High prevalent early damage independently predicts future mortality in an inception cohort of Takayasu arteritis

Objectives To evaluate early damage, its evolution, and prognostic relevance in an inception cohort of Takayasu arteritis (TAK) using the Large Vessel Vasculitis Index of Damage (LVVID). Methods LVVID was scored in an inception cohort of TAK within 3 months of cohort entry, at one year, and the last follow-up at or after one year. Association between initial LVVID scores and demographic characteristics, disease activity, and angiographic subtypes were evaluated. Hazard ratios (HR) for mortality per point/quartile increase in baseline LVVID scores were calculated using Cox proportional hazards regression. Damage evolution from baseline to one year or last follow-up was compared using paired Student’s t test. Pre-treatment predictors of progression were assessed using logistic regression. Results Among 199 patients (141 females, pediatric-onset 54), 192/199 (96.5%) had damage (most often cardiovascular) scored on LVVID at presentation, 112/114 (98.2%) at one year, and 126/130 (96.9%) at last follow-up. Initial LVVID was greater with active disease, Hata’s type V, or Goel’s cluster 1 angiographic subtypes. Higher baseline LVVID was associated with increased mortality [crude HR per point increase 1.49 (95%CI 1.24–1.80) or per quartile increase in LVVID 1.92 (1.21–3.05)] consistent despite adjustment for disease activity or angiographic subtype. Significant progression of LVVID was observed at 1 year (3.49 ± 2.01 vs 3.27 ± 1.86, p< 0.001) or at the last follow-up (3.73 ± 2.18 vs 3.28 ± 1.88 p< 0.001), predicted by active disease at presentation (OR 2.89) and glucocorticoid (OR 3.53) or immunosuppressant use (OR 3.52). Conclusion Most patients with TAK had recordable early damage which predicted future mortality.