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Cerebrovascular Events in Systemic Lupus Erythematosus

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Hanly JG1, Li Q2, Su L2, Urowitz MB3, Gordon C4, Bae SC5, Romero-Diaz J6, Sanchez-Guerrero J3, Bernatsky S7, Clarke AE8, Wallace DJ9, Isenberg DA10, Rahman A10, Merrill JT11, Fortin P12, Gladman DD3, Bruce IN13,14, Petri M15, Ginzler EM16, Dooley MA17, Steinsson K18, Ramsey-Goldman R19, Zoma AA20, Manzi S21, Nived O22, Jonsen A22, Khamashta MA23, Alarcón GS24, Chatham W24, van Vollenhoven RF25, Aranow C26, Mackay M26, Ruiz-Irastorza G27, Ramos-Casals M28, Lim SS29, Inanc M30, Kalunian KC31, Jacobsen S32, Peschken CA33, Kamen DL34, Askanase A35, Theriault C36, Farewell V2. Arthritis Care Res (Hoboken). 2018 Jan 5. doi: 10.1002/acr.23509. [Epub ahead of print]


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


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


Of 1,826 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, 103/109 (94.5%) were attributed to SLE and 44/109 (40.4%) were identified at enrollment. The predominant events were stroke [60/109 (55.0%)] and transient ischemia [28/109 (25.7%)]. CerVEs were associated with other NP events attributed to SLE (HR (95% CI): (3.16; 1.73-5.75) (p<0.001), non-SLE NP (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 stroke and sinus thrombosis [2.23 (1.11, 4.45) p=.024] and TIA [3.01 (1.15, 7.90) p=0.025]. Physician assessment indicated resolution or improvement in the majority but patients reported sustained reduction in SF-36 summary and subscale scores following CerVEs (P<0.0001).


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