scholarly journals Neuropsichyatric Manifestations of Systemic Lupus Erythematosus: Diagnosis and Treatment Approach

Author(s):  
Alesandra Tomic Lucic

Abstract Neuropsychiatric involvement in systemic lupus erythematosus includes heterogeneous manifestations involving both the central and peripheral nervous system. A major issue in clinical evaluation is the attribution of neuropsychiatric symptoms to systemic lupus erithematosus. Antiphospholipid antibodies, immune complex, microangiopathy, early and accelerated arteriosclerosis are factors that have the main role in pathogenesis of neuropsychiatric manifestations of systemic lupus erithematosus. Th ere are no neurological symptoms specific to systemic lupus erithematosus, but they can also occur very commonly in the general population. Lesions of nervous system can be focal or diff use and may be due to systemic lupus erithematosus itself (primary lesions), but it also may be caused by other diseases or disbalances. Therapy of the neuropsychiatric manifestations depends on the nature of the pathological process (dominant inflammation or thrombosis). If it is result of an inflammatory neurotoxic process and in the presence of an increased activity of systemic lupus erithematosus, therapy includes glycocorticoids independently or in combination with immunosuppressives. Focal neuropsychiatric syndrome with antiphospholipid antibodies positivity should be treated with anticoagulant and/ or antiplatelet therapy. In addition, control of classical cardiovascular risk factors, stop smoking, and treatment with hydroxychloroquine is recommended.

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Mohammad-Amin Khajezadeh ◽  
Gholamreza Zamani ◽  
Bobak Moazzami ◽  
Zahra Nagahi ◽  
Mahdie Mousavi-Torshizi ◽  
...  

Objective. Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by multisystem involvement, including the nervous system. In the present study, we aimed to assess neuropsychiatric manifestations in juvenile-onset systemic lupus erythematosus (JSLE) in Iran. Methods. One hundred and forty-six pediatric onset patients with SLE who had registered in our pediatric rheumatology database were evaluated prospectively and cross sectionally within 2013-2015. Data including sex, age, age at the time of diagnosis, age at the time of study, physical examination, laboratory review, and neuropsychiatric inventory were extracted from this database. Classification of neuropsychiatric JSLE was according to the 1999 American College of Rheumatology (ACR) neuropsychiatric manifestations of SLE case definitions. Result. A total number of 41 patients with neuropsychiatric symptoms were selected. The patients’ average age was 12.2 years. The most common neuropsychiatric symptoms were seizures, migraine, and depression. The mean age at the onset of symptoms was 10.2 ± 3 years. Mean follow-up period was 57±34 (range: 12-120) months. From 41 SLE patients, 18 (43.9) presented symptoms at the time of diagnosis. In thirteen (31.7%) patients, neurological symptoms were developed more than 1 year after SLE diagnosis. Headache was the most common feature (13%), followed by seizure (9.5%) and chorea (3.4%). Other neurological manifestations included cranial nerve involvement (0.7%), loss of consciousness (2.7%), and impaired deep tendon reflex neuropathy (2.5%). The least common neuropsychiatric JSLE manifestation was aseptic meningitis seen in only one patient (0.7%). Conclusion. The presence of headache, mood disorders, psychosis, depression, and other neuropsychological manifestations in a patient with JSLE should prompt investigations into diagnosis of the primary nervous system involvement in order to reduce mortality and morbidity.


Author(s):  
Elias Manca

AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neuropsychiatric symptoms can range from relatively mild manifestations, such as headache, to more severe complications, such as psychosis. The evaluation of the presence of the autoantibodies in the serum of these patients is the most helpful diagnostic tool for the assessment of the disease. The scientific progresses achieved in the last decades helped researchers and physicians to discover some of autoepitopes targeted by the autoantibodies, although the majority of them have not been identified yet. Additionally, the central nervous system is full of epitopes that cannot be found elsewhere in the human body, for this reason, autoantibodies that selectively target these epitopes might be used for the differential diagnosis between patients with and without the neuropsychiatric symptoms. In this review, the most relevant data is reported with regard to mechanisms implicated in the production of autoantibodies and the most important autoantibodies found among patients with systemic lupus erythematosus with and without the neuropsychiatric manifestations.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1491.2-1492
Author(s):  
R. Monahan ◽  
R. Fronczek ◽  
J. Eikenboom ◽  
H. Middelkoop ◽  
L. J. J. Beaart- van de Voorde ◽  
...  

