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Several anti-neural antibodies are associated with neuropsychiatric systemic lupus erythematosus (NPSLE) including anti-neuronal antibodies and anti-glial cell antibodies. The anti-neuronal antibodies has two types: anti-neuronal surface protein antibodies represented by anti-N-methyl-D-aspartate receptor (NMDAR) antibodies, and anti-neuronal intracellular protein antibodies. In this paper, we review and classify the anti-neural antibodies related to NPSLE.
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Objective:To investigate the incidence and clinical characteristics of cranial imaging abnormalities in children with systemic lupus erythematosus (SLE) at the initial diagnosis.Methods:The clinical data of 74 children with SLE admitted to the Department of Rheumatology in Children′s Hospital Affiliated to Xi′an Jiaotong University for the initial diagnosis from January 2012 to May 2019 were subject to retrospective analysis.They were divided into the cranial imaging abnormality group and the cranial imaging non-abnormality group according to the imaging.A description and statistical analysis were carried out for both groups with respect to the course before initial diagnosis, gender, rash, arthralgia, hair loss, pulmonary lesions, white blood cells (WBC), hemoglobin (Hb), platelets (PLT), erythrocyte sedimentation rate (ESR), serum ferritin (FER), serum complement values (C 3 and C 4), anticardiolipin antibody (ACA), alanine aminotransferase (ALT), aspartate transaminase (AST), triglyceride (TG), high-density lipoprotein (HDL), low-density lipoprotein (LDL), and total cholesterol (TC). Results:Seventy-four children with SLE underwent a cranial imaging exa-mination at the initial diagnosis, including 52 cases for magnetic resonance imaging (MRI) and 22 cases for CT.There were 36 abnormal cases (48.6%), including 27 cases (51.9%) in MRI and 9 cases (40.9%) in CT.Among 36 cases of abnormal cranial imaging in children with SLE, MRI abnormalities were mainly demyelinating lesions and sulcus widening (brain atrophy), while CT abnormalities were mainly sulcus widening (brain atrophy). There were 21 cases presenting with neurological symptoms, including 17 cases of headache, 11 cases of dizziness, 3 cases of convulsions, and 1 case of coma.There were no significant differences between both groups in the course before initial diagnosis, gender, rash, arthralgia and hair loss.Among the 36 cases of SLE with cranial imaging abnormalities, 20 cases presented with interstitial pulmonary lesions, of which 4 cases presented with pulmonary hemorrhage; Among 38 cases of SLE without cranial imaging abnormality, 8 cases presented with interstitial pulmonary lesions, which indicated that there were statistical differences between both groups; within terms of the laboratory test items, there were significant differences in PLT between both groups, and there was no significant difference in WBC, Hb, ESR, FER, C 3, C 4, ACA, ALT, AST, TG, HDL, LDL and TC. Conclusions:The cranial imaging abnormalities in children with SLE, especially the earlier occurrence in MRI, may occur before the manifestation of clinical symptoms of the nervous system.They were also associated with other important organ damages, such as abnormal blood system and lung lesions.Early detection may contribute to the short-term prognosis.
