Simple, readily available clinical indices predict early and late mortality among patients with ANCA-associated vasculitis

BMC Nephrol. 2017 Feb 23;18(1):76. doi: 10.1186/s12882-017-0491-z.


Background: The early identification of patients with ANCA-associated vasculitis (AAV) who are at increased risk for inferior clinical outcome at the time of diagnosis might help to optimize the immunosuppressive therapy. In this study we wanted to determine the predictive value of simple clinical characteristics, which may be applicable for early risk-stratification of patients with AAV.

Methods: We retrospectively analyzed the outcome of 101 consecutive patients with AAV receiving a protocolized immunosuppressive therapy. Baseline Birmingham Vasculitis Activity Score (BVAS) and non-vasculitic comorbidities were computed, then predictors of early (<90 days) and late (>90 days) mortality, infectious death, relapse and end stage kidney disease (ESKD) were evaluated.

Results: The baseline comorbidity score independently predicted early mortality (HR 1.622, CI 1.006-2.614), and showed association with infectious mortality (HR 2.056, CI 1.247-3.392). Patients with BVAS at or above median (=21) had worse early mortality in univariable analysis (HR 3.57, CI 1.039-12.243) (p = 0.031), and had more frequent relapses (p = 0.01) compared to patients with BVAS below median.

Conclusions: Assessing baseline comorbidities, beside clinical indices characterizing the severity and extension of AAV, might help clinicians in risk-stratification of patients. Future prospective studies are needed to investigate whether therapies based on risk-stratification could improve both short term and long term survival.

Keywords: ANCA; BVAS; Comorbidity; Immunosuppression; Outcome; Vasculitis.

MeSH terms

  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / drug therapy
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / mortality*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / physiopathology
  • Comorbidity
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infections / mortality*
  • Kidney Failure, Chronic / epidemiology*
  • Logistic Models
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Proportional Hazards Models
  • Recurrence
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index


  • Immunosuppressive Agents