Background:Fatigue is commonly described in chronic illnesses, especially auto-immune disorders such as systemic lupus erythematosus (SLE).Objectives:We aim to study the prevalence of fatigue in SLE patients with NP symptoms and compare fatigue in SLE patients with NP symptoms attributed to major organ involvement due to SLE (NPSLE) with SLE patients with NP symptoms not caused by major nervous system involvement (non-NPSLE).Methods:All patients visiting the tertiary referral center for NPSLE in the LUMC between 2007-2019 with the clinical diagnosis of SLE and age >18 years that signed informed consent were included in this study. Patients underwent a standardized multidisciplinary assessment, including two questionnaires: SF-36 (2007-2019) and multidimensional fatigue index (MFI, 2011-2019). Patients were classified as NPSLE in this study if NP symptoms were attributed to SLE and immunosuppressive or anticoagulant therapy was initiated, otherwise patients were classified as non-NPSLE. The vitality (VT) domain of the SF-36 domain was used to assess fatigue, which generates a score from 0-100, 100 representing the complete absence of fatigue. Patients with a score more than 1 standard deviation (SD) removed from age-related controls of the Dutch general population were classified as fatigued; patients more than 2 SD removed were classified as extremely fatigued1. The MFI was also used, which consists of 5 subdomain scores between 0-20, leading to a total score between 0-100, 100 representing the most extreme fatigue. All scores are presented as mean and standard deviation.Results:373 patients fulfilled the inclusion criteria and SF-36 questionnaires of 328 patients were available (88%). The majority of these patients was female (87%) and 98 were classified as NPSLE (30%). In NPSLE patients, average age was 41 ± 13 years and in non-NPSLE the average age was 45 ± 14 years. The average score of the SF-36 vitality domain was 36.0 ± 20.7 in NPSLE vs 33.9 ± 18.8. in non-NPSLE. Overall, 73.5% of the patients were fatigued and 46.9% extremely fatigued in NPSLE vs 77.8% fatigued and 45.7% extremely fatigued in non-NPSLE.The MFI questionnaire and VAS score were available for 222 patients, of which 65 patients were classified as NPSLE (29.3%). Table 1 depicts the scores of NPSLE and non-NPSLE patients on the MFI subdomains and the VAS score.Table.Patient characteristics at registry entry.NPSLE(N = 65)Non-NPSLE (N = 157)MFI(mean, sd)General Fatigue10.8 (1.8)11.1 (1.5)Physical Fatigue11.4 (2.4)12.3 (1.9)Reduced Activity9.6 (2.9)10.7 (2.2)Reduced Motivation10.7 (2.6)11.1 (1.9)Mental Fatigue9.5 (3.0)9.8 (2.7)Total score51.8 (9.9)54.9 (6.9)SF-36 Vitality (mean, sd)35 (20.7)32.7 (18.2)Conclusion:Nearly half of patients with SLE and NP symptoms are as extremely fatigued as only 2.5% of the general Dutch population. Extreme fatigue is not influenced by major nervous system involvement.References:[1]Aaronsonet al.J Clin Epidemiol. Vol. 51, No. 11, pp. 1055–1068, 1998Disclosure of Interests:Rory Monahan: None declared, Rolf Fronczek: None declared, Jeroen Eikenboom: None declared, Huub Middelkoop: None declared, L.J.J. Beaart- van de Voorde: None declared, Gisela Terwindt: None declared, Nic van der Wee: None declared, Thomas Huizinga Grant/research support from: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Consultant of: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Margreet Kloppenburg: None declared, G.M. Steup-Beekman: None declared


2021 ◽  
Vol 7 (3) ◽  
pp. 28-34
Author(s):  
Yu. Minakova ◽  
M. Silenko ◽  
O. Ivanova

Damage to the nervous system (neurolupus) is one of the most common clinical manifestations of systemic lupus erythematosus (SLE) in childhood, and is also considered as an unfavorable prognostic criterion for the course of this disease. Neurolupus is characterized by a wide range of clinical manifestations in both children and adult patients, which is due in most cases to a common pathogenetic mechanism - the formation of systemic microvasculitis. The non-specificity and variability of neuropsychiatric symptoms, which may appear already at the onset of the disease, significantly complicate the early diagnosis of SLE and necessitate a close acquaintance of the pediatrician with neurolupus polymorphism in children.


