Dry eye disease in systemic lupus erythematosus: a cross sectional study

AIM:To explore ocular surface manifestations of dry eye disease(DED)and its influencing factors in systemic lupus erythematosus(SLE)patients.METHODS:Ophthalmological examinations were conducted in SLE patients(n=43)and controls(n=41),including Ocular Surface Disease Index(OSDI),objective scatter index(OSI),tear meniscus height(TMH),lipid layer thickness(LLT),non-invasive Keratograph tear breakup time(NIKBUT),corneal fluorescein score(CFS),Schirmer I test.DED was diagnosed according to the Tear Film and Ocular Surface Society Dry Eye Workshop II Criteria.SLE patients were further divided into DED group and non-DED group,the disease activity,clinical manifestations and laboratory investigations were compared between the two groups.The disease activity was evaluated by Systemic Lupus Erythematosus Disease Activity Index 2000(SLEDAI-2K).Receiver operative characteristic(ROC)curve and multiplefactor binary logistic regression were performed.RESULTS:SLE patients showed higher OSDI[9.1(2.8-15.9)vs 6.3(2.2-7.5),P=0.035],higher OSI[1.67(1.09-2.60)vs 0.96(0.87-1.60),P=0.001],higher CFS[1(0-2)vs 0(0-1),P=0.001],lower LLT[65(42-100)vs 100(79.5-100),P=0.010],and lower NIKBUT[8.03(4.02-9.73)vs 9.67(5.26-12.71),P=0.030]than controls.The 32.6%of SLE patients had DED,which was higher than 12.2%of healthy controls.DED group showed higher SLEDAI-2K score[9.7±6.1 vs 5.4±3.4,P=0.025],higher anti-cardiolipin antibody(ACL)[8.7(3.5-13.2)vs 3.6(2.0-6.9),P=0.035],and higher proportion of patients with cutaneous eruption[42.9%vs 6.9%,P=0.015]than non-DED group.According to multiple-factor binary logistic regression analysis,the SLEDAI-2K score(OR=1.194,P=0.041)and cutaneous eruption(OR=7.094,P=0.045)could be consider as risk factors for DED in SLE patients.The ROC curve of the combined factors including age,disease duration,SLEDAI-2K score,ACL,and cutaneous eruption was analyzed,with a sensitivity of 0.786,a specificity of 0.793,and an area under curve of 0.820.CONCLUSION:Ocular surface affection is frequent in SLE patients,and patients with high disease activity and cutaneous eruption show increased risk of DED.

Systemic lupus erythematosus in a 15-year-old female with multiple splenic nodules:A case report

BACKGROUND Systemic lupus erythematosus(SLE)is a chronic inflammatory disease primarily affecting young females.SLE can invade any organ,and various forms of splenic invasion have been reported.Manifestations include splenomegaly and splenic infarction,rupture,and calcification.The study encountered a rare case of splenic involvement,with nodules of various sizes without calcifications or ruptures.CASE SUMMARY A 15-year-old girl presented with arthralgia,weight loss,fever,increased levels of inflammatory markers,and positive antinuclear antibody test results.The patient was diagnosed with SLE.She was asymptomatic while taking steroids and hydroxychloroquine.Ten months after discharge,the patient developed a fever and abdominal pain.Lupus enteritis was suspected,and abdominopelvic computed tomography(AP-CT)was performed.There were no specific findings in the gastrointestinal tract,but multiple splenic nodules were observed.Infection or hemangioma was considered;however,no specific radiological findings were observed.A biopsy of the spleen was performed to determine the possibility of malignancy.The histological findings of the spleen included extensive periarteriolar necrosis with hematoxylin bodies and numerous karyorrhectic debris.Based on the biopsy results,the patient was diagnosed with an SLE flare-up and was maintained on high-dose steroids and immunosuppressants.CONCLUSION As disease activity increased,multiple nodules in the spleen that were previously unseen were observed using AP-CT and histologically confirmed.Spleen invasion by SLE can appear in multiple nodular forms and patterns.Therefore,physicians should consider these findings when differentiating these nodules from infections and malignancies.

