Some patients with systemic lupus erythematosus experience neuropsychiatric symptoms.Although magnetic resonance imaging can detect abnormal signals in the white matter of the brain,conventional methods often struggle...Some patients with systemic lupus erythematosus experience neuropsychiatric symptoms.Although magnetic resonance imaging can detect abnormal signals in the white matter of the brain,conventional methods often struggle to accurately capture microstructural changes.Various diffusion models have been used to study white matter in systemic lupus erythematosus;however,comparative analyses of their sensitivity and specificity for detecting microstructural changes remain insufficient.To address this,our team designed a diagnostic trial that used multimodal diffusion imaging techniques to observe white matter microstructural changes in patients with systemic lupus erythematosus who had neuropsychiatric symptoms,with an aim to identify key diagnostic biomarkers for these patients.Patients with active lupus who received treatment at the Department of Rheumatology and Immunology,The First Affiliated Hospital of China Medical University,from September 2023 to March 2024 were recruited.According to the standards of the American College of Rheumatology,patients with systemic lupus erythematosus who had neuropsychiatric symptoms were assigned to the systemic lupus erythematosus group,whereas those without neuropsychiatric symptoms were assigned to the non-systemic lupus erythematosus group.Additionally,healthy volunteers matched by region,sex,and age were recruited as controls.All three groups underwent the same diffusion magnetic resonance imaging examination protocol to compare differences in diffusion parameters.Advanced diffusion imaging models were able to sensitively detect microstructural changes in the white matter fibers of patients with systemic lupus erythematosus who had neuropsychiatric symptoms,with specific diffusion parameters showing significant abnormalities in key brain regions.In the left superior longitudinal fasciculus subregion and the right thalamic radiations of patients with systemic lupus erythematosus who had neuropsychiatric symptoms,we also identified abnormal diffusion characteristics that were clearly correlated with disease activity,suggesting that microstructural changes in these areas may reflect the dynamic process of neuroinflammatory damage.The present study addresses critical challenges in the diagnosis of systemic lupus erythematosus by identifying specific white matter imaging biomarkers and elucidating the association between microstructural damage and clinical manifestations.The main contributions of our study include:1)establishing axial regression probability parameters from mean apparent propagator magnetic resonance imaging as sensitive biomarkers for systemic lupus erythematosus,particularly in the third subregion of the left superior longitudinal fasciculus;2)demonstrating that multimodal diffusion imaging may be superior to conventional diffusion tensor imaging for detecting white matter microstructural abnormalities in patients with systemic lupus erythematosus;and 3)integrating tract-based spatial statistics with clinically relevant analyses to link imaging findings to pathological mechanisms.展开更多
BACKGROUND Not all neuropsychiatric(NP)manifestations in patients with systemic lupus erythematosus(SLE)are secondary to lupus.The clarification of the cause of NP symptoms influences therapeutic strategies for SLE.AI...BACKGROUND Not all neuropsychiatric(NP)manifestations in patients with systemic lupus erythematosus(SLE)are secondary to lupus.The clarification of the cause of NP symptoms influences therapeutic strategies for SLE.AIM To understand the attribution of psychiatric manifestations in a cohort of Chinese patients with SLE.METHODS This retrospective single-center study analyzed 160 inpatient medical records.Clinical diagnosis,which is considered the gold standard,was used to divide the subjects into a psychiatric SLE(PSLE)group(G1)and a secondary psychiatric symptoms group(G2).Clinical features were compared between these two groups.The sensitivity and specificity of the Italian attribution model were explored.RESULTS A total of 171 psychiatric syndromes were recorded in 138 patients,including 87 cases of acute confusional state,40 cases of cognitive dysfunction,18 cases of psychosis,and 13 cases each of depressive disorder and mania or hypomania.A total of 141(82.5%)syndromes were attributed to SLE.In contrast to G2 patients,G1 patients had higher SLE Disease Activity Index-2000 scores(21 vs 12,P=0.001),a lower prevalence of anti-beta-2-glycoprotein 1 antibodies(8.6%vs 25.9%,P=0.036),and a higher prevalence of anti-ribosomal ribonucleoprotein particle(rRNP)antibodies(39.0%vs 22.2%,P=0.045).The Italian attribution model exhibited a sensitivity of 95.0%and a specificity of 70.0%when the threshold value was set at 7.CONCLUSION Patients with PSLE exhibited increased disease activity.There is a correlation between PSLE and anti-rRNP antibodies.The Italian model effectively assesses multiple psychiatric manifestations in Chinese SLE patients who present with NP symptoms.展开更多
