EULAR recommendations for neuropsychiatric systemic lupus erythematosus vs usual care: results from two European centres

Cristina Pamfil; Antonis Fanouriakis; Laura Damian; Mirela Rinzis; Prodromos Sidiropoulos; Georgios Tsivgoulis; Simona Rednic; George Bertsias; Dimitrios T Boumpas

doi:10.1093/rheumatology/keu482

EULAR recommendations for neuropsychiatric systemic lupus erythematosus vs usual care: results from two European centres

Rheumatology (Oxford). 2015 Jul;54(7):1270-8. doi: 10.1093/rheumatology/keu482. Epub 2015 Jan 30.

Authors

Cristina Pamfil¹, Antonis Fanouriakis¹, Laura Damian¹, Mirela Rinzis¹, Prodromos Sidiropoulos¹, Georgios Tsivgoulis¹, Simona Rednic¹, George Bertsias², Dimitrios T Boumpas³

Affiliations

¹ Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece.
² Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece.
³ Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece Department of Rheumatology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece, Department of Rheumatology, Emergency Clinical County Hospital, Cluj-Napoca, Romania, 2nd Department of Neurology, Attikon University Hospital, Medical School, University of Athens, Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Biomedical Research Foundation of the Academy of Athens and 4th Department of Internal Medicine, Attikon University Hospital, Medical School, University of Athens, Greece

PMID: 25638807
DOI: 10.1093/rheumatology/keu482

Abstract

Objective: To compare the European League Against Rheumatism (EULAR) recommendations for the management of NPSLE with usual care in two tertiary centres and to detect potential pitfalls in their use for diagnosis and treatment.

Methods: A chart-based review of NPSLE manifestations was conducted in two European centres. Diagnostic and treatment decisions were compared against the EULAR recommendations for general NPSLE and specific manifestations.

Results: We studied a total of 94 patients who experienced 123 lupus-related neuropsychiatric events over 10 years. In 80% of the events, at least one EULAR-defined risk factor (previous NPSLE, generalized disease activity or aPL positivity) was present. Overall, there was good concordance between clinical care and recommendations for diagnosis and treatment (68.7% and 62.7% of events, respectively). Brain MRI was performed in the absence of a clear EULAR recommendation in 42.9% of events; therein, it was more frequently normal compared with imaging performed according to the recommendations (52.4% vs 18.5%, P = 0.008), and it did not influence management. Among patients reporting cognitive dysfunction, only 27.8% underwent the recommended neuropsychological assessment. In line with the recommendations, immunosuppressants were more frequently given in events suggestive of an inflammatory process (80.5% vs 47.6% in non-inflammatory events, P < 0.001). Notably, 52% of cerebrovascular events were managed with combined immunosuppressive/antithrombotic therapy due to either coexisting generalized lupus activity or recurrence despite prior antithrombotic treatment.

Conclusion: Despite good concordance between EULAR recommendations for NPSLE and usual clinical practice, we identified a number of issues (such as overutilization of brain MRI, suboptimal evaluation of cognitive dysfunction, and frequent use of immunosuppressives in cerebrovascular disease) that need to be investigated further.

Keywords: EULAR recommendations; MRI; immunosuppressive therapy; neuropsychiatric SLE.

Publication types

Comparative Study
Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Brain / pathology
Disease Management*
Europe
Female
Fibrinolytic Agents / therapeutic use
Humans
Immunosuppressive Agents / therapeutic use
Lupus Vasculitis, Central Nervous System / diagnosis*
Lupus Vasculitis, Central Nervous System / therapy*
Magnetic Resonance Imaging
Male
Middle Aged
Neuropsychological Tests
Practice Guidelines as Topic*
Retrospective Studies
Tertiary Care Centers*
Treatment Outcome

Substances

Fibrinolytic Agents
Immunosuppressive Agents