Abstract

ABSTRACT BACKGROUND AND PURPOSE: Autoimmune rheumatic diseases (AIRD) can cause intracranial artery stenosis (ICAS) and lead to stroke. This study aimed to characterize patients with ICAS associated with AIRD. MATERIALS AND METHODS: Utilizing data from a high-resolution magnetic resonance imaging (HRMRI) database, we retrospectively reviewed AIRD patients with ICAS. Stratification into vasculitis, atherosclerosis, and mixed athero-vasculitis subtypes was based on imaging findings, followed by a comparative analysis of clinical characteristics and outcomes across these subgroups. RESULTS: Among 139 patients (45.1±17.3 years; 64.7% females), 56 (40.3%) were identified with vasculitis, 57 (41.0%) with atherosclerosis, and 26 (18.7%) with mixed athero-vasculitis. The average interval from AIRD-onset to HRMRI was 5 years. Patients with vasculitis presented with a younger age of AIRD-onset (34.5±19.4 years), nearly ten years earlier than other groups (P=0.010), with a higher artery occlusion incidence (44.6% vs. 21.1% and 26.9%, P=0.021). Patients with atherosclerosis showed the highest cardiovascular risk factor prevalence (73.7% vs. 48.2% and 61.5%, P=0.021) but lower intracranial artery wall enhancement instances (63.2% vs. 100% in others, P<0.001). The mixed athero-vasculitis group, predominantly male (69.2% vs. 30.4% and 25.6%, P<0.001), exhibited the most arterial involvement (5 arteries per person vs. 3 and 2, P=0.001). Over an average 21-month follow-up, 23 (17.0%) patients experienced stroke events, and 8 (5.9%) died, with the mixed athero-vasculitis group facing the highest risk of stroke events (32.0%) and the highest mortality (12.0%). CONCLUSIONS: Intracranial arteries are injured and lead to heterogeneous disease courses when exposed to AIRD and cardiovascular risk factors. While atherosclerosis acceleration is common, vasculitis may further contribute to early-developed occlusion and multiple artery involvement. Varied intracranial arteriopathies may result in different outcomes. ABBREVIATIONS: ICAS = intracranial artery stenosis; AIRD = Autoimmune rheumatic diseases; HRMRI = high-resolution magnetic resonance imaging.