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Objective:To investigate the clinical characteristics, prognosis, and risk factors for poor prognosis of neuropsychiatric systemic lupus erythematosus (NPSLE) .Methods:Patients who were diagnosed as NPSLE between January 2009 to January 2019 in Peking University First Hospital were included. Patients with neuro-psychiatric symptoms caused by other reasons such as infection and metabolic disorders were excluded. Patients were retrospectively followed up by telephone or medical records. Continuous variables were compared by student t test or Wilcoxon rank sum test. Quantitative variables were compared by chi-square test. Survival was analyzed by Kaplan-Meier curve. Predictive factors of prognosis was estimated by using Cox regression analysis. Results:One hundred and nine NPSLE patients were included. Thirteen (11.9%) were male and 96 (88.1%) were female with a median age of 33 years old. Central nervous system involvement was predominant (89/109, 81.7%) . The most common types were headache, cerebrovascular disease and epilepsy. Cranial neuropathy was the most common type at the initial onset of systemic lupus erythematosus (SLE) , while cerebrovascular disease was more common when SLE relapsed. Patients who demonstrated NPSLE at the initiation of SLE had shorter survival time than those who got NPSLE when SLE relapsed [ (32±26) months vs (197±79) months, t=2.834, P=0.037]. Among the 105 patients with complete followed up data, the follow up time was 118.0 (1.4, 525.7) months and 53.1 (0.4, 363.0) months from the onset of SLE and NPSLE, respectively. The mortality rate was 14.3% (15/105) . The survival rates of 1-5 years were 96.2%, 94.3%, 91.0%, 89.9% and 88.3%, respectively. The survival time was (180±138) months and (33±32) months, t=3.861 , P<0.01) from the onset of SLE and NPSLE, respectively. The major causes of death were infection, NSPLE and cardiovascular disease. Cerebrovascular disease was the independent risk factor for death [ RR=3.413, 95% CI (1.049, 11.102) , P=0.041]. Conclusion:Cranial neuropathy is the most common type at the initial onset of SLE, while cerebrovascular disease is more common when SLE relapsed. Patients who had NPSLE at the initiation of SLE have shorter survival time than those who got NPSLE when SLE relapsed. Cerebrovascular disease is the independent risk factor of death of NPSLE patients.
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Objective To analyze the clinical features of neuropsychiatric systemic lupus erythematosus (NPSLE) with convulsion or coma as the main manifestation to facilitate the improvement of such patients' diagnosis. Methods Ninety-two patients with NPSLE confirmed in Peking Union Medical Hospital from January 2013 to December 2016 were collected, 27 NPSLE patients with convulsion or coma were in the study group, and the remaining 65 cases were in the control group. The following items in the two groups were compared in order to discover the differences in characteristics between the two groups: including sex, age, the first NPSLE episode or not, history of systemic lupus erythematosus (SLE), kidney, blood, heart, lung, skin mucous membrane, gastrointestinal involvement and co-infection, cerebrospinal fluid (CSF) pressure, cerebrospinal fluid cell count, cerebrospinal fluid/serum protein ratio, cerebrospinal fluid/serum glucose ratio, cerebrospinal fluid/serum chlorine ratio, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement 3 (C3), complement 4 (C4), magnetic resonance imaging (MRI) results, double strand deoxyribonucleic acid (ds-DNA) antibody, treatment status and hospitalization days. Results The number of CSF cells in the study group was significantly lower than that in the control group (×106/L: 91.84±25.37 vs. 279.52±101.12, P < 0.01). The skin mucosa involvement rate in the study group was significantly higher than that in the control group [14.81% (4/27) vs. 1.54% (1/65), P < 0.05]. Cerebrospinal fluid/serum protein ratio was higher in the study group than that in the control group (0.12±0.02 vs. 0.04±0.01, P < 0.05); the cerebrospinal fluid/serum glucose ratio was significantly lower than that in the control group (0.55±0.17 vs. 0.70±0.20, P < 0.01). The positive rate of MRI in the study group was higher than that in the control group [81.48% (22/27) vs. 55.38% (36/65), P < 0.05]; there were no significant differences between the two groups in other indexes (all P > 0.05). Conclusion Few cerebrospinal fluid cells increased involvement of skin mucosa, increased cerebrospinal fluid/serum protein ratio, decreased cerebrospinal fluid glucose/serum glucose ratio and increased MRI positive results were the clinical features of NPSLE patients with convulsion or coma as the clinical manifestation, early detection of this type of patients and early intervention can be beneficial to improve the prognosis.
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Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy. We systemically assessed the patient using the International Classification of Functioning, Disability and Health model as a clinical problem-solving tool and provided comprehensive rehabilitation by focusing on her problems.