Refractory autoimmune hemolytic anemia in a patient with systemic lupus erythematosus and ulcerative colitis:A case report

BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no reports of UC with SLE in China,but six cases of SLE with UC have been reported in China.The combination of these two diseases has distinct effects on the pathogenesis of both diseases.CASE SUMMARY A female patient(30 years old)came to our hospital due to dull umbilical pain,diarrhea and mucous bloody stool in August 2018 and was diagnosed with UC.The symptoms were relieved after oral administration of mesalazine(1 g po tid)or folic acid(5 mg po qd),and the patient were fed a control diet.On June 24,2019,the patient was admitted for treatment due to anemia and tinnitus.During hospitalization,the patient had repeated low-grade fever and a progressively decreased Hb level.Blood tests revealed positive antinuclear antibody test,positive anti-dsDNA antibody,0.24 g/L C3(0.9-1.8 g/L),0.04 g/L C4(0.1-0.4 g/L),32.37 g/L immunoglobulin(8-17 g/L),and 31568.1 mg/24 h total 24-h urine protein(0-150 mg/24 h).The patient was diagnosed with SLE involving the joints,kidneys and blood system.Previously reported cases of SLE were retrieved from PubMed to characterize clinicopathological features and identify prognostic factors for SLE.CONCLUSION The patient was discharged in remission after a series of treatments,such as intravenous methylprednisolone sodium succinate,intravenous human immunoglobulin,cyclophosphamide injection,and plasma exchange.After discharge,the patient took oral prednisone acetate tablets,cyclosporine capsules,hydroxychloroquine sulfate tablets and other treatments for symptoms and was followed up regularly for 1 month,after which the patient's condition continued to improve and stabilize.

Analysis of status and influencing factors of mental health in patients with systemic lupus erythematosus

BACKGROUND Systemic lupus erythematosus(SLE)is a heterogeneous autoimmune disorder with varied clinical courses and prognoses,not only did the patients suffer from physical impairment,but also various physical and psychiatric comorbidities.Growing evidence have suggested that mental disorders in SLE patients,can lead to various adverse consequences.AIM To explored the features and influencing factors of mental health in patients with SLE and clarifying the correlations between mental health and personality characteristics and perceived social support.The results would provide a basis for psychological intervention in patients with SLE.METHODS The clinical data of 168 patients with SLE admitted at the First Affiliated Hospital of Hainan Medical University between June 2020 and June 2022 were collected.Psychological assessment and correlation analysis were conducted using the Symptom Checklist-90(SCL-90)and Perceived Social Support Scale,and the collected data were compared with the national norms in China.The relevant factors influencing mental health were identified by statistical analysis.A general information questionnaire,the Revised Life Orientation Test,and Short-Form 36-Item Health Survey were employed to assess optimism level and quality of life(QoL),respectively.RESULTS Patients with SLE obtained higher scores for the somatization,depression,anxiety,and phobic anxiety subscales than national norms(P<0.05).A correlation was identified between total social support and total SCL-90 score or each subscale(P<0.05).The factors significantly affecting patients’mental health were hormone dosage and disease activity index(DAI)(P<0.05).The average optimism score of patients with SLE was 14.36±4.42,and 30 cases were in the middle and lower levels.A positive correlation was found between optimism level and QoL scores.CONCLUSION Patients with SLE develop psychological disorders at varying degrees,which are significantly influenced by hormone dosage and DAI.Patients’mental health should be closely monitored during clinical diagnosis and treatment and provided adequate support in establishing positive,healthy thinking and behavior patterns and improving their optimism level and QoL.

Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.

BCD020 rituximab bioanalog compared to standard treatment in juvenile systemic lupus erythematosus: The data of 12 months casecontrol study

