NRP.</p>

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Clinical factors associated with long-term renal outcomes in Japanese patients with IgA nephropathy presenting with nephrotic range proteinuria: a multicenter, retrospective, cohort study

  • 期刊论文

  • 发表日期：

    2025年9月12日

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  • Tomohiro Saito,
  • Yukihiro Wada,
  • Takanori Shibata,
  • Takashi Yasuda,
  • Yoshinari Yasuda,
  • Keita Hirano,
  • Keiichi Matsuzaki,
  • Tetsuya Kawamura,
  • Yusuke Suzuki,
  • Shoichi Maruyama

摘要

Background

Patients with IgA nephropathy (IgAN) occasionally present with nephrotic range proteinuria (NRP), but the clinical features and long-term renal prognosis of patients with IgAN-related NRP (IgAN-NRP) with or without nephrotic syndrome (NS) remain elusive.

Methods

A retrospective, multicenter, cohort study enrolled 788 patients with IgAN from 42 medical centers in 2002–2004. Patients were divided into NRP (group I, n = 39) and non-NRP (group II, n = 749) groups. Group I patients were subdivided into NRP with NS (I-A, n = 14) and NRP without NS (I-B, n = 25) groups. The primary outcome (PO) was a 1.5-fold increase in serum creatinine from baseline. Clinical remission (CR) was defined as both hematuria and proteinuria remission on ≥3 consecutive tests over at least 6 months.

Results

Compared with group II, group I had significantly more severe renal dysfunction and received steroid therapy more frequently. During a median follow-up of 90.0 months, more patients reached the PO in group I (38.5%) than in group II (11.0%). Multivariate analysis of all enrolled patients showed that NRP was a significant independent risk factor for the PO. However, in group I, 10 of 11 patients who achieved CR had a favorable renal prognosis, and corticosteroid therapy significantly attenuated the risk for the PO. When comparing groups I-A and I-B, baseline renal impairment and cumulative probabilities of the PO were comparable.

Conclusion

Regardless of the presence of NS, IgAN-NRP is a critical factor leading to a poor prognosis, unless CR is achieved. Intensive treatment might be vital for IgAN-NRP.