Background: Recently, Brix et al. proposed a clinicopathologic score to predict renal outcome in ANCA- associated glomerulonephritis (AAGN). This score was based on data from 205 German patients. We here show results of a validation study of this risk score in an international cohort of patients.

Methods: This study included 143 adult patients with AAGN from centers in the U.S., Europe, and Asia. Percentage of normal glomeruli (>25%, 10-25% or < 10%), percentage tubular atrophy and interstitial fibrosis (≤25% or > 25%), and estimated glomerular filtration rate at the time of diagnosis (>15 or ≤ 15) were determined for each patient. Following the risk score, each parameters was assigned points, resulting in a low, intermediate or high risk to develop end-stage renal disease.

Results: At 36 months of follow-up, renal survival in our cohort was substantially higher compared to the cohort of Brix et al. (table 1). Although renal survival was significantly different between the three risk groups (P < 0.001), the clinical relevance between low and medium risk is doubtful, being 100% and 96%, respectively. In the high risk group, there was a substantial difference in the renal survival of our cohort (77%) in comparison to the reported renal survival from the Brix study (32%).

Conclusion: In this cohort, we validated the renal risk score proposed by Brix et al. and demonstrated significantly different renal survival between the three risk groups. The renal survival in our cohort was much higher than in the cohort from Brix. Their relatively poor renal survival might result from the German practice of early dialysis initiation which questions the applicability of the renal risk score to other populations.

Disclosures: None

Abstract 118 Table 1:

Renal survival at 36 months in our cohort and the cohort of Brix et al.

The present cohortThe training cohort by Brix et al.
Risk groupN of patientsRenal survival 36 monthsN of patientsRenal survival 36 months
Low (0 points)6100%30100%
Medium (2-7 points)9096%6484%
High (8-11 points)4777%2132%
The present cohortThe training cohort by Brix et al.
Risk groupN of patientsRenal survival 36 monthsN of patientsRenal survival 36 months
Low (0 points)6100%30100%
Medium (2-7 points)9096%6484%
High (8-11 points)4777%2132%
Abstract 118 Table 1:

Renal survival at 36 months in our cohort and the cohort of Brix et al.

The present cohortThe training cohort by Brix et al.
Risk groupN of patientsRenal survival 36 monthsN of patientsRenal survival 36 months
Low (0 points)6100%30100%
Medium (2-7 points)9096%6484%
High (8-11 points)4777%2132%
The present cohortThe training cohort by Brix et al.
Risk groupN of patientsRenal survival 36 monthsN of patientsRenal survival 36 months
Low (0 points)6100%30100%
Medium (2-7 points)9096%6484%
High (8-11 points)4777%2132%

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