ABSTRACT
Objective:To explore the clinical immune manifestations and high risk factors of children with neuropsychiatric systemic lupus erythematosus (NPSLE), so as to find the basis for early clinical differential diagnosis, prevention and treatment of NPSLE.Methods:Ninety-four children with systemic lupus erythematosus (SLE) admitted to Hunan Children′s Hospital from January 1, 2015 to April 30, 2022 were retrospectively selected. Among them, 19 children with NPSLE were in the NPSLE group, and 75 children without NPSLE were in the SLE group. The disease activity, immune system, lymphocyte subsets, inflammatory factors and autoantibodies were compared between the two groups.Results:Among 94 children with SLE, the incidence of NPSLE was 20.21%(19/94). The most common symptoms of 19 children with NPSLE were headache, epileptic seizure and cerebrovascular disease. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 score was (19.89±8.33)points. Most of them had a good prognosis through hormone intervention. Compared with the SLE group, NPSLE group had higher SLEDAI-2000 score, white blood cell count, urine protein, erythrocyte sedimentation rate (ESR), interferon (IFN)-α, positive rate of anti-Sm antibody and anticardiolipin antibody, lower platelet count, interleukin-2 (IL-2), C 3, C 4 level (all P<0.05), while there was no significant difference in positive rate of anti-dsDNA antibody and anti-SSA antibody between the two group (both P>0.05). Conclusions:Compared with SLE children, children with NPSLE are often complicated with many kinds of abnormal immune function, mainly abnormal humoral immune function and many kinds of positive antibody, with increased inflammatory factors.
ABSTRACT
Objective:To improve the ability of identification and differential diagnosis of severe systemic lupus erythematosus (SLE).Methods:A severe SLE patient with lupus myocarditis, neuropsychiatric lupus, thrombotic microangiopathy (TMA) and other multiple system involvement was reported and discussed.Results:A young female patient developed albuminuria 5 months ago, edema of both lower limbs 3 months ago, change of consciousness 1 month ago and two convulsions attack 2 days ago. She experienced life threatening manifestations such as neuropsychiatric lupus, myocardial involvement of lupus, and TMA. During the course, her condition was generally improved after glucocorticoid pulse therapy and plasma exchange.Conclusion:Various complicated clinical manifestations related to SLE need to be recognized earlier and intervened as soon as possible.
ABSTRACT
ABSTRACT Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.
RESUMO Lúpus eritematoso sistêmico (LES) é uma doença autoimune crônica que envolve múltiplos órgãos e sistemas, caracterizada pela produção de auto anticorpos e lesão tecidual. A etiologia do LES é parcialmente conhecida e envolve interação entre fatores genéticos e ambientais. Até 50% dos pacientes com LES apresentam envolvimento neurológico no decorrer da doença. Manifestações neurológicas estão associadas a prejuízo na qualidade de vida e altas taxas de mortalidade e morbidade. Foram identificadas 19 síndromes neuropsiquiátricas em pacientes com LES, divididas entre manifestações do sistema nervoso central e periférico. O objetivo deste artigo é revisar as manifestações neuropsiquiátricas mais importantes. Serão abordadas as características clínicas, os aspectos radiológicos e opções de tratamento dos eventos neuropsiquiátricos.
Subject(s)
Humans , Lupus Vasculitis, Central Nervous System/complications , Seizures/diagnosis , Autoantibodies/metabolism , Syndrome , Magnetic Resonance Imaging , Cerebrovascular Disorders/diagnostic imaging , Lupus Vasculitis, Central Nervous System/immunology , Lupus Vasculitis, Central Nervous System/therapy , Lupus Vasculitis, Central Nervous System/diagnostic imaging , Headache/diagnosis , Myelitis/diagnostic imaging , Neuropsychological TestsABSTRACT
Objective To investigate the metabolite changes in systemic lupus erythematosus (SLE) patients with and without neuropsychiatric symptoms using magnetic resonance spectroscopy (MRS) and explore the associations between image findings and clinical variables. Methods Twenty-two SLE patients with neuropsychiatric symptoms (NPSLE), twenty-one SLE patients without neuropsychiatric symptoms (non-NPSLE) and twenty healthy controls (HCs) underwent routine MRI scan and multivoxel magnetic reson-ance spectroscopy (MVS). The absolute metabolite concentrations were measured bilaterally in the posterior cingulate gyrus (PCG), dorsal thalamus (DT), lentiform nucleus (LN) and posterior paratrigonal white matter (PWM) using LCModel and SAGE software. The relationships between metabolite con-centrations and cognitive function scores were analyzed by Spearman rank correlation. Single-factor Chi-square analysis and t-test were used for analysis. Results ① Compared to control subjects, NPSLE patients had significantly lower N-acetylaspartate (NAA) values in bilateral PCG and DT, with the mean differences of -1.504 [95% confidence interval ( CI) (-2.335, -0.672), P=0.001], -1.460 [95%CI (-2.349, -0.570), P=0.002], -1.259 [95%CI (-1.894, -0.625), P=0.000] and -1.022[95%CI (-1.688, -0.356), P=0.003] for RPCG, LPCG, RDT and LDT, respectively. The concentration of total creatinine were observed to decline in RPCG and RDT, with the mean differences of-1.094 [95%CI (-1.845, -0.342), P=0.003], -0.955 [95%CI (-1.630, -0.280), P=0.006], -1.259 [95%CI (-1.894,-0.625), P=0.006] respectively. Glutamine and glutamate-values decreased significantly in RDT [mean difference=-2.586, 95%CI (-4.139, -1.033), P=0.002]. ② Compared to non-NPSLE patients, NPSLE patients had a lower NAA level in LPCG [mean difference=-1.256, 95%CI (-2.146, -0.367), P=0.006]. Positive correlations between mini-mental state examination scores [RPCG: rs=0.312, P<0.05; LPCG: rs=0.355, P<0.01], Montreal cognitive assessment scores (RPCG: rs=0.362, P<0.01; LPCG: rs=0.285, P<0.05) and NAA values in bilateral PCG were detected. Conclusion Both NPSLE and non-NPSLE patients may have metabolite dysfun-ctions in different brain regions. The cognitive disorder in SLE patients may be interpreted by neuronic damage of PCG.