BACKGROUND Systemic lupus erythematosus(SLE)is the most frequent and serious systemic connective tissue disease.Nowadays there is no clear guidance on its treatment in childhood.There are a lot of negative effects of standard-of-care treatment(SOCT),including steroid toxicity.Rituximab(RTX)is the biological B-lymphocyte-depleting agent suggested as a basic therapy in pediatric SLE.AIM To compare the benefits of RTX above SOCT.METHODS The data from case histories of 79 children from the Saint-Petersburg State Pediatric Medical University from 2012 to 2022 years,were analyzed.The diagnosis of SLE was established with SLICC criteria.We compared the outcomes of treatment of SLE in children treated with and without RTX.Laboratory data,doses of glucocorticosteroids,disease activity measured with SELENA-SLEDAI,RESULTS Patients,treated with RTX initially had a higher degree of disease activity with prevalence of central nervous system and kidney involvement,compared to patients with SOCT.One year later the disease characteristics became similar between groups with a more marked reduction of disease activity(SELENA-SLEDAI activity index)in the children who received RTX[-19 points(17;23)since baseline]compared to children with SOCT[-10(5;15.5)points since baseline,P=0.001],the number of patients with active lupus nephritis,and daily proteinuria.During RTX therapy,infectious diseases had three patients;one patient developed a bi-cytopenia.CONCLUSION RTX can be considered as the option in the treatment of severe forms of SLE,due to its ability to arrest disease activity compared to SOCT.

Outcomes of a 12-month course of early and late rituximab BCD020 biosimilar administration in juvenile systemic lupus erythematosus:A retrospective study

BACKGROUND Juvenile systemic lupus erythematosus(SLE)is a severe,life-threatening disease.However,the role of rituximab in managing juvenile SLE remains undefined,although early biological intervention may improve disease outcomes.AIM To assess the differences in the outcomes of different types of rituximab administration(early and late).METHODS In this retrospective cohort study,the information of 36 children with SLE with administration(LRA)was analyzed.We compared initial disease characteristics at onset,at baseline(start of rituximab),and at the end of the study(EOS)at 12 months,as well as outcomes and treatment characteristics.RESULTS The main differences at baseline were a higher daily median dose of corticosteroids,increased MAS frequency,and a higher Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)in the ERA group.No differences in the main SLE outcomes between groups at the EOS were observed.The part of lupus nephritis patients who achieved remission changed from 44%to 31%in ERA and 32%to 11%in the LRA group.Patients with ERA had a shorter time to achieve low daily corticosteroid dose(≤0.2 mg/kg)at 1.2(0.9;1.4)years compared to 2.8(2.3;4.0)years(P=0.000001)and higher probability to achieve this low dose[hazard ratio(HR)=57.8(95%confidence interval(CI):7.2-463.2),P=0.00001 and remission(SLEDAI=0);HR=37.6(95%CI:4.45-333.3),P=0.00001].No differences in adverse events,including severe adverse events,were observed.CONCLUSION ERA demonstrated a better steroid-sparing effect and a possibility of earlier remission or low disease activity,except for lupus nephritis.Further investigations are required.

Ovarian function in patients with systemic lupus erythematosus:Pathogenesis,drug application and prospective therapies

Systemic lupus erythematosus(SLE)is a chronic autoimmune disease in which multiple organs are damaged that prevails in fertile women.Currently,glucocorticoids and immunosuppressants are widely used to treat SLE patients.However,ovarian dysfunction occurs following the use of these drugs in women with SLE.Here,we summarize recent progress in terms of understanding ovarian injury,the effects of drug application and strategies to improve ovarian function in women with SLE.This review could be helpful to precisely cure SLE in women desiring to have offspring.

Unusual presentation of systemic lupus erythematosus as hemophagocytic lymphohistiocytosis in a female patient: A case report

BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.CASE SUMMARY We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus(SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.CONCLUSION The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.

Acute flare of systemic lupus erythematosus with extensive gastrointestinal involvement: A case report and review of literature

BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.

Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.

Peripheral CD4^(+)CD8^(+) double positive T cells:A potential marker to evaluate renal impairment susceptibility during systemic lupus erythematosus

Lupus nephritis(LN) has a high incidence in systemic lupus erythematosus(SLE) patients, but there is a lack of sensitive predictive markers. The purpose of the study was to investigate the association between the CD4^(+)CD8^(+)double positive T(DPT) lymphocytes and LN. The study included patients with SLE without renal impairment(SLE-NRI), LN, nephritic syndrome(NS), or nephritis. Peripheral blood lymphocyte subsets were analyzed by flow cytometry. Biochemical measurements were performed with peripheral blood in accordance with the recommendations proposed by the National Center for Clinical Laboratories. The proportions of DPT cells in the LN group were significantly higher than that in the SLE-NRI group(t=4.012, P<0.001), NS group(t=3.240,P=0.001), and nephritis group(t=2.57, P=0.011). In the LN group, the risk of renal impairment increased significantly in a DPT cells proportion-dependent manner. The risk of LN was 5.136 times(95% confidence interval, 2.115–12.473) higher in cases with a high proportion of DPT cells than those whose proportion of DPT cells within the normal range. These findings indicated that the proportion of DPT cells could be a potential marker to evaluate LN susceptibility, and the interference of NS and nephritis could be effectively excluded when assessing the risk of renal impairment during SLE with DPT cell proportion.

