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Abstract: PO1577

Natural History of IgA Nephropathy: Analysis of a UK National RaDaR IgA Nephropathy Cohort

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Barratt, Jonathan, University of Leicester, Leicester, Leicestershire, United Kingdom
  • Saleem, Moin, University of Bristol, Bristol, Bristol, United Kingdom
  • Braddon, Fiona E. M., UK Renal Registry, Bristol, Bristol, United Kingdom
  • Carroll, Kevin, KJC Statistics Ltd, Cheshire, England, United Kingdom
  • He, Ping, Travere Therapeutics Inc, San Diego, California, United States
  • Hendry, Bruce M., Travere Therapeutics Inc, San Diego, California, United States
  • Mercer, Alex, JAMCO Pharma Consulting, Stockholm, Sweden
  • Pitcher, David, UK Renal Registry, Bristol, United Kingdom
  • Steenkamp, Retha D., UK Renal Registry, Bristol, United Kingdom
  • Turner, A. Neil, University of Edinburgh, Edinburgh, Edinburgh, United Kingdom
  • Gale, Daniel P., Royal Free Hospital, London, London, United Kingdom
Background

Primary IgA nephropathy (IgAN) is the most common form of glomerulonephritis and a major cause of renal failure. Here we describe the natural history of IgAN using the UK National Registry of Rare Kidney Diseases (RaDaR). Since 2013, patients with biopsy-proven IgAN and eGFR <60 mL/min/1.73m2 or proteinuria ≥1g/24h have been enrolled into RaDaR from 107 adult and paediatric kidney units across the UK, including retrospective and prospective data. Patients with systemic vasculitis or pre-existing liver disease were excluded.

Methods

Baseline date was defined as first occurrence of renal biopsy, primary renal diagnosis or symptom presentation. Patients were grouped into those with ESKD (CKD stage 5 or renal replacement therapy) at or prior to baseline (ESKD group) and those without ESKD at baseline with ≥12 months follow-up (CKD group). For survival analyses, ESKD or death was applied with survival time calculated from baseline to last follow-up.

Results

In the ESKD group (n=326), median age at first dialysis (56% of patients) and kidney transplant (7%) was 38 yrs (IQR 29-50). In the CKD group (n=1838), median baseline age was 39 yrs (IQR 28-50) with paediatric onset of disease comprising 6%. Baseline median urine PCR was 1.5 g/g (IQR 0.6-3.2; n=356) and mean eGFR was 58 mL/min/1.73m2 (SD 32; n=440). Median follow-up was 9.2 years (IQR 5.1-16.3) and ESKD/death occurred in 53% of patients (<1% death). Kaplan-Meier survival curves of paediatric and adult patients show 50% survival probability of 24 & 10 years, respectively (Figure 1).

Conclusion

RaDaR contains a large cohort with long follow up enabling detailed investigation of the natural history of IgAN. These results indicate associations between rapid disease progression and poor outcomes, highlighting a need for effective treatments for patients with IgAN with renal impairment or >1g/24h proteinuria.

Funding

  • Commercial Support