Objective: Large vessel-vasculitis (LVV) accounts for up to 70% of patients with giant cell arteritis (GCA). Stenotic involvement in GCA-LVV remains largely unknown. The purpose of this study was to assess the long-term outcome and prognosis of GCA with stenotic LVV.
Methods: This was a retrospective multicenter study of 3,149 patients with GCA, including 198 (6.3%) with stenotic LVV. Hierarchical clustering on principal components was performed on baseline arterial localizations and logistic regression assessed factors associated with vascular complications.
Results: Stenotic LVV affected mostly the subclavian artery (63%), the carotid artery (58%), vertebral artery (37%), and axillary artery (33%), followed by the femoral artery (30%) and mesenteric arteries (13%). Stroke (31%) was the main complication, followed by limb ischemia (21%), myocardial infarction (4%), and mesenteric ischemia (2%). Cumulative incidence of vascular complications was 13.1% (95% confidence interval [CI] 8.9-18.3), 17.3% (95% CI 12.3-22.9), and 19.5% (95% CI 14.3-25.4), at 1, 5, and 10 years, respectively. Hierarchical clustering analysis identified three clusters among which cluster 1 (n = 123; 62%) included older patients with more arteritic anterior ischemic optic neuropathy (P = 0.04), vertebral artery stenosis, and a higher mortality rate (P < 0.044). In multivariate analysis, age at diagnosis (hazard ratio [HR] 1.06, 95% CI 1.03-1.10; P = 0.0004) and vertebral involvement (HR 1.87, 95% CI 1.03-3.40; P = 0.039) were significantly associated with higher risk of vascular complication.
Conclusion: Stenotic LVV accounts for less than 10% of GCA and is associated with poor vascular prognosis.
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