Cephalalgia ◽  
1990 ◽  
Vol 10 (1) ◽  
pp. 25-29 ◽  
Author(s):  
Gunnar Bovim ◽  
Størker Jørstad ◽  
Harald Schrader

Systemic lupus erythematosus (SLE) affects the nervous system in 75% of cases (1). A female with several neurological manifestations in the case history presented with severe headache, psychiatric disturbances, and increasing paraparesis. She was found to have bilateral subdural hematomas, and after evacuation her neuropsychiatric symptoms, including headache, disappeared. It is speculated that the reported low incidence of subdural hematomas in SLE may be more apparent than real. On the basis of our case, we recommend repeated neuroradiological investigations to uncover this important, treatable and otherwise potentially fatal cause of headache.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 620-620
Author(s):  
N. Koletsos ◽  
K. Dipla ◽  
A. Triantafyllou ◽  
A. Lazaridis ◽  
N. Papadopoulos ◽  
...  

Background:Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs, including the central nervous system. Subclinical brain lesions have been reported in SLE patients, even without overt neuropsychiatric manifestations (non-NPSLE). Studies using PET/MRI, examining structural or functional brain abnormalities in SLE, have been previously performed, either at rest or during a mental task (1–3). Exercise can be used to identify early alterations in brain oxygenation that might not detectable during resting conditions (4).Objectives:Our study aimed to examine possible differences in cerebral oxygenation during a handgrip exercise test between SLE patients without neuropsychiatric manifestations and age-matched controls.Methods:Fifty-two participants (26 non-NPSLE and 26 controls), following evaluation of handgrip strength, underwent a protocol involving a seated rest (baseline), a 3-min handgrip exercise (at 30% of maximal strength), and a 3-min recovery. Continuous-near-infrared-spectroscopy (NIRS) was used to monitor changes in cerebral-oxygenated hemoglobin (O2Hb), de-oxygenated (HHb) and total-hemoglobin (tHb). Beat-by-beat blood pressure (Finapres) was continuously monitored.Results:There were no differences between the two groups in age, body mass index, blood pressure, and smoking status. Median SLE duration was 7.5 (3.0 – 16.0) years. During exercise, cerebral -O2Hb increased in both groups; however, non-NPSLE exhibited a significantly lower increase in O2Hb vs. controls (average response:1.20±0.89 vs. 2.33±1.61μM, respectively, p<0.005) and lower tHb responses (p<0.05), with no differences in HHb.Conclusion:Our data show, for the first time, that SLE patients even without overt neuropsychiatric manifestations exhibit a blunted increase in cerebral-O2Hb during a submaximal exercise stimulus compared to age-matched controls. Examining brain oxygenation during a simple exercise task may assist in identifying patients with early alterations in cerebral function.References:[1]Mak A, Ren T, Fu EH yun, Cheak AA cia, Ho RCM. A Prospective Functional MRI Study for Executive Function in Patients with Systemic Lupus Erythematosus Without Neuropsychiatric Symptoms. Semin Arthritis Rheum. 2012;41(6):849–58.[2]Kozora E, Brown MS, Filley CM, Zhang L, Miller DE, West SG, et al. Memory impairment associated with neurometabolic abnormalities of the hippocampus in patients with non-neuropsychiatric systemic lupus erythematosus. Lupus. 2011;20(6):598–606.[3]Mackay M, Vo A, Tang CC, Small M, Anderson EW, Ploran EJ, et al. Metabolic and microstructural alterations in the SLE brain correlate with cognitive impairment. JCI Insight. 2019;4(1).[4]Triantafyllou GA, Dipla K, Triantafyllou A, Gkaliagkousi E, Douma S. Measurement and Changes in Cerebral Oxygenation and Blood Flow at Rest and During Exercise in Normotensive and Hypertensive Individuals. Vol. 22, Current Hypertension Reports. Springer; 2020.Disclosure of Interests:None declared


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