Effects of Comprehensive Rehabilitation on Patients with Progressive Multifocal Leukoencephalopathy Due to Systemic Lupus Erythematosus: A Case Report

Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describe a patient with SLE complicated by PML and ameliorated by comprehensive rehabilitation. We also review the epidemiology, pathology, imaging characteristics, and treatment of PML. Patient Concerns: We found a patient with SLE with PML improved by multidisciplinary rehabilitation techniques. Diagnoses, Interventions, and Outcomes: We diagnosed a PML with a 13-year history of SLE and lupus nephritis after longtime immunosuppressive therapy. The patient underwent a comprehensive, multifaceted rehabilitation program, including drug therapy, integrated physical therapy, occupational therapy, acupuncture, music therapy, computer-aided cognitive rehabilitation training, and behavioral management training. This rehabilitation program improved her motor function and activities of daily living. Conclusions: Her condition improved in the short term through comprehensive rehabilitation, including physical, speech, and cognitive therapy. Therefore, we recommend comprehensive rehabilitation to improve the function and activities of daily living in patients with PML.

Glucocorticoid reduction induced chorea in pediatric-onset systemic lupus erythematosus:A case report

BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication reported in 2.4%-7%of SLE patients.In particular,chorea induced by glucocorticoid dose reduction is even rarer.Herein,we report the case of a girl with SLE,who developed chorea during the process of glucocorticoid therapy reduction.CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE.She was treated with methylprednisolone and rituximab,and her symptoms improved.On the second day after the methylprednisolone dose was reduced according to the treatment guidelines,the patient developed chorea.Her condition improved after adjusting her glucocorticoid regimen.CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.

Altered expression of miR-125a and dysregulated cytokines in systemic lupus erythematosus: Unveiling diagnostic and prognostic markers

BACKGROUND Systemic lupus erythematosus(SLE)is a chronic autoimmune disorder impacting multiple organs,influenced by genetic factors,especially those related to the immune system.However,there is a need for new biomarkers in SLE.MicroRNA-125a(miR-125a)levels are decreased in T cells,B cells,and dendritic cells of SLE patients.MiR-125a plays a regulatory role in controlling the levels of tumor necrosis factor-alpha(TNF-α)and interleukin 12(IL-12),which are crucial pro-inflammatory cytokines in SLE pathogenesis.AIM To assess the levels of miR-125a,IL-12,and TNF-αin SLE patients’plasma,evaluating their diagnostic and prognostic value.METHODS The study included 100 healthy individuals,50 newly diagnosed(ND),and 50 SLE patients undergoing treatment.The patients were monitored for a duration of 24 wk to observe and record instances of relapses.MiR-125a expression was measured using real-time reverse transcription polymerase chain reaction,while ELISA kits were used to assess IL-12 and TNF-αproduction.RESULTS The results showed significantly reduced miR-125a expression in SLE patients compared to healthy individuals,with the lowest levels in ND patients.TNF-αand IL-12 expression levels were significantly elevated in SLE patients,especially in the early stages of the disease.Receiver operating characteristic curve analyses,and Cox-Mantel Log-rank tests indicated miR-125a,TNF-α,and IL-12 as proper diagnostic biomarkers for SLE.A negative correlation was found between plasma miR-125a expression and IL-12/TNF-αlevels in SLE patients.CONCLUSION Decreased miR-125a levels may be involved in the development of SLE,while elevated levels of IL-12 and TNF-αcontribute to immune dysregulation.These findings offer new diagnostic and prognostic markers for SLE.Moreover,the negative correlation observed suggests an interaction between miR-125a,TNF-α,and IL-12.Further research is necessary to uncover the underlying mechanisms that govern these relationships.