ABSTRACT
Objective To identify biomarkers in cerebrospinal fluid (CSF) by proteomic technology and develop a diagnostic model for neuropsychiatric lupus (NPSLE).Methods CSF proteomic spectra of 27 patients with NPSLE before and after treatment,and 27 controls including 17 patients with scoliosis,and 10 SLE patients without neuropsychiatric manifestation (non-NPSLE) were generated by matrix assisted laser desorption/ionization time of flight mass spectrometry (MALDI-TOF-MS) combined with weak cationic exchange (WCX) magnetic beads.Data were analyzed with t test,non-parametric Kruskal-Wallis H test or Wilcoxon sign-rank test.A decision tree model for NPSLE classification was built based on the discriminating peaks.In addition,CSF samples of 12 patients with NPSLE,12 patients with lumbar disc herniation and 9 patients with other neurological conditions were employed as blind test group to verify the accuracy of the model.Results Twelve discriminating mass-to-charge (m/z) peaks were identified between NPSLE and controls.The diagnostic decision tree model,built with a panel of m/z peaks 8595,7170,7661,7740 and 5806,recognized NPSLE with the sensitivity and specificity of 92.6％ and 92.6％ based on training group samples,91.7％ and 85.7％ based on blind test group,respectively.Conclusion Potential CSF NPSLE biomarkers are identified by proteomic technology,the novel diagnostic model is sensitive and relatively specific for the diagnosis of NPSLE.
ABSTRACT
Objective To investigate the magnetic resonance imaging (MRI) characteristics of cerebral lesions,laboratory data and prognosis of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) and evaluate the correlation of MRI findings,autoantibodies and prognosis of NPSLE.Methods Thirty-one patients with NPSLE admitted during Jan 1998 to Oct 2011 were enrolled into this study,including 2 males and 29 females.MRI characteristics of cerebral lesions,laboratory data and clinical outcomes were retrospectively analyzed.x2-test and Spearman's correlation were used for statistical analysis.Results Cranial MRI scans on admission were abnormal in 27 (87％) of the 31 patients,among which 17 cases showed diffuse manifestations,9 cases showed focal lesions in brain and 1 case showed brain atrophy.The percentage of cases with focal lesions in the dead group (86％) was significantly higher than that of the survival group (13％) (P＜0.01),while the percentage of diffuse lesion cases was significantly lower in dead group (P＜0.01).Although the mean lesion numbers in the dead group were fewer compared with the survival group,lesion numbers in brainstem (18％) (P＜0.01) or basal ganglia (29％) (P＜0.05) were much more in the dead group.The positive rate of anti-ribosomal P protein antibodies shown in the dead group (43％) was markedly higher than that in the survival group (29％) (P＜0.05).The positive rates of ACL in cases with diffuse lesions and cases with focal lesion were higher compared with cases showing normal MR images (P＜0.01).Furthermore,the positive rate of anticardiolipin antibodies (ACL) in cases with focal lesions (56％) were higher than cases with diffuse lesion (35％) (P＜0.05).There was no correlation between the seven types of autoantibodies and lesion locations such as basal ganglia,subcortical white matter,anterior and posterior horn of lateral ventricle,semiovale center,cerebral cortex,brainstem and cerebellum.Conclusion MRI findings and autoantibodies are correlated in patients with NPSLE and both of them may be used as prognostic markers for NPSLE.Focal lesions,location of brainstem and basal ganglia on cranial MR images,positive anti-ribosomal P protein antibody may predict an unfavorable clinical outcome of NPSLE; however,the correlation between ACL and prognosis could not be determined.
