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Kidney Week

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


  • 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

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.


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.


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).


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.


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