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Abstract: PO1621

Validation of the Renal Risk Score in Anti-GBM Disease

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Li, Anna S., The University of Manchester Faculty of Biology Medicine and Health, Manchester, Manchester, United Kingdom
  • Floyd, Lauren, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
  • Brown, Nina, Salford Royal NHS Foundation Trust, Salford, Salford, United Kingdom
  • Alderson, Helen, Salford Royal NHS Foundation Trust, Salford, Salford, United Kingdom
  • Dhaygude, Ajay Prabhakar, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
  • Reid, Graeme Thomas, Manchester University NHS Foundation Trust, Manchester, Greater Manchester, United Kingdom
  • Brix, Silke R., The University of Manchester Faculty of Biology Medicine and Health, Manchester, Manchester, United Kingdom
Background

Anti-glomerular basement membrane (GBM) disease is a rare immune mediated kidney lesion of an aggressive nature often resulting in end stage kidney disease (ESKD). Clinical and histological variables predicting outcome are needed to individualise therapy and improve outcome.

Methods

We performed a retrospective multicentre analysis and investigated the Renal Risk Score (RRS) proposed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis for its prognostic value in anti-GBM disease. We used the published cut-offs for percentage of normal glomeruli (N0 > 25%, N1 10 - 25%, N2 < 10%), estimated glomerular filtration rate (eGFR, G0 > 15 ml/min/1.73 m2, G1 ≤ 15 ml/min/1.73 m2) and a simplified cut off for tubular atrophy and interstitial fibrosis (T0 ≤ mild to moderate, T1 ≥ moderate). We assigned points to each parameter (N1 = 4, N2 = 6, G1 = 3, T1 = 2 points) and patients to risk groups, low (0), intermediate (2 - 7), and high risk (8 - 11 points).

Results

Seventy patients with GBM nephritis were identified, 20 patients presented double positive for ANCA and GBM antibodies (28.57%). Median age was 64 years (Interquartile range, IQR 43 – 76 years). 39 patients were female (55.7%). Median eGFR at presentation was 593 ml/min (IQR 419.75 – 835.75 ml/min). Median follow up was 41 months (IQR 11 - 77.5 months), and fifty patients developed ESKD (71.42%). Forty-seven biopsies were available for scoring. Four patients were low risk, and none developed ESKD (0%). Eight patients belonged in the medium risk group, and five of these developed permanent kidney-failure (62.5%). Of 35 patients in the high-risk group, 30 patients developed ESKD (85.7%). Three patients had the highest score of 11 points, and none remained dialysis independent (100%). The risk groups differed in renal survival (p<0.001).

Conclusion

Low percentage of normal glomeruli, higher grade of tubulointerstitial damage and severe kidney failure are associated with poor outcome in anti-GBM disease. Combining these variables, the Renal Risk Score accurately predicted kidney survival in anti-GBM disease.