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Abstract: FR-PO591

Predicting Death in Anti-Neutrophil Cytoplasmic-Antibody Vasculitis With Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Fage, Nicolas, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Riou, Jérémie, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Boud'hors, Charlotte, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Desouche, Alice, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Vinatier, Emeline, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Samoreau, Clément, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Wacrenier, Samuel, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Henry, Nicolas, CH Laval, Laval, France
  • Djema, Assia Ilham, Centre Hospitalier de Cholet, Cholet, France
  • Pouteau, Lise-Marie, CH Laval, Laval, France
  • Copin, Marie-Christine, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Piccoli, Giorgina B., Centre Hospitalier du Mans, Le Mans, Pays de la Loire, France
  • Augusto, Jean francois, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
  • Brilland, Benoit, Centre Hospitalier Universitaire d'Angers, Angers, Pays de la Loire, France
Background

Several scores have been developed to predict death in Anti-Neutrophil Cytoplasmic-Antibody vasculitis (AAV). However, the performance of these scores in AAV with glomerulonephritis (AAV-GN) remains uncertain. We aimed to evaluate their prognostic value and developed our own death prediction risk model in a large cohort of AAV-GN.

Methods

This multicentric retrospective study included patients newly diagnosed with ANCA-GN in 4 French nephrology departments (Maine-Anjou Registry) between 2000 and 2021. Scores (Five Factors Score, FFS; Japanese Vasculitis Activity Score, JVAS; multivariable index for AAV, MVIA; Maldini Score) were assessed at diagnosis before any therapeutic intervention. Clinical, biological and histological characteristics at diagnosis were retrieved. A multivariable cox analysis was performed to determine a death prediction model. Performance (time dependent concordance with C-index and AUC, and prediction accuracy with Brier Score) of these scores and of our model were assessed.

Results

Among the 167 patients included, FFS (HR = 2.78 [2.08-3.73]), JVAS (HR = 1.42 [1.25-1.60]), MVIA (HR = 3.06 [1.66-5.64]) and Maldini [HR = 1.23 (1.10-1.37]) scores were significantly associated with death (with performance decreasing in that order) (Figure 1). In multivariable analysis, age, diabetes, need of early kidney replacement therapy or eGFR, and hemoglobin at diagnosis were associated with death. Two new models including these variables were fit (Table 1) and were found with better performance than the existing scores (Figure 1).

Conclusion

ANCA-GN-specific models perform better in predicting death than existing scores.