Objective This study aims to investigate the exosome-derived metabolomics profiles in systemic lupus erythematosus(SLE),identify differential metabolites,and analyze their potential as diagnostic markers for SLE and l...Objective This study aims to investigate the exosome-derived metabolomics profiles in systemic lupus erythematosus(SLE),identify differential metabolites,and analyze their potential as diagnostic markers for SLE and lupus nephritis(LN).Methods Totally,91 participants were enrolled between February 2023 and January 2024 including 58 SLE patients[30 with nonrenal-SLE and 28 with Lupus nephritis(LN)]and 33 healthy controls(HC).Ultracentrifugation was used to isolate serum exosomes,which were analyzed for their metabolic profiles using liquid chromatography–tandem mass spectrometry(LC–MS/MS).Endogenous metabolites were identified via public metabolite databases.Random Forest,Lasso regression and Support Vector Machine Recursive Feature Elimination(SVM-RFE)algorithms were employed to screen key metabolites,and a prediction model was constructed for SLE diagnosis and LN discrimination.ROC curves were constructed to determine the potential of these differential exosome-derived metabolites for the diagnosis of SLE.Furthermore,Spearman’s correlation was employed to evaluate the potential links between exosome-derived metabolites and the clinical parameters which reflect disease progression.Results A total of 586 endogenous serum exosome-derived metabolites showed differential expression,with 225 exosome-derived metabolites significantly upregulated,88 downregulated and 273 exhibiting no notable changes in the HC and SLE groups.Machine learning algorithms revealed three differential metabolites:Pro-Asn-Gln-Met-Ser,C24:1 sphingolipid,and protoporphyrin IX,which exhibited AUC values of 0.998,0.992 and 0.969 respectively,for distinguishing between the SLE and HC groups,with a combined AUC of 1.0.In distinguishing between the LN and SLE groups,the AUC values for these metabolites were 0.920,0.893 and 0.865,respectively,with a combined AUC of 0.931,demonstrating excellent diagnostic performance.Spearman correlation analysis revealed that Pro-Asn-Gln-Met-Ser and protoporphyrin IX were positively correlated with the SLE Disease Activity Index(SLEDAI)scores,urinary protein/creatinine ratio(ACR)and urinary protein levels,while C24:1 sphingolipid exhibited a negative correlation.Conclusions This study provides the first comprehensive characterization of the exosome-derived metabolites in SLE and established a promising prediction model for SLE and LN discrimination.The correlation between exosome-derived metabolites and key clinical parameters strongly indicated their potential role in SLE pathological progression.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)patients are admitted to the intensive care unit(ICU)for disease flares and infections,both of which carry a high mortality risk.Studies characterizing the outcome predictor...BACKGROUND Systemic lupus erythematosus(SLE)patients are admitted to the intensive care unit(ICU)for disease flares and infections,both of which carry a high mortality risk.Studies characterizing the outcome predictors of SLE are few in the Asian continent.This study characterized the clinical profile,treatment,and outcome predictors of ICU admissions with SLE.AIM To ascertain the reasons for ICU admission among SLE patients and to explore outcome predictors in these patients.The primary outcome was ICU mortality.Secondary outcomes included need for ventilation,inotropes,renal replacement therapy,and length of ICU and hospital stay.METHODS A retrospective study of 77 SLE patients was conducted in the medical ICU of a tertiary care teaching hospital in India.Clinical features,treatment,and outcomes of patients admitted between January 2018 and December 2022 were recorded.Factors associated with mortality were explored using bivariate and multivariate logistic regression analysis and reported as adjusted odds ratios with 95%confidence intervals.RESULTS The mean(SD)age was 31.1(10.3)years;83.1%were female.The median(interquartile)duration of SLE before admission was 12(1-60)months;SLE was newly diagnosed in the current admission in 23.4%.The median Acute Physiology and Chronic Health Evaluation II score was 16.3(14.5-18.2)and similar among survivors and nonsurvivors;32 had evidence of disease flare,44 had an infection,and one patient had an intracranial bleed.ICU admission was for respiratory failure(46.7%),hemodynamic instability(32.5%),and status epilepticus(14.3%).Twenty-nine patients(37.7%)had autoimmune hemolytic anemia,and 11(14.3%)had diffuse alveolar hemorrhage.Immunomodulation included corticosteroids(96.1%),cyclophosphamide(33.8%),mycophenolate(23.4%),plasma exchange(13%),and immunoglobulins(11.7%).All patients received broad-spectrum antibiotics.Respiratory support,inotropes,and renal replacement therapy were required in 93.5%,51.7%,and 32.5%,respectively.ICU mortality was 50.7%(95%confidence interval:39%-62%).The mean±SD hospital length of stay was 18.9±14.3 days.On multivariate analysis,only shock(P=0.004)was independently associated with mortality.CONCLUSION Intercurrent infection and disease flare are common reasons for ICU admission in SLE patients.Despite multimodal therapy,mortality is high.Shock was independently associated with mortality.