Article Text

Download PDFPDF
Extended report
Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus
  1. J G Hanly1,
  2. M B Urowitz2,
  3. L Su3,
  4. S C Bae4,
  5. C Gordon5,
  6. D J Wallace6,
  7. A Clarke7,
  8. S Bernatsky8,
  9. D Isenberg9,
  10. A Rahman9,
  11. G S Alarcón10,
  12. D D Gladman2,
  13. P R Fortin2,
  14. J Sanchez-Guerrero11,
  15. J Romero-Diaz11,
  16. J T Merrill12,
  17. E Ginzler13,
  18. I N Bruce14,
  19. K Steinsson15,
  20. M Khamashta16,
  21. M Petri17,
  22. S Manzi18,
  23. M A Dooley19,
  24. R Ramsey-Goldman20,
  25. R Van Vollenhoven21,
  26. O Nived22,
  27. G Sturfelt22,
  28. C Aranow23,
  29. K Kalunian24,
  30. M Ramos-Casals25,
  31. A Zoma26,
  32. J Douglas1,
  33. K Thompson1,
  34. V Farewell3
  1. 1Division of Rheumatology, Department of Medicine and Department of Pathology, Queen Elizabeth II Health Sciences Centre and Dalhousie University, Halifax, Nova Scotia, Canada
  2. 2Centre for Prognosis Studies in the Rheumatic Diseases, Toronto Western Hospital and University of Toronto, Ontario, Canada
  3. 3MRC Biostatistics Unit, Institute of Public Health, University Forvie Site, Cambridge, UK
  4. 4Division of Rheumatology, The Hospital for Rheumatic Diseases and Hanyang University Medical Centre, Seoul, Korea
  5. 5Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
  6. 6Cedars-Sinai/David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  7. 7Divisions of Clinical Immunology/Allergy and Clinical Epidemiology, Montreal General Hospital, McGill University Health Centre, Montreal, Quebec, Canada
  8. 8Divisions of Rheumatology and Clinical Epidemiology, Montreal General Hospital, McGill University Health Centre, Montreal, Quebec, Canada
  9. 9Centre for Rheumatology Research, University College, London, UK
  10. 10Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA
  11. 11Instituto Nacional de Ciencias Medicas y Nutrición, Mexico City, Mexico
  12. 12Department of Clinical Pharmacology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA
  13. 13Department of Medicine, SUNY Downstate Medical Center, Brooklyn, New York, USA
  14. 14The Kellgren Centre for Rheumatology and ARC Epidemiology Unit School of Translational Medicine, The University of Manchester, Manchester, UK
  15. 15Centre for Rheumatology Research, Landspitali University hospital, Reykjavik, Iceland
  16. 16Lupus Research Unit, The Rayne Institute, St Thomas' Hospital, King's College London School of Medicine, London, UK
  17. 17Department of Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA
  18. 18Division of Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  19. 19University of North Carolina, Chapel Hill, North Carolina, USA
  20. 20Northwestern University and Feinberg School of Medicine, Chicago, Illinois, USA
  21. 21Department of Rheumatology, Karolinska Institute, Stockholm, Sweden
  22. 22Department of Rheumatology, University Hospital Lund, Lund, Sweden
  23. 23Columbia University Medical Center, New York, USA
  24. 24UCSD School of Medicine, La Jolla, California, USA
  25. 25Servicio Enfermedades Autoinmunes Hospital Clínico y Provincial, Barcelona, Spain
  26. 26Lanarkshire Centre for Rheumatology, Hairmyres Hospital, East Kilbride, Scotland, UK
  1. Correspondence to Dr John G Hanly, QEII Health Sciences Centre and Dalhousie University, 1341 Summer Street, Halifax, NS B3H 4K4, Canada; john.hanly{at}


Objectives To determine the frequency, accrual, attribution and outcome of neuropsychiatric (NP) events and impact on quality of life over 3 years in a large inception cohort of patients with systemic lupus erythematosus (SLE).

Methods The study was conducted by the Systemic Lupus International Collaborating Clinics. Patients were enrolled within 15 months of SLE diagnosis. NP events were identified using the American College of Rheumatology case definitions, and decision rules were derived to determine the proportion of NP disease attributable to SLE. The outcome of NP events was recorded and patient-perceived impact determined by the SF-36.

Results 1206 patients (89.6% female) with a mean (±SD) age of 34.5±13.2 years were included in the study. The mean disease duration at enrolment was 5.4±4.2 months. Over a mean follow-up of 1.9±1.2 years, 486/1206 (40.3%) patients had ≥1 NP events, which were attributed to SLE in 13.0–23.6% of patients using two a priori decision rules. The frequency of individual NP events varied from 47.1% (headache) to 0% (myasthenia gravis). The outcome was significantly better for those NP events attributed to SLE, especially if they occurred within 1.5 years of the diagnosis of SLE. Patients with NP events, regardless of attribution, had significantly lower summary scores for both mental and physical health over the study.

Conclusions NP events in patients with SLE are of variable frequency, most commonly present early in the disease course and adversely impact patients' quality of life over time. Events attributed to non-SLE causes are more common than those due to SLE, although the latter have a more favourable outcome.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Funding JGH (Canadian Institutes of Health Research grant MOP-57752, Capital Health Research Fund); MBU (Canadian Institutes of Health Research grant MOP-49529, Lupus Foundation of Ontario, Ontario Lupus Association, Lupus UK, Lupus Foundation of America, Lupus Alliance Western New York, Conn Smythe Foundation, Tolfo Family (Toronto); LS (MRC (UK) grant U.1052.00.009); SCB (Korea Healthcare technology R & D project, Ministry for Health, Welfare and Family Affairs, Republic of Korea (A080588)); CG (Lupus UK, Arthritis Research Campaign, Wellcome Trust Clinical Research Facility in Birmingham, UK); AC (Fonds de la recherche en santé de Quebec National Scholar, Singer Family Fund for Lupus Research); SB (Canadian Institutes of Health Research Junior Investigator Award; Fonds de la recherche en santé du Québéc Jeune Chercheure; Canadian Arthritis Network Scholar Award; McGill University Health Centre Research Institute); GSA (University of Alabama at Birmingham, grant P60AR48095); DDG (Canadian Institutes of Health Research); PRF (distinguished senior research investigator of the Arthritis Society and Arthritis Centre of Excellence); MP (Hopkins Lupus Cohort grant AR 43727, Johns Hopkins University General Clinical Research Center grant MO1 RR00052); SM(National Institutes of Health research grants R01 AR46588, K24 AR002213 and M01 RR000056); RR-G (National Institutes of Health research grants M01-RR00048; K24 AR02318; P60 AR 48098); ON (Swedish Medical Research council grant 13489); GS (Swedish Medical Research council grant 13489); VF (MRC(UK) grant U.1052.00.009).

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the Capital Health Research Ethics Board, Halifax, Nova Scotia, Canada and by the institutional research ethics boards of participating centres in accordance with the Declaration of Helsinki's guidelines for research in humans.

  • Provenance and peer review Not commissioned; externally peer reviewed.