Article Text

EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs
  1. G K Bertsias1,
  2. J P A Ioannidis2,
  3. M Aringer3,
  4. E Bollen4,
  5. S Bombardieri5,
  6. I N Bruce6,
  7. R Cervera7,
  8. M Dalakas8,
  9. A Doria9,
  10. J G Hanly10,
  11. T W J Huizinga11,
  12. D Isenberg12,
  13. C Kallenberg13,
  14. J C Piette14,
  15. M Schneider15,
  16. N Scolding16,
  17. J Smolen17,
  18. A Stara18,
  19. I Tassiulas19,
  20. M Tektonidou20,
  21. A Tincani21,
  22. M A van Buchem22,
  23. R van Vollenhoven23,
  24. M Ward24,
  25. C Gordon25,
  26. D T Boumpas1
  1. 1Rheumatology, Clinical Immunology and Allergy, University of Crete, Heraklion, Greece
  2. 2Clinical Trials and Evidence-Based Medicine Unit, Department of Hygiene and Epidemiology, University of Ioannina School of Medicine, Ioannina, Greece
  3. 3Department of Medicine III, Division of Rheumatology, University Medical Center Carl Gustav Carus, Technical University of Dresden, Dresden, Germany
  4. 4Division of Neurology, Leiden University Medical Center, Leiden, The Netherlands
  5. 5Cattedra di Reumatologia, Universita di Pisa, Pisa, Italy
  6. 6Arc Epidemiology Unit, Manchester Academic Health Sciences Centre, The University of Manchester, Manchester, UK
  7. 7Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain
  8. 8Clinical Neurosciences, Neuromuscular Diseases, Imperial College, London, UK
  9. 9Division of Rheumatology, Clinical and Experimental Medicine, University of Padova, Padova, Italy
  10. 10Division of Rheumatology, Department of Medicine and Department of Pathology, Capital Health and Dalhousie University, Halifax, Canada
  11. 11Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
  12. 12Centre for Rheumatology, University College London Hospitals, London, UK
  13. 13Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, Groningen, The Netherlands
  14. 14Service de médecine interne, centre hospitalier universitaire Pitié-Salpêtrière, Université Pierre-et-Marie-Curie, Paris, France
  15. 15Rheumatology, Heinrich-Heine-University, Düsseldorf, Germany
  16. 16Institute of Clinical Neurosciences, Frenchay Hospital, Bristol, UK
  17. 17Department of Rheumatology, Medical University of Vienna, Vienna, Austria
  18. 18Patient representative
  19. 19Hospital for Special Surgery, New York, USA
  20. 20Division of Rheumatology, University of Athens, Athens, Greece
  21. 21Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy
  22. 22Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands
  23. 23Rheumatology Unit, Department of Medicine, Karolinska University, Stockholm, Sweden
  24. 24National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA
  25. 25Centre for Immune Regulation, School of Immunity and Infection, The University of Birmingham, Birmingham, UK
  1. Correspondence to Dr D T Boumpas, Departments of Internal Medicine and Rheumatology, University of Crete School of Medicine, 71 003, 1 Voutes Street, Heraklion, Greece; boumpasd{at}


Objectives To develop recommendations for the diagnosis, prevention and treatment of neuropsychiatric systemic lupus erythematosus (NPSLE) manifestations.

Methods The authors compiled questions on prevalence and risk factors, diagnosis and monitoring, therapy and prognosis of NPSLE. A systematic literature search was performed and evidence was categorised based on sample size and study design.

Results Systemic lupus erythematosus (SLE) patients are at increased risk of several neuropsychiatric manifestations. Common (cumulative incidence >5%) manifestations include cerebrovascular disease (CVD) and seizures; relatively uncommon (1–5%) are severe cognitive dysfunction, major depression, acute confusional state (ACS), peripheral nervous disorders psychosis. Strong risk factors (at least fivefold increased risk) are previous or concurrent severe NPSLE (for cognitive dysfunction, seizures) and antiphospholipid antibodies (for CVD, seizures, chorea). The diagnostic work-up of suspected NPSLE is comparable to that in patients without SLE who present with the same manifestations, and aims to exclude causes unrelated to SLE. Investigations include cerebrospinal fluid analysis (to exclude central nervous system infection), EEG (to diagnose seizure disorder), neuropsychological tests (to assess cognitive dysfunction), nerve conduction studies (for peripheral neuropathy) and MRI (T1/T2, fluid-attenuating inversion recovery, diffusion-weighted imaging, enhanced T1 sequence). Glucocorticoids and immunosuppressive therapy are indicated when NPSLE is thought to reflect an inflammatory process (optic neuritis, transverse myelitis, peripheral neuropathy, refractory seizures, psychosis, ACS) and in the presence of generalised lupus activity. Antiplatelet/anticoagulation therapy is indicated when manifestations are related to antiphospholipid antibodies, particularly thrombotic CVD.

Conclusions Neuropsychiatric manifestations in SLE patients should be first evaluated and treated as in patients without SLE, and secondarily attributed to SLE and treated accordingly.

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  • Funding This study was funded by the European League Against Rheumatism.

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