Abstract: SA-PO694
Validation of the Renal Risk Score for ANCA-Associated Glomerulonephritis
Session Information
- Pathology and Lab Medicine: Clinical
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1602 Pathology and Lab Medicine: Clinical
Authors
- Wester Trejo, Maria, Leiden University Medical Center, Leiden, Netherlands
- Van Daalen, Emma, Leiden University Medical Center, Leiden, Netherlands
- Ferrario, Franco, San Gerardo Hospital, Monza, Italy
- Joh, Kensuke, The Jikei University School of Medicine, Tokyo, Japan
- Ogawa, Yayoi, Hokkaido Renal Pathology Center, Sapporo, Japan
- Wilhelmus, Suzanne, Pathan B.V., Rotterdam, Netherlands
- Ball, Miriam Jane, Ipswich Hospital NHS Trust, Ipswich, United Kingdom
- Honsova, Eva, IKEM, Prague, Czechia
- Hruskova, Zdenka, Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czechia
- Kain, Renate, Medical University of Vienna, Wien, Austria
- Kronbichler, Andreas, Medical University Innsbruck, Innsbruck, Austria
- Puéchal, Xavier, National Referral Centre for Rare Systemic Autoimmune Diseases, Paris, France
- Salvatore, Steven, Weill Cornell Medical College, New York, New York, United States
- Szpirt, Wladimir M., Rigshospitalet P2132, Copenhagen, Denmark
- Tesar, Vladimir, General University Hospital, Prague, Czechia
- Wolterbeek, Ron, Leiden University Medical Center, Leiden, Netherlands
- Bos, Willem Jan W., St. Antonius Hospital, Nieuwegein, Netherlands
- Bruijn, Jan A., Leiden University Medical Center, Leiden, Netherlands
- Bajema, Ingeborg M., Leiden University Medical Center, Leiden, Netherlands
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 parameter was assigned points, resulting in a low, intermediate or high risk to develop end-stage renal disease.
Results
At 36 months follow-up, renal survival in our cohort was substantially higher compared to the Brix et al. cohort (table 1). Although renal survival differed significantly 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%). Only one patient from five with the maximum risk score of 11 developed ESRD in our cohort versus all patients with a maximum score in the German cohort.
Conclusion
In this international 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.
Table 1. Renal survival at 36 months in our cohort and the cohort of Brix et al.
| The present cohort | The training cohort by Brix et al. | |||
| Risk group | N of patients | Renal survival 36 months | N of patiens | Renal survival 36 months |
| Low (0 points) | 6 | 100% | 30 | 100% |
| Medium (2-7 points) | 90 | 96% | 64 | 84% |
| High (8-11 points) | 47 | 77% | 21 | 32% |