Clinical Presentation of Neuropsychiatric Systemic Lupus Erythematosus and Demographic and Radiological Characteristics of Patients

Fatma Şimşek; Mustafa Ceylan

Research Article

Clinical Presentation of Neuropsychiatric Systemic Lupus Erythematosus and Demographic and Radiological Characteristics of Patients

Year 2021, Volume: 2 Issue: 2, 123 - 129, 15.09.2021

Fatma Şimşek Mustafa Ceylan

Abstract

Introduction: Systemic lupus erythematosus (SLE) is a vasculitis that may affect numerous systems such as the kidneys, skin, joints, heart, lungs and nervous system. The purpose of our study is to evaluate patients with SLE in whom central nervous system involvement is monitored.
Material and method: Files of 1028 patients who were followed up with systemic lupus erythematosus (SLE) diagnosis have been reviewed. Demographic, clinical and radiological characteristics were recorded for patients with a final diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) with central involvement.
Results: Among 1028 patients diagnosed with SLE, 1.07% had NPSLE. Mean age was 37+5.3. 90.9% of the patients (n = 10) were female, while 9.1% (n = 1) were male. From a clinical aspect, 45.4% complained from hemiparesis, 27.3% from headache, 18.2% from psychiatric complaints and 9.1% complained from impairment of consciousness. From a radiological aspect, 45.4% (n=5) were consistent with subcortical plaque, 36.4% (n=4) with ischemic stroke, 9.1% (n=1) with cerebral venous thrombosis, and 9.1% (n=1) appeared consistent with posterior reversible encephalopathy syndrome (PRES). Mortality rate was 9.1% (n=1). The central involvement type that caused mortality was ischemia.
Conclusion: Since magnetic resonance imaging (MRI) is not sufficient for showing microvascular involvement in NPSLE patients, it is possible for NPSLE diagnosis to be delayed despite consistent clinical characteristics. In case of clinical suspicion, other imaging methods should be applied apart from MRI. This is because early diagnosis is an important factor that reduces morbidity and mortality.

Keywords

Systemic Lupus Erythematosus;, Central Nervous System;, Magnetic Resonance Imaging

