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Abstract: PO1938

Validation of a Clinical-Pathologic Renal Risk Score in ANCA-Associated Glomerulonephritis: The US Experience

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kant, Sam, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Brix, Silke R., Manchester Royal Infirmary, Manchester, Manchester, United Kingdom
  • Costigliolo, Francesca, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Fenaroli, Paride, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Rosenberg, Avi Z., Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Geetha, Duvuru, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
Background

The prognostication of renal disease in the setting of ANCA associated vasculitis (AAV) continues to pose a significant clinical challenge given lack of validated clinical and pathologic correlates. A risk score has been developed by a German consortium (Brix et al Kidney International 2018), to stratify risk of AAV-related renal disease progression to ESKD. We applied this risk score in our cohort of AAV patients to ascertain its reproducibility.

Methods

A single center retrospective cohort study was performed reviewing 148 renal biopsies of patients with AAV GN. Data for score calculation was available of 119 patients with a median follow up of 58 months (IQR 28 – 97 months). Three parameters were used in the risk prediction score: (1) # of normal glomeruli (N0 >25%, N1 10-25%, N2 <10%), (2) tubular atrophy and interstitial fibrosis (T0 <25%, T1 >25%) and (3) eGFR at the time of diagnosis (G0 >15, G1 <15). A weighted assignment of points to each parameter was as follows: N1 [4], N2 [6], T1 [2], G1[3], and the resulting aggregate risk score used to classify predicted ESRD risk was low (0), intermediate (2 to 7), or high (8 to 11 points).

Results

In the cohort of 148 patients, median age was 63 years and mean eGFR at diagnosis was 27.7. Seventy-six were MPO, 57 were PR3 positive and 15 were ANCA negative. With regards to risk stratification, 34 were in low risk category, 59 in the medium risk category and 26 patients in the high-risk category. Overall, 23 patients (19.3%) progressed to ESKD with 2 (5.9%), 11 (18.6%), 10 (38.5%) in low, medium and high risk groups, respectively. A Kaplan-Meier survival curve (Figure1) demonstrates worsening of renal survival across the risk groups (p=0.0035).

Conclusion

This AAV renal risk score was able to reliably predict risk for progression to ESKD. A further analysis revalidating cut-offs and risk score points would likely refine the score improving its prediction accuracy.