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)can affect multiple organs or systems.The involvement of the central nervous system can result in the manifestation of epilepsy,an acute confusional state,and other rare neu...BACKGROUND Systemic lupus erythematosus(SLE)can affect multiple organs or systems.The involvement of the central nervous system can result in the manifestation of epilepsy,an acute confusional state,and other rare neuropsychiatric presentations,such as catatonia.CASE SUMMARY We present a case of an adolescent male patient with first-onset SLE who presented with neuropsychiatric symptoms including epilepsy and delirium.The initial utilization of olanzapine to alleviate symptoms of agitation precipitated the emergence of catatonia,which was mitigated by discontinuing olanzapine and supplementing with lorazepam.In this case,whether the catatonia was secondary to the utilization of antipsychotics or to an organic disease is a question that warrants differential diagnosis.CONCLUSION Multidisciplinary collaborative management is the cornerstone for the successful management of severe cases of SLE.展开更多
AIM:To summarize and quantitatively evaluate vasculature alteration of foveal zone in systemic lupus erythematosus(SLE)patients by secondary literature analysis.METHODS:A systematic search of PubMed,Embase,Web of Scie...AIM:To summarize and quantitatively evaluate vasculature alteration of foveal zone in systemic lupus erythematosus(SLE)patients by secondary literature analysis.METHODS:A systematic search of PubMed,Embase,Web of Science,Cochrane Library,CBM,CNKI WanFang Data and VIP was conducted.Studies were about retinal vessel density in SLE patients from January 2000 to April 2023 and valid data were extracted.The Joanna Briggs Institute(JBI)critical appraisal checklist was used to evaluate the cross-sectional studies and prospective studies.The measurement data for combined effect size were weighted mean difference(WMD)and 95%confidence interval(CI).The heterogeneity was evaluated by I2 test.The fixed-effect model was adopted when P>0.1 or I2<50%,and random-effect model was adopted in the contrary.Subgroup and sensitivity analysis were utilized to analyze the sources of heterogeneity.The publication bias was evaluated by Egger tests and funnel plots.RESULTS:A total of 14 studies with 445 subjects and 441 healthy controls from 9 countries were enrolled and 11 studies were included in Meta-analysis.The JBI scores of studies were no less than 14 points.The Metaanalysis results indicated that mean parafoveal superficial vessel density(SVD;WMD=-1.22,95%CI:-1.67,-0.76),mean perifoveal SVD(WMD=-1.42,95%CI:-1.95,-0.89),mean whole SVD(WMD=-1.66,95%CI:-2.53,-0.79),mean parafoveal deep vessel density(WMD=-1.67,95%CI:-2.75,-0.59)and mean whole deep vessel density(WMD=-4.09,95%CI:-7.67,-0.52)was significantly lower than the control,while mean foveal SVD(WMD=-1.71,95%CI:-4.65,1.24),mean foveal avascular zone(FAZ)area(WMD=0.04,95%CI:-0.01,0.09)and mean acircularity index(AI;WMD=0.00,95%CI:-0.02,0.02)were not different between SLE patients and controls.Subgroup analysis indicated that the heterogeneity in SVD was partially due to the scanning area.Ocellus or binoculus data contributed partially to the heterogeneity in parafoveal deep vessel density and FAZ area.Sensitivity analysis indicated that the results were robust after changing the analysis model except for foveal SVD and FAZ area.There was no bias in included studies except whole SVD.CONCLUSION:Parafoveal superficial and deep vessel density are significantly lower in SLE patients while FAZ area and AI are not different between SLE patients and the control.展开更多
INTRODUCTION Cardiovascular system involvement is an important determinant of long-term prognosis in patients with systemic lupus erythematosus(SLE).Aneurysmal dilatation of the aortic root combined with Stanford type...INTRODUCTION Cardiovascular system involvement is an important determinant of long-term prognosis in patients with systemic lupus erythematosus(SLE).Aneurysmal dilatation of the aortic root combined with Stanford type A aortic dissection(TAAD)is a highly catastrophic complication in these patients.展开更多
AIM:To evaluate the choroidopathy in patients with systemic lupus erythematosus(SLE)using enhanced depth imaging spectral domain optical coherence tomography(EDI SD-OCT)and optical coherence tomography angiography(OCT...AIM:To evaluate the choroidopathy in patients with systemic lupus erythematosus(SLE)using enhanced depth imaging spectral domain optical coherence tomography(EDI SD-OCT)and optical coherence tomography angiography(OCTA).METHODS:A total of 74 patients with SLE and 40 healthy volunteers were included in this cross-sectional study.SLE patients were further divided into three subgroups based on clinical and blood biochemistry findings.Ocular parameters obtained on ophthalmologic examination and optical imaging(EDI SD-OCT and OCTA)included the best corrected distance visual acuity(logMAR CDVA),subfoveal choroidal thickness(SCT),choroidal