Background and aims To determine the frequency, associations and outcomes of cerebrovascular events (CerVEs) in a multi-ethnic/racial, prospective, inception cohort of SLE patients.

Methods Patients were assessed annually for 19 neuropsychiatric events including 5 types of CerVEs: (i) Stroke; (ii) Transient ischemia; (iii) Chronic multifocal ischemia; (iv) Subarachnoid and intracranial haemorrhage; (v) Sinus thrombosis. Global disease activity (SLEDAI-2K), SLICC/ACR damage index (SDI) and SF-36 subscale, mental (MCS) and physical (PCS) component summary scores were collected. Time to event, linear and logistic regressions and multi-state models were used as appropriate.

Results Of 1826 SLE patients, 88.8% were female, 48.8% Caucasian, mean±SD age 35.1±13.3 years, disease duration 5.6±4.2 months and follow-up 6.6±4.1 years. CerVEs were the fourth most frequent NP event: 82/1,826 (4.5%) patients had 109 events and 103/109 (94.5%) were attributed to SLE. The predominant events were stroke [60/109 (55.0%)] and transient ischemia [28/109 (25.7%)]. CerVEs were associated with NP events attributed to SLE (HR (95% CI): (3.16; 1.73–5.75), non-SLE (2.60; 1.49–4.51) (p<0.001), African ancestry at US SLICC sites (2.04; 1.01–4.13) (p=0.047) and organ damage (p=0.041). Lupus anticoagulant increased the risk of first CerVE (1.77; 0.99–3.16). Physician assessment indicated resolution or improvement in the majority but patients reported a sustained reduction in SF-36 summary and subscale scores following CerVEs (p<0.0001).

Conclusions CerVEs, the fourth most frequent NP event in SLE, are usually attributable to lupus early in the disease course. In contrast to good physician reported outcomes, patients report a sustained reduction in health related quality of life following CerVEs.