Cited by 742Systemic Lupus Erythematosus ResearchVasculitis and related conditionsSystemic Sclerosis and Related Diseases

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

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Bertsias, G K, Ioannidis, J P A, Aringer, M, Bollen, E, Bombardieri, S, Bruce, I N, Cervera, R, Dalakas, M +18 more·2010·Annals of the Rheumatic Diseases

Abstract

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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  • Distinction between inflammatory and embolic/thrombotic/ischaemic processes may not be easy in clinical practice, or the two processes may coexist in the same patient.

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Research works printed but not linked: 3

  1. The American College of Rheumatology nomenclature and case defi nitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42 : 599 -608 .
  2. Ainiala H, Hietaharju A, Loukkola J, et al Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: a population-based evaluation. Arthritis Rheum 2001; 45 : 419 -23 .
  3. Kozora E, West SG, Kotzin BL, et al Magnetic resonance imaging abnormalities and cognitive defi cits in systemic lupus erythematosus patients without overt central nervous system disease. Arthritis Rheum 1998; 41 : 41 -7 .

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Authors
Bertsias, G. K., Ioannidis, J. P. A., Aringer, M., Bollen, E., Bombardieri, S., Bruce, I. N., Cervera, R., Dalakas, M., Doria, A., Hanly, J. G., Huizinga, T. W. J., Isenberg, D., Kallenberg, C., Piette, J. C., Schneider, M., Scolding, N., Smolen, J., Stara, A., Tassiulas, I., Tektonidou, M., Tincani, A., van Buchem, M. A., van Vollenhoven, R., Ward, M., Gordon, C., & Boumpas, D. T.
Affiliations
  • University of Crete (GR)
  • University of Ioannina (GR)
  • University Hospital Carl Gustav Carus (DE)
  • Leiden University (NL)
  • University of Pisa (IT)
  • Manchester Academic Health Science Centre (GB)
  • Hospital Clínic de Barcelona (ES)
  • Imperial College London (GB)
  • University of Padua (IT)
  • Dalhousie University (CA)
  • University of London (GB)
  • University Medical Center Groningen (NL)
  • Heinrich Heine University Düsseldorf (DE)
  • Frenchay Hospital (GB)
  • Medical University of Vienna (AT)
  • Hospital for Special Surgery (US)
  • National and Kapodistrian University of Athens (GR)
  • Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT)
  • National Institutes of Health (US)
  • University of Birmingham (GB)
Corresponding author
Boumpas, D T
Venue
Annals of the Rheumatic Diseases
Publisher
Elsevier BV
Published
2010-12
Volume
69
Issue
12
Pages
2074-2082
ISSN
0003-4967 (ISSN-L)
OpenAlex
W2114638396
Cited by
742
References
88
Citations by year
2026: 39 · 2025: 35 · 2024: 55 · 2023: 39 · 2022: 41 · 2021: 51 · 2020: 48 · 2019: 50 · 2018: 59 · 2017: 54 · 2016: 56 · 2015: 55 · 2014: 53 · 2013: 43 · 2012: 39
FWCI
23.41
Type
journal article
Language
en
Open access
Locations
  • Annals of the Rheumatic Diseases (publishedVersion)
  • PubMed (publishedVersion)
  • University of Groningen research database (University of Groningen / Centre for Information Technology) (publishedVersion)
  • Data Archiving and Networked Services (DANS) (submittedVersion)
  • Leiden Repository (Leiden University) (submittedVersion)
  • CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
  • UCL Discovery (University College London) (submittedVersion)
  • Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
  • HighWire Press Open Archive (submittedVersion)
  • University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
  • Research Explorer (The University of Manchester) (submittedVersion)
  • Padua Research Archive (University of Padova) (submittedVersion)
  • University of Groningen research database (University of Groningen / Centre for Information Technology) (submittedVersion)
Funders
European League Against Rheumatism
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
Keywords
neuropsychiatric lupus, systemic lupus erythematosus, cerebrovascular disease, seizures, cognitive impairment, depression, delirium, peripheral neuropathy, psychosis, antiphospholipid antibodies, anticoagulation, immunosuppressive therapy, glucocorticoids, diagnosis, risk factors, prognosis
MeSH terms
Mental Disorders, Cranial Nerve Diseases, Diagnostic Techniques, Neurological, Humans, Peripheral Nervous System Diseases, Risk Factors, Spinal Cord Diseases, Evidence-Based Medicine, Lupus Vasculitis, Central Nervous System

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