The Initial Presentation of Cerebrovascular Disease Attributed to Lupus Is Most Frequent Early in the Disease Course: Results from an International, Inception Cohort Study

John G. Hanly¹, Qiuju Li², Li Su³, Murray Urowitz^4,5, Juanita Romero-Diaz⁶, C. Gordon⁷, Sang-Cheol Bae⁸, Sasha R Bernatsky⁹, Ann Clarke¹⁰, Daniel J Wallace¹¹, Joan T. Merrill¹², David A. Isenberg¹³, Anisur Rahman¹⁴, Ellen M. Ginzler¹⁵, Michelle Petri¹⁶, Ian N. Bruce¹⁷, Mary Anne Dooley¹⁸, Paul Fortin¹⁹, Dafna D. Gladman²⁰, Jorge Sanchez-Guerrero²¹, Kristján Steinsson²², Rosalind Ramsey-Goldman²³, Munther Khamashta²⁴, Cynthia Aranow²⁵, Graciela S. Alarcon²⁶, Barri J. Fessler²⁷, Susan Manzi²⁸, Ola Nived²⁹, Gunnar K. Sturfelt³⁰, Asad Zoma³¹, Ronald F. van Vollenhoven³², Manuel Ramos-Casals³³, Guillermo Ruiz-Irastorza³⁴, S. Sam Lim³⁵, Kenneth C. Kalunian³⁶, Murat Inanc³⁷, Diane L. Kamen³⁸, Christine A. Peschken³⁹, Søren Jacobsen⁴⁰, Anca Askanase⁴¹, Chris Theriault⁴² and Vernon Farewell⁴³, ¹Rheumatology, Division of Rheumatology, Capital Health and Dalhousie University, Halifax, NS, Canada, ²MRC Biostatistics Unit, Institute of Public Health, University Forvie Site, Cambridge, United Kingdom, ³Nova Scotia Rehab Site, Division of Rheumatology, Capital Health and Dalhousie University, Halifax, NS, Canada, ⁴Rheumatology, TWH, Toronto, ON, Canada, ⁵Rheumatology, U of Toronto, Toronto Western Hospital, Toronto, ON, Canada, ⁶Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico city, Mexico, ⁷School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom, ⁸Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea, ⁹Division of Rheumatolog, McGill Unversity Health Cener, Montreal, QC, Canada, ¹⁰Division of Rheumatology, Department of Medicine, University of Calgary, Calgary, AB, Canada, ¹¹Cedars-Sinai Medical Center, West Hollywood, CA, ¹²Clinical Pharmacology, Oklahoma Medical Research Foundation, Oklahoma City, OK, ¹³Rayne Institute, Centre for Rheumatology Research, UCL Division of Medicine, London, United Kingdom, ¹⁴Centre for Rheumatology Research, U College of London, London, United Kingdom, ¹⁵Medicine/Box 42, SUNY-Downstate, Brooklyn, NY, ¹⁶Rheumatology, Johns Hopkins University Hospital, Baltimore, MD, ¹⁷Stopford Building, Arthritis Research UK Centre for Epidemiology, Centre for Musculoskeletal Research, Institute of Inflammation and Repair, Manchester Academic Health Science Centre, The University of Manchester, Manchester, United Kingdom, ¹⁸UNC Kidney Centre, Chapel Hill, NC, ¹⁹Medicine, Division of Rheumatology, Centre Hospitalier Universitaire de Quebec et Universite Laval, Quebec, QC, Canada, ²⁰Department of Medicine, Division of Rheumatology, University of Toronto, Toronto, ON, Canada, ²¹Rheumatology, Toronto Western Hospital, Toronto, ON, Canada, ²²Rheumatology, Univ. Hospital, Reykjavik, Iceland, ²³Northwestern University Feinberg School of Medicine, Chicago, IL, ²⁴Graham Hughes Lupus Research Laboratory, The Rayne Institute, St Thomas' Hospital, London, United Kingdom, ²⁵Feinstein Institute for Medical Research, Manhasset, NY, ²⁶Medicine, University of Alabama at Birmingham, Birmingham, AL, ²⁷UAB, Birmingham, AL, ²⁸Rheumatology, Allegheny Health Network, Pittsburgh, PA, ²⁹Rheumatology, Inst of Clinical sciences, Lund, Sweden, ³⁰Department of Rheumatology, Univ Hospital Lund, Lund, Sweden, ³¹Rheumatology, Hairmyres Hospital, East Kilbride, United Kingdom, ³²Department of Medicine, Unit for Clinical Therapy Research, Inflammatory Diseases (ClinTRID), The Karolinska Institute, Stockholm, Sweden, ³³Autoimmune Diseases, IDIBAPS, Hospital Clinic, Barcelona, Spain, ³⁴Universidad del Pais Vasco, Servicio de Medicina Interna, Hospital de Cruces, Bizkaia, Spain, ³⁵Emory University School of Medicine, Atlanta, GA, ³⁶Division of Rheumatology, Allergy & Immunology, UCSD School of Medicine Center for Innovative Therapy, La Jolla, CA, ³⁷Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey, ³⁸Medicine, Medical University of South Carolina, Charleston, SC, ³⁹Rheumatology, Univ of Manitoba, Winnipeg, MB, Canada, ⁴⁰Rheumatology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark, ⁴¹Columbia University College of Physicians & Surgeons, New York, NY, ⁴²Dept of Medicine, Queen Elizabeth II Health Sciences Centre and Dalhousie University, Halifax, NS, Canada, ⁴³Medicine, Division of Rheumatology, Capital Health and Dalhousie University, Halifax, NS, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: CNS Lupus

