Research Article: Mortality risk stratification in antineutrophil cytoplasmic antibody-associated vasculitis: baseline severity, accumulated damage burden and cardio-renal vulnerability in a Chinese two-center cohort
Abstract:
Mortality in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is influenced by active inflammation, organ-threatening severity, irreversible damage and host vulnerability. We evaluated how BVAS-2003, FFS-2009 and VDI relate to all-cause mortality, and compared their endpoint discrimination with a baseline cardio-renal vulnerability model informed by the DANGER framework.
We retrospectively studied 122 patients with AAV from two tertiary rheumatology centers. BVAS-2003 and FFS-2009 were assessed at diagnosis. VDI was updated during follow-up and was interpreted as accumulated damage burden rather than as a time-fixed baseline predictor. Overall survival was estimated using Kaplan–Meier analysis. Endpoint associations were assessed using a parsimonious multivariable logistic model, and discrimination was compared using receiver operating characteristic curves.
During a median follow-up of 36?months, 36 patients died. Estimated 1-, 3- and 5-year survival rates were 78, 58 and 39%, respectively. Non-survivors had higher BVAS-2003, higher FFS-2009, higher accumulated VDI, lower hemoglobin, higher serum creatinine and more frequent dialysis. In the endpoint multivariable model, FFS-2009 and VDI retained associations with all-cause mortality, whereas BVAS-2003 did not retain an independent association after adjustment. The DANGER-variable model showed numerically highest discrimination (area under the curve 0.78), followed by VDI (0.74), FFS-2009 (0.73) and BVAS-2003 (0.71).
In this severe real-world AAV cohort, mortality stratification was associated with baseline organ-threatening severity, endpoint accumulated damage burden and cardio-renal vulnerability more than with global activity alone. Because VDI was not measured at a uniform landmark, VDI-related findings should be interpreted as endpoint damage-burden associations rather than validated prospective prediction.
Introduction:
Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a potentially life-threatening group of small-vessel vasculitides, including granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Although modern immunosuppression has improved survival, excess mortality persists, especially among patients with renal failure, pulmonary involvement, infection, cardiovascular comorbidity and treatment-related complications ( 1–3 ). Contemporary management…
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