vascularity index(CVI)and vessel density(VD)of superficial capillary plexus(SCP)and deep capillary plexus(DCP).RESULTS:SLE patients had significantly lower values for CVI and VD of DCP(DVD)than control subjects.Amongst SLE patients,gender and chloroquine dose were found to be independent determinants of CVI while age predicted SCT.Steroid dose was a significant predictor for foveal VD of SCP(SVD),chloroquine dose for parafoveal SVD,gender for total DVD,and gender and steroid dose for perifoveal DVD.No correlation of logMAR CDVA and SCT was noted between SLE patients and control subjects.No correlation of SCT was noted with disease duration,Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score,hydroxychloroquine(HCQ)dose or steroid dose.No correlation of CVI was noted with patient age,disease duration,SLEDAI score,HCQ dose or steroid dose.No significant difference was noted between SLE subgroups in terms of any of the ocular parameters studied.CONCLUSION:The findings reveal the presence of ocular findings suggestive of early onset choroidopathy on EDI SD-OCT and OCTA in SLE patients,in the absence of ocular manifestations or active disease.展开更多
In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style...In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> 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.展开更多
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 eryt...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.展开更多
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 a...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.展开更多
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by ...Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by pro-tein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroen-teropathies. Most of these complications have good therapeutic responses to corticosteroids and immu-nosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and proki-netic medications are helpful in facilitating functional recovery and improving the outcome.展开更多
Objective:To review the efficacy and safety of rituximab therapy for systemic lupus erythematosus(SLE).Methods:We searched for randomized controlled trails and observational studies that evaluated the effect of rituxi...Objective:To review the efficacy and safety of rituximab therapy for systemic lupus erythematosus(SLE).Methods:We searched for randomized controlled trails and observational studies that evaluated the effect of rituximab based on the systemic lupus erythematosus disease activity index(SLEDAI),British Isles lupus assessment group index(BILAG),urine protein levels,and the prednisolone dose,and had adequate data to calculate the mean,standard deviation(SD),and 95% confidence intervals,and to systematically review and meta-analyze observational studies with fixed effects model or random effects model.Results:We included 2 randomized controlled studies and 19 observational clinical studies.We summarized the data from the 19 observational studies,analyzed the heterogeneity of the literature,and then used fixed effect model or random effect model for statistical analysis.The SLEDAI,BILAG,and urine protein levels and the prednisolone dosage were decreased after rituximab treatment,and the decreases in the BILAG,urine protein levels,and the prednisolone dose were found to be significant(P<0.05),when compared with baseline level.Rituximab's adverse effects generally could be controlled with an effective dosing regimen.Conclusions:Although there are still controversies about rituximab's treatment on SLE,but our study had showed that rituximab had favorable effects on refractory lupus.The long-term efficacy and safety of rituximab require further study.展开更多
BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated ...BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.展开更多
The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique...The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique to investigate the immunophenotyping expression of lymphocytes T and B in the peripheral blood of active SLE patients and its clinical value. It was showed that, compared with normal controls, the expression of CD + 3, CD + 4 and the ratio of CD + 4/CD + 8 in the peripheral blood of these patients were decreased , while the expression of CD + 8, CD + 20 was significantly increased . It was suggested that both T and B cells in patients with active SLE involved in immunoregulation, were activated. The abnormal expression of lymphocyte immunophenotyping could influence the immune reaction in SLE patients, which might be one of the important pathogenesis factors in SLE.展开更多
I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus (SLE). In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, b...I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus (SLE). In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, but is rarely confirmed histologically. We described a case of a 32-year-old man with increased activity of SLE, who presented with hematochezia and abdominal pain due to ischemic colitis with small vessel vasculitis which was proven by sigmoidoscopic biopsy. The clinical course of the patient was improved after steroid and conservative management.展开更多
