The prognostic value of two histopathologic classification models of ANCA-associated glomerulonephritis: a prospective study

Abstract

Background Berden Classification and anti-neutrophil cytoplasmic antibody (ANCA) Renal Risk Score are classification models for rating renal histology and predicting outcome in patients with ANCA-associated Vasculitis/Glomerulonephritis (AAV/GN). In the present study we compare their ability to predict renal function outcome in short- and long-term follow up. Methods Patients with an initial diagnosis of AAV/GN based on kidney biopsy were classified according to Berden and Renal Risk Score, started on the same treatment protocol, and were followed prospectively for up to 60 months. Renal function was recorded at 3mo(T3), 6mo(T6) and 60mo(T60), and results were compared to both classification systems. Results Ninety four AAV/GN patients, M/F = 36/58, age = 60.05 (18–82)yrs were included. Based on Berden classification, patients grouped as Focal (n = 24), Crescentic (n = 35), Mixed (n = 21) and Sclerotic (n = 14), had significant differences in estimated glomerular filtration rate (eGFR) only at T3, while the percentage of those requiring hemodialysis differed at T0, T3, T6 but not at T60. According to the Renal Risk Score, patients were classified as Low (n = 8), Medium (n = 47) and High (n = 39) risk, and showed significant differences in both eGFR levels, proportion of hemodialysis, at T0, T3, T6 and end-stage kidney disease (ESKD) at T60. Even patients classified as Mixed (Berden) and as Medium or High risk (Renal Risk Score) had significant improvement from T0 to T6. Relapse could not be predicted by either system. Conclusion Both methods were able to predict short-term renal function outcome and need for hemodialysis, but the Renal Risk Score showed significant superiority in predicting renal function outcome and ESKD after long-term follow up. Graphical Abstract

Will the ANCA Kidney Risk Score Become the Gold Standard for Prognostication in ANCA-Associated GN?

“…Within the competing prognostication frameworks, there have been a dearth of large-scale external and independent validations, especially ones that compare models (exceptions include Kong et al 7 and Christodoulou et al 8 ). Although AAV is a relatively rare disease, it means that there is a wider need for intercenter, international, and intercontinental collaboration as individual centers will likely not have sufficient patient cohorts to have sufficient power.…”