Session Information

Date: Tuesday, November 10, 2015

Title: Systemic Lupus Erythematosus - Clinical Aspects and Treatment Poster Session III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Neuropsychiatric (NP) disease in
patients with SLE includes cerebrovascular events. We determined the frequency,
characteristics and attribution in a large, multi-ethnic/racial, inception
cohort of SLE patients.

Methods: A prospective study of new onset SLE
patients was performed by an international network of 32 academic centers in 11
countries. Patients were evaluated at enrollment and annually for up to 14
years. Data were collected at each assessment on demographic and clinical
manifestations, medications, SLE disease activity index-2000 (SLEDAI-2K) and
Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage index
(SDI). Nervous system events were recorded using the ACR case definitions for
19 NP syndromes. These include cerebrovascular events and consisted of: (i)
Stroke; (ii) Transient ischemia; (iii) Chronic multifocal ischemia; (iv)
Subarachnoid and intracranial hemorrhage; (v) Sinus thrombosis. Pre-defined
rules determined the attribution of NP events to SLE and non-SLE causes.

Results: Of 1,826 SLE patients, 88.8% were
female, 48.8% Caucasian with mean±SD age 35.1±13.3
years. At enrollment, mean SLE duration was 5.6±4.2
months, SLEDAI-2K was 5.3±5.4, SDI was0.31±0.74. The mean follow-up was 5.8±3.8
years. Over the study 912 (49.9%) patients
had 1,770 NP events of which 537 (30.3%) were attributed to SLE. Cerebrovascular
events were the fourth most frequent NP event: 69/1,826 (3.8%) patients had 86
events of which 82/86 (95.3%) were attributed to SLE. Eleven (15.9%) patients
had ≥ 2
cerebrovascular events (9
attributed to SLE and 2 to non-SLE factors) and other NP events occurred concurrently
in 42/69 (60.8%) patients. The predominant
cerebrovascular events were stroke [45/86 (52.3%)] and transient ischemia
[23/86 (26.7%)] followed by subarachnoid and intracranial hemorrhage [9/86
(10.5%)], chronic multifocal ischemia [7/86 (8.1%)] and sinus thrombosis [2/86
(2.3%)]. Forty-one of 86 (47.7%) cerebrovascular events were identified at the
enrollment assessment and by the fifth annual assessment 71/86 events (82.6%)
had occurred (Figure).