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Project Number

Thanks

References

1. Tamirou F, Arnaud L, Talarico R, et al. Systemic lupus erythematosus: state of the art on clinical practice guidelines. RMD Open 2018; 4(2): e000793.
2. Rees F, Doherty M, Grainge MJ, Lanyon P, Zhang W. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology (Oxford) 2017; 56(11):1945–1961.
3. Zettl UK, Stuve O, Patejdl R. Immune-mediated CNS diseases: a review on nosological classification and clinical features. Autoimmun Rev 2012;11(3): 167-173.
4. Cohen D, Rijnink EC, Nabuurs RJ, et al. Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement. Rheumatology 2016;56(1):77-86.
5. Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults Neurol Clin, 2010;28(1): 61-73.
6. de Amorim LCD, Maia FM, Rodrigues CEM. Stroke in systemic lupus erythematosus and antiphospholipid syndrome: risk factors, clinical manifestations, neuroimaging, and treatment Lupus, 2017;26(5):529-536.
7. Jeltsch-David H, Muller S. Autoimmunity, neuroinflammation, pathogen load: a decisive crosstalk in neuropsychiatric SLE. J Autoimmun 2016; 74:13–26.
8. Abbott NJ, Mendonça LLF, Dolman DEM. The blood-brain barrier in systemic lupus erythematosus. Lupus 2003; 12(12):908–915.
9. Unterman A, Nolte JE, Boaz M, Abady M, Shoenfeld Y, Zandman-Goddard G. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta- analysis. Semin Arthritis Rheum. 2011; 41(1): 1–11.
10. Sciascia S, Bertolaccini ML, Baldovino S, Roccatello D, Khamashta MA, Sanna G. Central nervous system involvement in systemic lupus erythematosus: overview on classification criteria. Autoimmun Rev 2013;12(3):426-429.
11. Gal Y, Twig G, Mozes O, Greenberg G, Hoffmann C, Shoenfeld Y. Central nervous system involvement in systemic lupus erythematosus: an imaging challenge. Isr Med Assoc J 2013;15(7):382-386.
12. Bertsias GK, Ioannidis JPA, Aringer M, et al. 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. Ann Rheum Dis 2010; 69:2074–2082.
13. Petri M. Use of hydroxychloroquine to prevent thrombosis in systemic lupus erythematosus and in antiphospholipid antibody-positive patients. Curr Rheumatol Rep 2011; 13(1):77–80.
14. Hanly JG, Urowitz MB, Su L, et al. Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus. Ann. Rheum. Dis. 2010; 69(3): 529–535.
15. Ho RC, Thiaghu C, Ong H, et al. A meta-analysis of serum and cerebrospinal fluid autoantibodies in neuropsychiatric systemic lupus erythematosus. Autoimmun. Rev. 2016;15(2): 124–138.
16. Mok CC, Lau CS, Wong RW. Neuropsychiatric manifestations and their clinical associations in southern Chinese patients with systemic lupus erythematosus. J. Rheumatol. 2001;28(4): 766–771.
17. Steup-Beekman GM, Zirkzee EJ, Cohen D, et al. Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann. Rheum. Dis. 2013; 72 (2):76–79.
18. Govoni M, Bortoluzzi A, Padovan M, et al. The diagnosis and clinical management of the neuropsychiatric manifestations of lupus. J. Autoimmun. 2016;74: 41–72.
19. Hanly JG, Li Q, Su L, et al. Cerebrovascular events in systemic lupus erythematosus: results from an international inception cohort study. Arthritis Care Res. 2018;70(10): 1478–1487.
20. Wichainun R, Kasitanon N, Wangkaew S, Hongsongkiat S, Sukitawut W, Louthrenoo W. Sensitivity and specificity of ANA and anti-dsDNA in the diagnosis of systemic lupus erythematosus: a comparison using control sera obtained from healthy individuals and patients with multiple medical problems. Asian Pac J Allergy Immunol 2013; 31(4):292–298.
21. ter Borg EJ, Horst G, Hummel EJ, Limburg PC, Kallenberg CG. Measurement of increases in anti-double-stranded DNA antibody levels as a predictor of disease exacerbation in systemic lupus erythematosus. A long-term, prospective study. Arthritis Rheum 1990; 33(5):634–643.
22. Li Wang, Hua Chen, Yao Zhang, et al. "Clinical Characteristics of Cerebral Venous Sinus Thrombosis in Patients with Systemic Lupus Erythematosus: A Single-Centre Experience in China", Journal of Immunology Research, vol. 2015, Article ID 540738, 7 pages, 2015.
23. Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, Part 2: Diagnostic work-up, imaging evaluation, and differential diagnosis. Am J Neuroradiol 2015; 36(9): 1580–1588.
24. Lee JY, Kim YS, Kim HY, Park DW, Bae S, Lee Y. Non-aneurysmal subarachnoid hemorrhage in two patients with systemic lupus erythematosus: Case reports and literature review. Int J Rheum Dis 2018; 21(3): 761–766.
25. Cohen D, Rijning EC, Nabuurs RJ, et al. Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement. Rheumatology (Oxford) 2017; 56(1):77–86.
26. Luyendijk J, Steens SCA, Ouwendijk WJN, et al. Neuropsychiatric systemic lupus erythematosus: lessons learned from magnetic resonance imaging. Arthritis Rheum. 2011;63(3): 722–732.
27. Kürtüncü M, Altunrende B, Akman Demir G. Autoimmune Diseases Associated with Epilepsy. Journal of the Turkish Chapter of International League Against Epilepsy 2016;22(l 1):53–66.
28. Barile- Fabris L, Ariza-Andraca R, Olguin-Ortega LJ, et al. Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann. Rheum. Dis. 2005;64(4): 620–625.
29. Dale RC, Brilot F, Duffy LV, et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology 2014;83(2):142–150.