ABSTRACT
Objecfive To observe the changes of regional cerebral blood flow (rCBF) in systemic lupus erythematosus (SLE) patients with neuropsychiatrie disorders and evaluate rCBF SPECT imaging in the detection of neuropsychiatric problems in SLE patients.Methods Twenty neuropsychiatrie SLE patients were enrolled in the study and were performed SPECT rCBF imaging anti CT/MRI scans,respectively.Twenty SLE patients without neuropsychiatrie manifestatiuns (SLE) and 20 healthy volunteers also underwent SPECT rCBF imaging as controls.Semiquantitative analysis was conducted as designed by selecting 3 consecutive crosssections and delineating ROI,which generated HQ and the results were compared between with SLE and controls.Results SPECT rCBF findings were almormal in 20 NPSLE.in which 19 of the 20 with abnormal SPECT findings showed focal uptake defects the other patient showed increased foeal uptake.Four N PSLE patients had ahnormal CT/MRI scans findings.Compared with the methods of rCBF and CT/MRI imaging,the difference was significant (P＜0.01).Nineteen SLE patients without neuropsychiatrie manifestations had normal SPECT findings.the other 1 patient showed focal uptake defect.SPECT findings were all normal in 20 healthy volunteers.Temporal cortex was the most commonly involved region,followed by frontal cortex.Specific values generated from semiquantitative analysis (HQ) of temporal and frontal regions in neruopsychiatrie SLE patients were lower than that in SLE patients and healthy volunteers (P＜0.01 and P＜0.05).Conclusion SPECT rCBF imaging has the potential to be a sensitive tool to detect the neuropsyehiatric disordersin SLE patients,and has important value in helping for early diagnosis and therapy.Hypoperfusion on ECD-SPECT under the territory of the middle cerebral artery (MCA) is the most common observation,and which temporal and frontal regions are the most frequently involved regions in neruopsychiatrie SLE patients.
ABSTRACT
Objective To define the clinical characteristics of NPSLE in Chinese children by analyzing their demographic data,clinical manifestations,laboratory parameters and treatment.Methods Sixtytwo children with SLE who were diagnosed between 1990 and 2006 were retrospectively reviewed.The demographic data,clinical manifestations,laboratory parameters,imaging and treatment were analyzed.The syndromes were compared with those reported in other studies.Results Nineteen (31%) SLE patients with 21 NP events were identified.The most common NP manifestations were cognitive dysfunction (48%),seizure disorder (43%) and headache (29%).The abnormality percentage of SPECT was 80%.86% patients received MP pulses therapy.Most (74%) patients recovered without long-term neurological complications.Conclusi NP symptoms are common in Chinese children with SLE,however,the prese-ntations are different from that reported in other studies.SPECT and MR are sensitive tests for NPSLE.The prognosis of NPSLE in our series is favorable.
ABSTRACT
Objective To investigate the level and clinical significance of laminin (LN) and hyalu-ronic acid (HA) in cerebrospinal fluid (CSF) of patients with neuropsychiatric lupus (NPLE). Methods The level of LN and HA in CSF in 31 cases of NPLE, 36 cases of no-NPLE SLE, 23 cases of no-SLE cerebrovascular disease and 39 normal controls were messured by radioimmunoassay method. Results The level of LN and HA in CSF of NPLE patients was higher than that of the no-NPLE, no-SLE cerebrovascular disease and normal controls (P
ABSTRACT
Objective To establish the bedside screening scale for cognition examination in systemic lupus erythematosus patients.Methods The modified mini-mental status examination(MMMSE)(revised by the Neurology Department of Hua Shan Hospital)was applied to examine the recognition function of SLE pa- tients.The results were compared with those of the traditional mini-mental status examination(MMSE).Results MMSE examination results showed that NPSLE score was lower than that of normal control group(P＜0.01),no significant difference was found between NPSLE patients and SLE control group(P＞0,05),and the completion time was longer than SLE control group and normal control group(P＜0.01);but no significant difference was found between SLE control group and normal control group.The result of MMMSE examination showed that the score of NPSLE group was lower than that of SLE control group and normal control group(P＜0.01),and the completion time was longer than SLE control group and normal control group(P＜0.01);but the score of SLE control group was lower than the normal control group,and its completion time was longer than normal control group,the difference was statistically significant(P＜0.01).Conclusion MMSE is the most widely used dementia scale,but it is not sensitive in demonstrating the impairment of recognition function.The several items we added to the MMMSE can detect recognition impairment more sensitively,and ean be very easily applied,costs less time(within 10 minutes).Therefore,it can be used for SLE bedside screening.
ABSTRACT
Objective To evaluate the intrathecal injection of combining methotrexate (MTX) and dexamethasone (DXM) into lupus patients with central nervous system involvement. Methods Twenty-eight cases of central nervous system involvement with systemic lupus erythematosus (SLE) accepted intrathecal injection of MTX plus DXM. Data including clinical manifestations, cerebrospinal fluid and skull CT or MRI before and after treatment were analyzed. Meanwhile, the mortalities between 28 cases treated with intrathecal injection and 57 cases received normal management without intrathecal injection were compared. Results Intrathecal injection of MTX plus DXM was given to 26 SLE-central nervous system (CNS) patients except 2 cases death. The symptoms and signs were remitted or relieved obviously including the decrease of pressure and protein and elevation of glucose in cerebrospinal fluid. Positive ANA in 6 cases had turned to negative after treatment. Conclusion Without any severe side effects, intrathecal injection with MTX plus DXM yielded satisfactory effects on patients with SLE-CNS, particularly in those with focus manifestations.