Magnusiomyces capitatus in Immune-Competent Patients with Pulmonary Haemorrhage and Systemic Lupus Erythematosus

Invasive fungal infections have grown significantly over the last two decades, owing to an increase in immunocompromised hosts and geriatric patients. When the host’s defenses are compromised, such infections are associated with severe morbidity and mortality. Here, a rare case of fungal infection in a 61-year-old immunocompetent male patient from Saudi Arabia was reported, who suffered from pulmonary hemorrhage and Systemic Lupus Erythematous. Bronchoalveolar Lavage was used as a diagnostic tool to identify the fungus reported in the case. The pathogenic fungal specie identified as Magnusiomyces capitatus, in macroscopic and microscopic morphological characteristics of the colonies. Based on clinical evidence, liposomal amphotericin formulation was recommended for initial therapy against fungal infection. Also, liposomal amphotericin B induced mycological eradication up to 70 percent in patients with proven Magnusiomyces capitatus infection. In addition to addressing suspected Systemic lupus erythematosus, the patient’s health has improved with no evidence of pulmonary bleeding and hemoptysis.

Combining single-cell RNA-sequencing and bulk data to reveal immunity-related genes expression pattern in the systemic lupus erythematosus and target organ kidney

Background:Systemic lupus erythematosus(SLE)is a complex chronic autoimmune disease with no known cure.However,the regulatory mechanism of immunity-related genes is not fully understood in SLE.In order to explore new therapeutic targets,we used bioinformatical methods to analyze a series of data.Methods:After downloading and processing the data from Gene Expression Omnibus database,the differentially expressed genes of SLE were analyzed.CIBERSORT algorithm was used to analyze the immune infiltration of SLE.Based on single-cell RNA-sequencing data,the role of immune-related genes in SLE and its target organ(kidney)were analyzed.Key transcription factors affecting immune-related genes were identified.Cell-cell communication networks in SLE were analyzed.Results:In total,15 hub genes and 4 transcription factors were found in the bulk data.Monocytes and macrophages in GSE81622(SLE)showed more infiltration.There were four cell types were annotated in scRNA sequencing dataset(GSE135779),as follows T cells,monocyte,NK cells and B cells.Immunity-related genes were overexpressed in monocytes.Conclusion:The present study shows that immune-related genes affect SLE through monocytes and play an important role in target organ renal injury.

A Review of Systemic Lupus Erythematosus (SLE): Symptoms, Risk Factors, Treatment, and Health Related Quality of Life Issues

In this paper, we present a thorough review of one of the most life-threatening autoimmune diseases, Systemic lupus erythematosus (lupus). Symptoms, risk factors, including genetic and epidemiological factors are discussed. Treatment, life expectancies, and Health Related Quality of Life of patients with SLE will be discussed as well. Special attention will be given to Lupus Nephritis.

Neurological Involvement in Systemic Lupus Erythematosus (SLE): Our Recent Experience

