Prognosis and long term outcome of stenotic large vessel involvement in giant cell arteritis

Abstract

Objectives: Large vessel-vasculitis (LVV) account 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: Retrospective multicenter study of 3,149 patients with GCA, including 198 (6.3%) with stenotic LVV. Hierarchical clustering on principal components (HCPC) 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%), 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% 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 3 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 [hazard ratio (HR): 1.87 (95% CI 1.03-3.40), p=0.039] were significantly associated with higher risk of vascular complication.

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