Neuropsychiatric systemic lupus erythematosus – diagnosis and treatment
Authors:
M. Olejárová 1,2; V. Balajková 1,2; M. Hušáková 1,2; M. Elišák 3
Authors‘ workplace:
Revmatologický ústav Praha
1; Revmatologická klinika 1. LF UK, Praha
2; Neurologická klinika, 2. LF UK a FN Motol, Praha
3
Published in:
Cesk Slov Neurol N 2025; 88(4): 225-235
doi:
https://doi.org/10.48095/cccsnn2025225
Overview
Neuropsychiatric involvement in systemic lupus erythematosus (SLE), known as neuropsychiatric lupus (NPSLE), is one of the most common, variable, and simultaneously least pathophysiologically understood forms of organ involvement in SLE. It represents a rather heterogeneous group of 19 syndromes, which according to the American College of Rheumatology (ACR), can be classified into diffuse neuropsychiatric syndromes (affective disorders, anxiety disorders, cognitive dysfunctions, psychoses, acute confusional states), central nervous system syndromes (headaches, cerebrovascular diseases, epileptic seizures, demyelinating diseases, aseptic meningitis, myelitis, movement disorders), and peripheral nervous system syndromes (mononeuritis simplex/multiplex, cranial neuropathies, plexopathies, polyneuropathies, myasthenia gravis, autonomic disorders, Guillain-Barré syndrome). NPSLE affects up to 80% of SLE patients, including mild forms, and is more frequently manifested at disease onset. Some forms have a severe negative impact on the patient‘s prognosis. The pathogenesis of NPSLE has not yet been fully elucidated, but it is certain that both inflammatory and non-inflammatory (ischemic, thromboembolic) mechanisms contribute in varying proportions. Involvement of the nervous system may also be related to complications from other organ involvement in the context of SLE or its treatment. The diagnosis of NPSLE is based on clinical examination (neurological, psychiatric, and possibly clinical psychological), supplemented by brain MRI and typically also cerebrospinal fluid analysis. Additional examinations are indicated depending on the type of involvement (electrophysiological studies, etc.). The management strategy for NPSLE depends on the type and severity of neuropsychiatric involvement, the predominant pathophysiological mechanism, SLE activity, and the presence of other organ involvement. In inflammatory forms of NPSLE, immunosuppressive therapy is indicated, while ischemic and thromboembolic forms are treated with anticoagulation or antiplatelet therapy. Symptomatic medications are also administered according to the type of involvement (analgesics, anticonvulsants, antidepressants, antipsychotics, etc.). Broader experience with biologic or targeted therapies for NPSLE is currently lacking.
Keywords:
Systemic lupus erythematosus – neuropsychiatric lupus – NPSLE – NPSLE diagnosis – NPSLE therapy
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| Příloha 1. Screeningový dotazník pro neuropsychiatrické postižení při SLE. (vyplňuje pacient) |
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1. Trpíte bolestmi hlavy? 2. Pokud trpíte bolestmi hlavy – kdy tyto bolesti začaly? 3. Zlepší se vám bolest hlavy po požití denní dávky kortikosteroidů? 4. Pozoroval/a jste v posledních 3 měsících bez varovných příznaků náhlou ztrátu síly či citlivosti končetin? 5. Zpozoroval/a jste v posledních 3 měsících náhlou poruchu řeči trvající méně než 24 hodin? 6. Měl/a jste v posledních 3 měsících pocit těžkých končetin nebo jejich slabost? 7. Měl/a jste v posledních 3 měsících pocit nestability nebo ztráty rovnováhy? 8. Pozoroval/a jste na sobě v posledních 3 měsících poruchu čití (např. necitlivost, brnění, píchání…) v některé části vašeho těla? 9. Měla jste v posledních 3 měsících zamlžené, dvojité či rozostřené vidění? 10. Trpíte záchvaty křečí? 11. Pozoroval/a jste na sobě v posledních 3 měsících náhlé, neúčelné, nerytmické, mimovolní pohyby? 12. Měl/a jste v posledních 3 měsících náhle vzniklou bolest hlavy, provázenou světloplachostí (zvýšená citlivost na světlo), ztuhlostí krku, zmateností a horečkou? 13. Měl/a jste v posledních 3 měsících problémy s dlouhodobým soustředěním? 14. Měl/a jste v posledních 3 měsících problémy s plánováním a vykonáváním běžných denních aktivit či s organizací aktivit nových? 15. Pozoroval/a jste v posledních 3 měsících problém s pamatováním či vybavením si obličejů, tras a obrazů? 16. Měl/a jste v posledních 3 měsících poruchu spánku (nespavost s probuzením kolem 2–4. ranní hodiny)? 17. Pociťoval/a jste posledních 3 měsících ztrátu zájmu o běžné denní činnosti? 18. Měl/a jste v posledních 3 měsících deprese? 19. Měl/a jste v posledních 3 měsících sebevražedné myšlenky? 20. Cítil/a jste se v posledních 3 měsících více nervózní a úzkostlivá než obvykle? 21. Pociťoval/a jste v posledních 3 měsících neodůvodněný strach? 22. V posledních 3 měsících, sdělil vám někdo z vašeho okolí, že se chováte neobvykle a nevhodně? 23. Měl/a jste v posledních 3 měsících pocit, že máte více než jednu osobnost? 24. Stalo se vám v posledních 3 měsících, že jste často opakoval/a dokola stejné činnosti? 25. Choval/a jste se v posledních 3 měsících nepředvídatelně impulzivně? 26. Slyšel/a jste v posledních 3 měsících hlasy, které vám dávaly příkazy nebo jste měl/a halucinace? 27. Cítil/a jste se být v posledních 3 měsících pronásledovaný/á? |
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