Introduction: The central, psychiatric and peripheral neurological manifestations of lupus are among the most severe visceral disorders and are grouped under the general term of “neuro-psychiatric systemic lupus erythematosus” (NPSLE). We conducted a cross-sectional observational study within our Department of Internal Medicine aimed at describing the clinical and evolutionary aspects of central neurological disorders of SLE, excluding lupus myelopathy. Patients and Methods: This was a retrospective and observational cross-sectional study carried out from 1 January 2015 to 31 October 2017, in the Department of Internal Medicine of Aristide le Dantec University Hospital in Dakar (Senegal). All patients hospitalized during this period who met the 1997 ACR classification criteria of SLE and who presented with a central neuropsychiatric syndrome attributable to SLE (as defined by ACR 1999) were included. Patients with isolated headache, acute myelitis or secondary neurological involvement attributable to a toxic, metabolic, infectious or tumour-related cause were excluded from our study. Results: During the study period, 10 patients with neuropsychiatric lupus involvement were treated at our institution, including 9 women and 1 man;the median age was 29 years (20 - 55 years). Neurological involvement occurred during the course of lupus evolution in 9/10 cases. The median time to SLE evolution was 18 months (0 - 60 months). Neuropsychiatric syndromes as defined by the 1999 ACR were commonly associated and more than half of our patients had multiple neuropsychiatric syndromes. There were 5 cases of confusion syndrome and coma, 4 cases of seizure, 3 cases of psychosis, 2 cases of acute cerebrovascular disease and 1 case of aseptic meningitis. Among the extra-neurological manifestations of SLE, haematological and dermatological involvements were common. Renal involvement affected half of the patients. The other manifestations were: polyarthritis in 3 patients, serositis in 2 patients, 5 cases of fever, 4 cases of deterioration of the general state, and one isolated case of ophthalmological involvement. Therapeutically, 8 patients received a bolus of methylprednisolone and 3 patients received a bolus of cyclophosphamide. Oral corticosteroids and hydroxychloroquine were administered to all patients, and azathioprine was administered in 2 patients. The evolution was favorable in 4 patients, other 2 patients maintained neurological sequelae and 2 patients were transferred to intensive care. Death was recorded in 4 patients. Conclusion: Neuropsychiatric manifestations of lupus are rare and sometimes severe, potentially life-threatening. In our patients, we have identified some of the most severe neurological syndromes according to the ACR nomenclature. The neurological involvement is exceptionally revealing, as these syndromes are often associated and integrated into a systemic context of lupus. The evolution is rapidly unfavorable and requires early diagnosis and optimal management.

The Analysis of Clinical Features of Systemic Lupus Erythematosus (SLE) in Children

Objective:Observe the clinical characteristics of children with SLE,namely,to observe the symptoms and laboratory examinations,such as blood routine,blood lipid,immunoglobulin,complement,autoantibodies,serum 25(OH)D and other indicators,and to explore the clinical characteristics,the difference and the significance of vitamin D supplements between male and female SLE patients in children respectively.Methods:We enrolled 64 cases of SLE patients in children who were admitted into the department of pediatrics and rheumatology of the third affiliated hospital of sun yat-sen university in guangzhou from May 1,2011 to February 1,2019,They were analyzed retrospectively,adoptingΧ²test for statistical analysis.Results:64 cases of SLE in children,which included 10 cases of male and 54 cases of female.Clinical manifestations:facial skin rash in 48 patients(75%),fever in 38 cases(59.4%),arthritis in 28 cases(43.8%),oral ulcer in 18 cases(28.1%),serositis in13 cases(20.3%),and the sun allergy in 9 cases(14.1%),the damage of central nervous system in 7 cases(10.9%).Laboratory examination:30 cases of leukopenia(46.9%),anemia in 30 cases(46.9%),thrombocytopenia in 12 cases(18.8%),hematuria in 18 cases(28.1%),proteinuria in 33 cases(51.2%),6 patients with renal impairment(9.4%),antinuclear antibody positive in 63 cases(98.4%),anti-double-stranded DNA(dsDNA)antibody positive in 48 cases(75%),anti SSA antibody positive in 44 cases(68.7%),SSB antibody positive in 33 cases(51.6%),Sm antibody positive in 40 cases(62.5%),nucleosome antibody positive in 28 cases(43.8%).Among these children,male SLE patients were higher than female children with SLE in the damage of kidney,Sm antibodies and resisting nucleosome antibody positive rates(Χ²=4.451,8.336,6.803,P<0.05),the female children with SLE was higher than male SLE Children in the anti-SSB antibody positive rate(Χ²=4.945,P<0.05).In 64 cases of SLE children,which included 52 cases were lower than the normal level of serum 25(OH)D measurements,12 cases were in the normal lower limit of serum 25(OH)D measurements,at the same time,the female SLE.Patients was higher than male children with SLE in the reduce rate of serum 25(OH)D(Χ²=8.351,P<0.05).Conclusion:Male SLE patients which appeared damage of kidney easier than female patients,the proteinuria was the most common in the damage of kidney.Resistance to Sm antibodies which was the risk factor of renal injury with higher incidence in male children with SLE;Anti nucleosome antibody which was the risk factor for the disease activity in male children with SLE were higher than female children with SLE.It was estimated that the risk of Sjogren’s syndrome appeared in female with SLE were higher than that in male SLE children.In this retrospective study,the serum 25(OH)D levels were significantly lower in children with SLE,and vitamin D supplementation was required.