Objectives: This study investigated whether the earliest total Vasculitis Damage Index (VDI) score could significantly predict all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Methods: This study included AAV patients who were first diagnosed at this hospital from 2001 to 2022. The earliest total VDI score was defined as the first VID assessed more than 3 months after AAV diagnosis in 93.5% of patients or after the first AAV presentation in 6.5% of patients. The optimal cut-off of the earliest total VDI score for all-cause mortality was obtained using the receiver operating characteristic curve.
Results: The median age and earliest VDI score were 60.0 years (35.5% men), and 3.0. The most common damaged system in the earliest VDI was the pulmonary (55.3%) system. Among the AAV patients, 39 (13.3%) died. When the optimal cut-off of the earliest total VDI score for all-cause mortality was set at 3.0 (sensitivity 64.1%, specificity 75.2%), AAV patients with the earliest total VDI score ≥3.0 exhibited a significantly higher risk for all-cause mortality than those without (relative risk 6.090). AAV patients with the earliest total VDI score ≥3.0 exhibited a significantly lower cumulative patients' survival rate than those without. In the multivariable Cox hazards model analyses, not only the earliest total VDI score but also the earliest total VDI score ≥3.0 were independently associated with all-cause mortality.
Conclusions: This study was the first to demonstrate that the earliest total VDI score could predict all-cause mortality during follow-up in AAV patients.