Neuropsychiatric Systemic Lupus Erythematosus


Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystemic involvement and diverse manifestations. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a complex neurological disorder characterized by neuropsychological dysfunction. NPSLE is associated with increased morbidity and mortality. In 1999, the American College of Rheumatology developed 19 discrete neuropsychiatric syndromes that comprised NPSLE. Nervous system disease in systemic lupus erythematosus is manifested by a wide variety of clinical manifestations. The pathogenesis of NPSLE is due to autoantibodies, neuronal and non neuronal antigens and the generation of proinflammatory cytokines and mediators. Anatomopathological lesions are attributed to in situ thrombosis, edema, hemorrhage, vasculitis, atherosclerosis or atheroembolism. The diagnosis of NPSLE remains largely one of exclusion and is approached by clinical evaluation, and supported when necessary by autoantibody profiles, diagnostic imaging, electrophysiologic studies and objective assessment of cognitive performance. Brain MRI abnormalities in NPSLE might show small punctate focal lesions in white matter being the most common MRI finding, followed by cortical atrophy, ventricular dilation, cerebral edema, diffuse white matter abnormalities, focal atrophy, cerebral infarction, acute leukoencephalopathy and intracranial hemorrhage. The treatment is based on the use of symptomatic therapies, immunosuppressives and non-pharmacologic interventions. This review paper was designed to understand the pathophysiology for better management of NPSLE.

Share and Cite:

Z. Souirti, M. Lahlou, O. Ouali, N. Chtaou, C. Aarab, F. Ghazouani, W. Bono, I. Rammouz, F. Belahsen and O. Messouak, "Neuropsychiatric Systemic Lupus Erythematosus," Open Journal of Rheumatology and Autoimmune Diseases, Vol. 3 No. 2, 2013, pp. 86-91. doi: 10.4236/ojra.2013.32013.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] J. G. Hanly and M. J. Harrison, “Management of Neuropsychiatric Lupus,” Best Practice & Research Clinical Rheumatology, Vol. 19, No. 5, 2005, pp. 799-821. doi:10.1016/j.berh.2005.04.003
[2] T. S. Ramachandran, “Neurologic Manifestations of Systemic Lupus,” 2011.
[3] J. Mikdashi, A. Krumholz and B. Handwerger, “Factors at Diagnosis Predict Subsequent Occurrence of Seizures in Systemic Lupus Erythematosus,” Neurology, Vol. 64, No. 12, 2005, pp. 2102-2107. doi:10.1212/01.WNL.0000165959.98370.D5
[4] M. J. Cuadrado and M. A. Khamashta, “Can Neurologic Manifestation of Hughes (Antiphospholipide Syndrome Be Distinguished from Multiple Sclerosis) Analyse of 27 Patients and Review of Literature,” Medicine, Vol. 79, No. 1, 2000, pp. 57-68. doi:10.1097/00005792-200001000-00006
[5] D. D. Mitsikostas, P. P. Sfikakis and P. J. Goadsby, “A Meta-Analysis for Headache in Systemic Lupus Erythematosus: The Evidence and the Myth,” Brain, Vol. 127, No. 5, 2004, pp. 1200-1209. doi:10.1093/brain/awh146
[6] H. Ainiala, J. Loukkola, J. Peltola, M. Korpela and A. Hietaharju, “The Prevalence of Neuropsychiatric Syndromes in Systemic Lupus Erythematosus,” Neurology, Vol. 57, No. 3, 2001, pp. 496-500. doi:10.1212/WNL.57.3.496
[7] H. Ainiala, A. Hietaharju, J. Loukkola, J. Peltola, M. Korpela and R. Metsanoja, “Validity of the New American College of Rheumatology Criteria for Neuropsychiatric Lupus Syndromes: A Population-Based Evaluation,” Arthritis Rheum, Vol. 45, No. 5, 2001, pp. 419-423. doi:10.1002/1529-0131(200110)45:5<419::AID-ART360>3.0.CO;2-X
[8] R. Cervera, R. A. Asherson, J. Font, M. Tikly, L. Pallares and A. Chamorro, “Chorea in the Antiphospholipid Syndrome. Clinical, Radiologic, and Immunologic Characteristics of 50 Patients from Our Clinics and the Recent Literature,” Medicine, Vol. 76, No. 3, 1997, pp. 203-212. doi:10.1097/00005792-199705000-00006
[9] F. Ahmed, T. Aziz and L. D. Kaufman, “Progressive Multifocal Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus,” The Journal of Rheumatology, Vol. 26, No. 7, 1999, pp. 1609-1612.
[10] L. Barile and C. Lavalle, “Transverse Myelitis in Systemic Lupus Erythematosus—The Effect of IV Pulse Methylprednisolone and Cyclophosphamide,” Journal of Rheumatology, Vol. 19, No. 3, 1992, pp. 370-372.
[11] J. G. Hanly, K. Cassell and J. D. Kiska, “Cognitive Function in Systemic Lupus Erythematosus: Results of a Five-Year Prospective Study,” Arthritis & Rheumatism, Vol. 40, No. 8, 1997, pp. 1542-1543. doi:10.1002/art.1780400825
[12] R. M. Carbotte, S. D. Denburg and J. A. Denburg, “Cog- nitive Dysfunction and Systemic Lupus Erythematosus,” In: R. G. Lahit, Ed., Systemic Lupus Erythematosus, Churchill Livingstone, New York, 1992, pp. 865-881.
[13] J. C. Piette, D. Marra, C. Lafitte and Z. Amoura, “Neurological Manifestations of Systemic Lupus Erythematosus,” In: H. Dechy, B. Wechsler, P. Hausfater, G. Rancurel and J. C. Piette, Eds., Neurological Damage during Systemic Diseases, Flammarion Medicine-Sciences, Paris, 2003, pp. 141-147.
[14] O. Meyer and M. F. Kahn, “Lupus Erythematosus,” In: M. F. Kahn, A. P. Peltier, O. Meyer and J. C. Piette, Eds., Systemic Diseases and Syndromes, Flammarion Medicine-Sciences, Paris, 2000, pp. 131-368.
[15] F. G. Joseph, G. A. Lammie and N. J. Scolding, “CNS Lupus: A Study of 41 Patients,” Neurology, Vol. 69, No. 7, 2007, pp. 644-654. doi:10.1212/01.wnl.0000267320.48939.d0
[16] M. J. Jarek, S. G. West, M. R. Baker and K. M. Rak, “Magnetic Resonance Imaging in Systemic Lupus Erythematosus Patients without a History of Neuropsychiatric Lupus Erythematosus,” Arthritis & Rheumatism, Vol. 37, No. 11, 1994, 1609-1613. doi:10.1002/art.1780371108
[17] D. W. Paty, “Magnetic Resonance Imaging in the Assessment of Disease Activity in Multiple Sclerosis,” Canadian Journal of Neurological Sciences, Vol. 15, No. 3, 1988, pp. 266-272.
[18] C. L. Bell, C. Partington, M. Robbins, F. Graziano, P. Turski and S. Kornguth, “Magnetic Resonance Imaging of Central Nervous System Lesions in Patients with Lupus Erythematosus. Correlation with Clinical Remission and Antineurofilament and Anticardiolipin Antibody Titers,” Arthritis & Rheumatism, Vol. 34, No. 4, 1991, pp. 432-441. doi:10.1002/art.1780340408
[19] G. Sanna, L. Bertolaccini and A. Mathieu, “Central Nervous System Lupus: A Clinical Approach to Therapy,” Lupus, Vol. 12, No. 12, 2003, pp. 935-942.
[20] L. Stojanovich, R. Stojanovich, V. Kostich and E. Dzjolich, “Neuropsychiartic Lupus Response Favourable to Low Dose i.v. Cyclophosphamide and Prednisolone,” Lupus, Vol. 12, No. 1, 2003, pp. 3-7. doi:10.1191/0961203303lu251oa
[21] P. Bartolucci, S. Bréchignac, P. Cohen, V. Le Guern and L. Guillevin, “Adjunctive Plasma to Treat Neuropsychiatric Lupus Exchanges: A Retrospective Study Were 10 Patients,” Lupus, Vol. 16, No. 10, 2007, pp. 817-822. doi:10.1177/0961203307081840
[22] T. Mikiko, S. Kazuyoshi, K. Daisuke, I. Yoshitaka, T. Fujii, N. Shingo, T. Shizuyo, N. Masao, I. Shigeru, A. Taeko, M. Tsuneyo and T. Yoshiya, “Efficacy of Rituximab (Anti-CD20) for Refractory Systemic Lupus Erythematosus Involving the Central Nervous System,” Annals of the Rheumatic Disease, Vol. 66, No. 4, 2007, pp. 470-475.
[23] G. Zandman-Goddard, J. Chapman and Y. Shoenfeld. “Autoantibodies Involved in Neuropsychiatric SLE and Antiphospholipid Syndrome,” Seminars in Arthritis and Rheumatism, Vol. 36, No. 5, 2007, pp. 297-315.
[24] J. A. Mikdashi, J. M. Esdaile, G. S. Alarcón, et al., “Ad Hoc Committee on Lupus Response Criteria: Cognition Sub-Committee,” Lupus, Vol. 16, No. 6, 2007, pp. 418- 425.

Copyright © 2023 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.