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Adult , Female , Humans , Cerebral Infarction , International Classification of Functioning, Disability and Health , Lupus Erythematosus, Systemic , Lupus Vasculitis, Central Nervous System , Peripheral Nervous System , Polyneuropathies , Posterior Leukoencephalopathy Syndrome , Quality of Life , RehabilitationABSTRACT
Objective Neuropsychiatric systemic lupus erythematosus ( SLE) is a common complication of SLE, whose path-ogenesis is not yet clear but associated with the alteration of cerebral blood flow ( CBF) in some studies.This study was to investigate the CBF alteration in SLE patients without overt neuropsychiatric symptoms by arterial spin labeling ( ASL) MRI. Methods Twenty-eight SLE patients without overt neuropsychiatric symptoms and 30 age-and sex-matched healthy controls underwent conventional MRI and ASL examinations, and all received such neuropsychologic tests as number connecting test-A ( NCT-A ) , digit symbol test ( DST ) , self-rating anxiety scale ( SAS ) , and self-rating depression scale ( SDS) .Independent sample-t test was used to detect the mean CBF in the whole brain, gray matter, and white matter of the SLE patients and healthy controls.The voxel-wise CBF maps of the two groups of subjects were further analyzed with the SPM8 software to compare the regional CBF between the two groups, followed by evaluation of the correlation between the regional CBF values and clinical markers. Results In comparison with the healthy controls, the SLE pa-tients showed significantly reduced CBF in the gray matter (40.5 ±3.7 vs 37.3 ±6.5, P=0.028) and the whole brain (38.0 ±3.5 vs 35.1 ±6.1, P=0.032), especially in the supplementary motor area and the adjacent middle cingulate, anterior cingulate, left medial frontal gyrus, left inferior frontal gyrus, and left insula (P<0.05, FWE corrected).The NCT-A score was negatively correlated with the CBF values of the left medial frontal gyrus (r=-0.402, P=0.032) and left inferior frontal gyrus (r=-0.382, P=0.045) of the SLE patients. Conclusion ASL and MRI showed significantly reduced cerebral blood flow in the SLE patient without overt neu-ropsychiatric manifestations, which was correlated with the change of the patient's cognitive function.
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Neuropsychiatric systemic lupus erythematosus(NPSLE)of children is a serious complication of children with systemic lupus erythematosus (SLE),and the incidence is even up to 95 ％.The pathogenic pathogenesis of NPSLE maybe result from participation of many factors including the blood-brain barrier damage,blood clots,neuroendocrine disorders,autoantibody production and inflammatory mediators.Its clinical manifestations are complexity and diversity.Headache,epilepsy,mental disorders are comom in children with NPSLE.There is no unified diagnosis for NPSLE,which must be evaluated by the general methods including immunoserological testing,cerebrospinal fluid testing,neuroimaging and neuropsychological assessments.The management of patients with NPSLE consists of immunosuppressive,biotherapy and symptomatic therapies.Early diagnosis and standard treatment may improve the prognosis of children with NPSLE.
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Objective To explore the diagnostic value of MRI in diagnosis of neuropsychiatric systemic lupus erythematosus (NP-SLE).Methods 9 cases with NP-SLE were examined with PICKER 0.23T imaging system during 1～6 months after presenting clinical signs of NP-SLE. All patients with NP-SLE were imaged by spin-echo and fast spin-echo sequences in axial,sagittal and coronal planes with a head coil.Results In all 9 cases, 5 cases were diffuse NP-SLE which showed multiple points,patch abnormal signal,isointense on T 1WI and hyperintense on T 2WI images,both sides of white matter were involved . 2 patients showed focal abnormal signal in brain, while 1 case showed isointense on T 1WI and small patch hyperintense on T 2WI in basal ganglia which didn't change after treatment, another one showed small patch low signal intensity on T 1WI and high signal intensity on T 2WI( meaning antiquated hemorrhagic infarction)in the head of left caudate nucleus. Among 9 cases, 2 cases showed normal MRI signals.Conclusion MRI is sensitive in displaying cerebral focus,and has important value in diagnosis of NP-SLE. It is important that MRI must be combined with clinical and laboratory data,so that,the characteristic of high sensitivity can be used abundantly and correct diagnosis acquired. In the same time MRI can instruct clinical treatment.