Abstract
BackgroundThe 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.MethodsWe 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.ResultsThe 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.ConclusionsAssessing 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.
Highlights
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
Baseline comorbidity score was assessed by determining conditions that had been present before the AAV, namely history of myocardial infarction, congestive heart failure, peripheral vascular disease, cerebrovascular disease, chronic pulmonary disease, peptic ulcer disease, liver disease, diabetes or malignancy
The cumulative probability of survival was 0.441 (95%Confidence interval (CI) 0.231–0.633) versus 0.233 (95%CI 0.126–0.359) (p = 0.028) in patients with a Birmingham Vasculitis Activity Score (BVAS) score below versus at or above median, respectively (Fig. 1)
Summary
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. Patients should receive a treatment tailored to the severity of their disease. Other factors, such as age, the extent of organ involvement and baseline comorbidities may influence the outcome [3,4,5,6,7].
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