Conclusion: Cerebrovascular events are the fourth
most frequent NP event in lupus patients and are usually attributable to SLE. Over
80% of cases present in the first 5 years following the diagnosis of SLE.

Disclosure: J. G. Hanly, None; Q. Li, None; L. Su, Bristol-Myers Squibb, 2; M. Urowitz, Bristol-Myers Squibb, 2; J. Romero-Diaz, None; C. Gordon, Bristol-Myers Squibb, 2; S. C. Bae, None; S. R Bernatsky, None; A. Clarke, None; D. J. Wallace, Bristol-Myers Squibb, 2; J. T. Merrill, Bristol-Myers Squibb, 2; D. A. Isenberg, Bristol-Myers Squibb, 2; A. Rahman, Bristol-Myers Squibb, 2; E. M. Ginzler, None; M. Petri, None; I. N. Bruce, Bristol-Myers Squibb, 2; M. A. Dooley, Lilly, 9,Aurinia, 9,GSK, 5,Genentech and Biogen IDEC Inc., 5,EMD Serono, 5,Medimmune, 5,UCB, 5; P. Fortin, None; D. D. Gladman, Abbvie, 2,Amgen, 5,BMS, 5,Celgene, 2,Eli Lilly and Company, 5,Janssen Pharmaceutica Product, L.P., 2,Novartis, 2,Pfizer Inc, 5,UCB, 2; J. Sanchez-Guerrero, None; K. Steinsson, Bristol-Myers Squibb, 2; R. Ramsey-Goldman, None; M. Khamashta, Bristol-Myers Squibb, 2; C. Aranow, None; G. S. Alarcon, None; B. J. Fessler, None; S. Manzi, Bristol-Myers Squibb, 2,Bristol-Myers Squibb, 2; O. Nived, Bristol-Myers Squibb, 2; G. K. Sturfelt, Bristol-Myers Squibb, 2; A. Zoma, Bristol-Myers Squibb, 2; R. F. van Vollenhoven, AbbVie, BMS, GSK, Pfizer, Roche, UCB, 2,AbbVie, Biotest, BMS, Crescendo, GSK, Janssen, Lilly, Merck, Pfizer, Roche, UCB, Vertex, 5; M. Ramos-Casals, None; G. Ruiz-Irastorza, Bristol-Myers Squibb, 2; S. S. Lim, None; K. C. Kalunian, Bristol-Myers Squibb, 2; M. Inanc, None; D. L. Kamen, None; C. A. Peschken, None; S. Jacobsen, Bristol-Myers Squibb, 2; A. Askanase, None; C. Theriault, None; V. Farewell, Bristol-Myers Squibb, 2.

To cite this abstract in AMA style:

Hanly JG, Li Q, Su L, Urowitz M, Romero-Diaz J, Gordon C, Bae SC, R Bernatsky S, Clarke A, Wallace DJ, Merrill JT, Isenberg DA, Rahman A, Ginzler EM, Petri M, Bruce IN, Dooley MA, Fortin P, Gladman DD, Sanchez-Guerrero J, Steinsson K, Ramsey-Goldman R, Khamashta M, Aranow C, Alarcon GS, Fessler BJ, Manzi S, Nived O, Sturfelt GK, Zoma A, van Vollenhoven RF, Ramos-Casals M, Ruiz-Irastorza G, Lim SS, Kalunian KC, Inanc M, Kamen DL, Peschken CA, Jacobsen S, Askanase A, Theriault C, Farewell V. The Initial Presentation of Cerebrovascular Disease Attributed to Lupus Is Most Frequent Early in the Disease Course: Results from an International, Inception Cohort Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/the-initial-presentation-of-cerebrovascular-disease-attributed-to-lupus-is-most-frequent-early-in-the-disease-course-results-from-an-international-inception-cohort-study/. Accessed .

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