CD4＋CD25＋ regulatory T cells （Tregs） and the expression of their molecular markers （GITR, Foxp3） in peripheral blood of the patients with systemic lupus erythematosus （SLE） were investigated in order to reveal...CD4＋CD25＋ regulatory T cells （Tregs） and the expression of their molecular markers （GITR, Foxp3） in peripheral blood of the patients with systemic lupus erythematosus （SLE） were investigated in order to reveal the pathogenesis of SLE on the cellular and molecular levels. The level of Tregs in peripheral blood was detected by flow cytometry. The expression levels of GITR and Foxp3 mRNA in peripheral blood mononuclear cells （PBMCs） were assayed by reverse transcriptase-polymerase chain reaction （RT-PCR）. The level of IL-6 in the plasma was measured by ELISA. Comparisons were made among 3 groups： the active SLE group, the inactive SLE group, and normal control group. The level of Tregs in the active SLE group and the inactive SLE group was significantly lower than in the normal control group （P〈0.01）. The level of Tregs in the active group was lower than in the inactive group with the difference being not significant （P〉0.05）. The level of Tregs in SLE patients was significantly negatively correlated with the disease active index in SLE （SLEDAI） （r=-0.81, P〈0.01）. The expression levels of GITR mRNA in PBMCs of the active SLE group and the inactive SLE group were significantly higher than in the normal control group （P〈0.05）, and those of Foxp3 mRNA in SLE patients of both active and inactive SLE groups were significantly lower than in the normal control group （P〈0.05）. There was no significant difference in the expression of GITR and Foxp3 mRNA between the active SLE group and inactive SLE group （P〉0.05）. The plasma levels of IL-6 in both the inactive SLE group and active SLE group were significantly higher than in the normal control group （P〈0.01）. The plasma level of IL-6 in the active SLE group was sig- nificantly increased as compared with that in the inactive SLE group （P〈0.05）, and the plasma level of IL-6 in SLE was significantly positively correlated with SLEDAI scores （r=0.58, P〈0.01） and significantly negatively correlated with the ratio of CD4＋CD25＋ cells/CD4＋ cells （r=-0.389, P〈0.05）. It was concluded that the levels of Tregs and Foxp3 mRNA in peripheral blood of SLE patients were decreased and the levels of GITR mRNA and plasma IL-6 were increased. The Tregs and their molecular markers GITR, Foxp3 as well as the plasma IL-6 might play an important role in the pathogenesis of SLE.展开更多
Systemic lupus erythematosus(SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows:(1) immunological comorbilities(overlap syndromes);(2) non-immunological comorbilities associ...Systemic lupus erythematosus(SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows:(1) immunological comorbilities(overlap syndromes);(2) non-immunological comorbilities associated to SLE; and(3) a putative liver damage induced by SLE itself, referred to as "lupus hepatitis". In the first group, liver injury can be ascribed to overlapping hepatopathies triggered by autoimmune mechanisms other than SLE occurring with higher incidence in the context of lupus(e.g., autoimmune hepatitis, primary biliary cirrhosis). The second group includes non-autoimmune liver diseases, such as esteatosis, hepatitis C, hypercoagulation state-related liver lesions, hyperplasic parenchymal and vascular lesions, porphyria cutanea tarda, and drug-induced hepatotoxicity. Finally, the data in the literature to support the existence of a hepatic disease produced by SLE itself, or the occurrence of a SLE-associated prone condition that increases susceptibility to acquire other liver diseases, is critically discussed. The pathological mechanisms underlying each of these liver disorders are also reviewed. Despite the high heterogeneity in the literature regarding the prevalence of SLE-associated liver diseases and, in most cases, lack of histopathological evidence or clinical studies large enough to support their existence, it is becoming increasingly apparent that liver is an important target of SLE. Consequently, biochemical liver tests should be routinely carried out in SLE patients to discard liver disorders, particularly in those patients chronically exposed to potentially hepatotoxic drugs. Diagnosing liver disease in SLE patients is always challenging, and the systematization of the current information carried out in this review is expected to be of help both to attain a better understanding of pathogenesis and to build an appropriate work-up for diagnosis.展开更多
A 48 year-old Chinese woman suffering from polyarthritis,irregular fever and trichomadesis was admitted to the hospital.A diagnosis of systemic lupus erythematosus(SLE)was made based on polyarthritis,pancytopenia,redu...A 48 year-old Chinese woman suffering from polyarthritis,irregular fever and trichomadesis was admitted to the hospital.A diagnosis of systemic lupus erythematosus(SLE)was made based on polyarthritis,pancytopenia,reduced complement 3,multiple positive autoantibodies,a positive Coomb’s test and protein in her urine.In addition,splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging,indicating that the patient had SLE and portal hypertension.Further negative investigations ruled out the possibility of cirrhosis.The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension(NCPH)without liver histopathology,due to the patient’s refusal for liver biopsy.Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone,hydroxychloroquine and metoprolol tartrate.To date,SLE complicated by NCPH has rarely been reported,as it is under-recognized clinically as well as pathologically.Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics,which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.展开更多
