Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis
NEPHRON, cilt.150, sa.6, ss.364-372, 2026 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 150 Sayı: 6
- Basım Tarihi: 2026
- Doi Numarası: 10.1159/000550772
- Dergi Adı: NEPHRON
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, EMBASE, MEDLINE, Health Research Premium Collection (ProQuest), Pharma Collection (ProQuest)
- Sayfa Sayıları: ss.364-372
- Ankara Üniversitesi Adresli: Evet
Özet
Introduction: Pauci-immune necrotizing and crescentic glomerulonephritis (PiNCGN) is a leading cause of rapidly progressive kidney failure. Several prognostic tools - Berden classification, Mayo Clinic Chronicity Score (MCCS), Percentage of ANCA Crescents Score (PACS), ANCA Renal Risk Score (ARRS), and the improved ANCA Kidney Risk Score (AKRiS) - have been developed to predict renal outcomes, but data on their performance in anti-neutrophil cytoplasmic antibody (ANCA)-negative patients remain scarce. This study evaluated the prognostic value of these scoring systems in a PiNCGN cohort. Methods: We retrospectively analyzed 100 patients with biopsy-proven PiNCGN. Demographic, laboratory, and histopathological data were collected, and patients were categorized according to all five risk scores. Outcomes included all-cause mortality and end-stage kidney disease (ESKD), defined as initiation of dialysis or kidney transplantation. The Kaplan-Meier survival and log-rank tests were applied to assess prognostic discrimination. Results: Of 100 patients, 86 were ANCA-positive and 14 ANCA-negative. Median age was 58.5 years; 41% were male. Induction therapy consisted of glucocorticoids with cyclophosphamide or rituximab, followed by azathioprine, mycophenolate, or rituximab for maintenance. Over a median follow-up of 12 months, 52 patients died and 21 progressed to ESKD. In ANCA-positive patients, ARRS and AKRiS best predicted ESKD. In ANCA-negative patients, AKRiS additionally predicted both mortality and ESKD. Other scores demonstrated limited utility. Conclusion: ARRS and AKRiS provided the most consistent prognostic stratification in PiNCGN, with AKRiS uniquely retaining value in ANCA-negative patients. These findings highlight the potential clinical utility of composite scoring systems across the spectrum of PiNCGN, although prospective multicenter validation remains warranted.