Nephron · 2026
Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.
СтатьяСравнительное исследованиеНазвание и аннотация: Перевод в очереди
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Yıldırım Saliha, Öğüt Betül, Yaşar Emre, Tomar Veysel Baran, Şahin Hatice, Gök Oğuz Ebru, Gönül İpek Işık, Ayli Mehmet Deniz, Güz Galip
Аннотация
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.
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.