The role played by cytokines, other than interferon （IFN）-a, in the differentiation and function of dendritic cells （DCs） in systemic lupus erythematosus （SLE）, remains unclear. Serum interleukin-10 （IL-10） le...The role played by cytokines, other than interferon （IFN）-a, in the differentiation and function of dendritic cells （DCs） in systemic lupus erythematosus （SLE）, remains unclear. Serum interleukin-10 （IL-10） levels are generally elevated in SLE patients, which might modulate the differentiation of DCs. In this study, DCs were induced from monocytes either by transendothelial trafficking or by culture with granulocyte-macrophage colony-stimulating factor （GM-CSF） ＋ IL-4 ＋ tumor necrosis factor （TNF）-a. Both systems were used to investigate the effects of elevated serum IL-10 level on DC differentiation in SLE patients. The results showed that monocyte-derived DCs induced by either SLE serum or exogenous IL-10 reduced the expression of human leukocyte antigen （HLA）-DR and CD80, decreased IL-12p40 level, and increased IL-10 level, and exhibited an impaired capacity to stimulate allogenic T-cell proliferation. These results indicate that serum IL-10 may be involved in the pathogenesis of SLE by modulating the differentiation and function of DCs.展开更多
基金supported by the National Natural Science Foundation Joint Fund,No.U22A20309(to PY)the Natural Science Foundation of LiaoningProvince,No.2023-MS-07(to HuL)the Unveiling Key Scientific and Technological Projects of Liaoning Province,No.2021JH1/10400051(to HuL).
文摘Some patients with systemic lupus erythematosus experience neuropsychiatric symptoms.Although magnetic resonance imaging can detect abnormal signals in the white matter of the brain,conventional methods often struggle to accurately capture microstructural changes.Various diffusion models have been used to study white matter in systemic lupus erythematosus;however,comparative analyses of their sensitivity and specificity for detecting microstructural changes remain insufficient.To address this,our team designed a diagnostic trial that used multimodal diffusion imaging techniques to observe white matter microstructural changes in patients with systemic lupus erythematosus who had neuropsychiatric symptoms,with an aim to identify key diagnostic biomarkers for these patients.Patients with active lupus who received treatment at the Department of Rheumatology and Immunology,The First Affiliated Hospital of China Medical University,from September 2023 to March 2024 were recruited.According to the standards of the American College of Rheumatology,patients with systemic lupus erythematosus who had neuropsychiatric symptoms were assigned to the systemic lupus erythematosus group,whereas those without neuropsychiatric symptoms were assigned to the non-systemic lupus erythematosus group.Additionally,healthy volunteers matched by region,sex,and age were recruited as controls.All three groups underwent the same diffusion magnetic resonance imaging examination protocol to compare differences in diffusion parameters.Advanced diffusion imaging models were able to sensitively detect microstructural changes in the white matter fibers of patients with systemic lupus erythematosus who had neuropsychiatric symptoms,with specific diffusion parameters showing significant abnormalities in key brain regions.In the left superior longitudinal fasciculus subregion and the right thalamic radiations of patients with systemic lupus erythematosus who had neuropsychiatric symptoms,we also identified abnormal diffusion characteristics that were clearly correlated with disease activity,suggesting that microstructural changes in these areas may reflect the dynamic process of neuroinflammatory damage.The present study addresses critical challenges in the diagnosis of systemic lupus erythematosus by identifying specific white matter imaging biomarkers and elucidating the association between microstructural damage and clinical manifestations.The main contributions of our study include:1)establishing axial regression probability parameters from mean apparent propagator magnetic resonance imaging as sensitive biomarkers for systemic lupus erythematosus,particularly in the third subregion of the left superior longitudinal fasciculus;2)demonstrating that multimodal diffusion imaging may be superior to conventional diffusion tensor imaging for detecting white matter microstructural abnormalities in patients with systemic lupus erythematosus;and 3)integrating tract-based spatial statistics with clinically relevant analyses to link imaging findings to pathological mechanisms.
