Prognosis and Long-Term Outcome of Stenotic Large Vessel Involvement in Giant Cell Arteritis.

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. 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. 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. Stenotic LVV accounts for less than 10% of GCA and is associated with poor vascular prognosis.