Year 2021, Volume: 2 Issue: 2, 123 - 129, 15.09.2021

Fatma Şimşek Mustafa Ceylan

Abstract

Project Number

References

1. Tamirou F, Arnaud L, Talarico R, et al. Systemic lupus erythematosus: state of the art on clinical practice guidelines. RMD Open 2018; 4(2): e000793.
2. Rees F, Doherty M, Grainge MJ, Lanyon P, Zhang W. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology (Oxford) 2017; 56(11):1945–1961.
3. Zettl UK, Stuve O, Patejdl R. Immune-mediated CNS diseases: a review on nosological classification and clinical features. Autoimmun Rev 2012;11(3): 167-173.
4. Cohen D, Rijnink EC, Nabuurs RJ, et al. Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement. Rheumatology 2016;56(1):77-86.
5. Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults Neurol Clin, 2010;28(1): 61-73.
6. de Amorim LCD, Maia FM, Rodrigues CEM. Stroke in systemic lupus erythematosus and antiphospholipid syndrome: risk factors, clinical manifestations, neuroimaging, and treatment Lupus, 2017;26(5):529-536.
7. Jeltsch-David H, Muller S. Autoimmunity, neuroinflammation, pathogen load: a decisive crosstalk in neuropsychiatric SLE. J Autoimmun 2016; 74:13–26.
8. Abbott NJ, Mendonça LLF, Dolman DEM. The blood-brain barrier in systemic lupus erythematosus. Lupus 2003; 12(12):908–915.
9. Unterman A, Nolte JE, Boaz M, Abady M, Shoenfeld Y, Zandman-Goddard G. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta- analysis. Semin Arthritis Rheum. 2011; 41(1): 1–11.
10. Sciascia S, Bertolaccini ML, Baldovino S, Roccatello D, Khamashta MA, Sanna G. Central nervous system involvement in systemic lupus erythematosus: overview on classification criteria. Autoimmun Rev 2013;12(3):426-429.
11. Gal Y, Twig G, Mozes O, Greenberg G, Hoffmann C, Shoenfeld Y. Central nervous system involvement in systemic lupus erythematosus: an imaging challenge. Isr Med Assoc J 2013;15(7):382-386.
12. Bertsias GK, Ioannidis JPA, Aringer M, et al. 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. Ann Rheum Dis 2010; 69:2074–2082.
13. Petri M. Use of hydroxychloroquine to prevent thrombosis in systemic lupus erythematosus and in antiphospholipid antibody-positive patients. Curr Rheumatol Rep 2011; 13(1):77–80.
14. Hanly JG, Urowitz MB, Su L, et al. Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus. Ann. Rheum. Dis. 2010; 69(3): 529–535.
15. Ho RC, Thiaghu C, Ong H, et al. A meta-analysis of serum and cerebrospinal fluid autoantibodies in neuropsychiatric systemic lupus erythematosus. Autoimmun. Rev. 2016;15(2): 124–138.
16. Mok CC, Lau CS, Wong RW. Neuropsychiatric manifestations and their clinical associations in southern Chinese patients with systemic lupus erythematosus. J. Rheumatol. 2001;28(4): 766–771.
17. Steup-Beekman GM, Zirkzee EJ, Cohen D, et al. Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann. Rheum. Dis. 2013; 72 (2):76–79.
18. Govoni M, Bortoluzzi A, Padovan M, et al. The diagnosis and clinical management of the neuropsychiatric manifestations of lupus. J. Autoimmun. 2016;74: 41–72.
19. Hanly JG, Li Q, Su L, et al. Cerebrovascular events in systemic lupus erythematosus: results from an international inception cohort study. Arthritis Care Res. 2018;70(10): 1478–1487.
20. Wichainun R, Kasitanon N, Wangkaew S, Hongsongkiat S, Sukitawut W, Louthrenoo W. Sensitivity and specificity of ANA and anti-dsDNA in the diagnosis of systemic lupus erythematosus: a comparison using control sera obtained from healthy individuals and patients with multiple medical problems. Asian Pac J Allergy Immunol 2013; 31(4):292–298.
21. ter Borg EJ, Horst G, Hummel EJ, Limburg PC, Kallenberg CG. Measurement of increases in anti-double-stranded DNA antibody levels as a predictor of disease exacerbation in systemic lupus erythematosus. A long-term, prospective study. Arthritis Rheum 1990; 33(5):634–643.
22. Li Wang, Hua Chen, Yao Zhang, et al. "Clinical Characteristics of Cerebral Venous Sinus Thrombosis in Patients with Systemic Lupus Erythematosus: A Single-Centre Experience in China", Journal of Immunology Research, vol. 2015, Article ID 540738, 7 pages, 2015.
23. Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, Part 2: Diagnostic work-up, imaging evaluation, and differential diagnosis. Am J Neuroradiol 2015; 36(9): 1580–1588.
24. Lee JY, Kim YS, Kim HY, Park DW, Bae S, Lee Y. Non-aneurysmal subarachnoid hemorrhage in two patients with systemic lupus erythematosus: Case reports and literature review. Int J Rheum Dis 2018; 21(3): 761–766.
25. Cohen D, Rijning EC, Nabuurs RJ, et al. Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement. Rheumatology (Oxford) 2017; 56(1):77–86.
26. Luyendijk J, Steens SCA, Ouwendijk WJN, et al. Neuropsychiatric systemic lupus erythematosus: lessons learned from magnetic resonance imaging. Arthritis Rheum. 2011;63(3): 722–732.
27. Kürtüncü M, Altunrende B, Akman Demir G. Autoimmune Diseases Associated with Epilepsy. Journal of the Turkish Chapter of International League Against Epilepsy 2016;22(l 1):53–66.
28. Barile- Fabris L, Ariza-Andraca R, Olguin-Ortega LJ, et al. Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann. Rheum. Dis. 2005;64(4): 620–625.
29. Dale RC, Brilot F, Duffy LV, et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology 2014;83(2):142–150.

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Details

Primary Language	English
Subjects	Internal Diseases
Journal Section	Research Articles
Authors	Fatma Şimşek 0000-0003-1662-5534 Mustafa Ceylan 0000-0002-9916-0156
Project Number	NA
Publication Date	September 15, 2021
Submission Date	December 31, 2020
Published in Issue	Year 2021 Volume: 2 Issue: 2

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EndNote	Şimşek F, Ceylan M (September 1, 2021) Clinical Presentation of Neuropsychiatric Systemic Lupus Erythematosus and Demographic and Radiological Characteristics of Patients. New Trends in Medicine Sciences 2 2 123–129.

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