基金Supported by STI2030-Major Projects,No.2021ZD0202001National Natural Science Foundation of China,No.T2341003Capital Funds for Health Improvement and Research,No.CFH 2022-2-4012.
文摘BACKGROUND Not all neuropsychiatric(NP)manifestations in patients with systemic lupus erythematosus(SLE)are secondary to lupus.The clarification of the cause of NP symptoms influences therapeutic strategies for SLE.AIM To understand the attribution of psychiatric manifestations in a cohort of Chinese patients with SLE.METHODS This retrospective single-center study analyzed 160 inpatient medical records.Clinical diagnosis,which is considered the gold standard,was used to divide the subjects into a psychiatric SLE(PSLE)group(G1)and a secondary psychiatric symptoms group(G2).Clinical features were compared between these two groups.The sensitivity and specificity of the Italian attribution model were explored.RESULTS A total of 171 psychiatric syndromes were recorded in 138 patients,including 87 cases of acute confusional state,40 cases of cognitive dysfunction,18 cases of psychosis,and 13 cases each of depressive disorder and mania or hypomania.A total of 141(82.5%)syndromes were attributed to SLE.In contrast to G2 patients,G1 patients had higher SLE Disease Activity Index-2000 scores(21 vs 12,P=0.001),a lower prevalence of anti-beta-2-glycoprotein 1 antibodies(8.6%vs 25.9%,P=0.036),and a higher prevalence of anti-ribosomal ribonucleoprotein particle(rRNP)antibodies(39.0%vs 22.2%,P=0.045).The Italian attribution model exhibited a sensitivity of 95.0%and a specificity of 70.0%when the threshold value was set at 7.CONCLUSION Patients with PSLE exhibited increased disease activity.There is a correlation between PSLE and anti-rRNP antibodies.The Italian model effectively assesses multiple psychiatric manifestations in Chinese SLE patients who present with NP symptoms.
基金funded by National Natural Science Foundation of China to Ping Yang with Grant number No.82202600by Nanjing Drum Tower Hospital to Ping Yang with Grant number No.2024-LCYJ-MS-11then to Shou-bin Zhan with Grant number No.2023-JCYJ-QP-25.
文摘Objective This study aims to investigate the exosome-derived metabolomics profiles in systemic lupus erythematosus(SLE),identify differential metabolites,and analyze their potential as diagnostic markers for SLE and lupus nephritis(LN).Methods Totally,91 participants were enrolled between February 2023 and January 2024 including 58 SLE patients[30 with nonrenal-SLE and 28 with Lupus nephritis(LN)]and 33 healthy controls(HC).Ultracentrifugation was used to isolate serum exosomes,which were analyzed for their metabolic profiles using liquid chromatography–tandem mass spectrometry(LC–MS/MS).Endogenous metabolites were identified via public metabolite databases.Random Forest,Lasso regression and Support Vector Machine Recursive Feature Elimination(SVM-RFE)algorithms were employed to screen key metabolites,and a prediction model was constructed for SLE diagnosis and LN discrimination.ROC curves were constructed to determine the potential of these differential exosome-derived metabolites for the diagnosis of SLE.Furthermore,Spearman’s correlation was employed to evaluate the potential links between exosome-derived metabolites and the clinical parameters which reflect disease progression.Results A total of 586 endogenous serum exosome-derived metabolites showed differential expression,with 225 exosome-derived metabolites significantly upregulated,88 downregulated and 273 exhibiting no notable changes in the HC and SLE groups.Machine learning algorithms revealed three differential metabolites:Pro-Asn-Gln-Met-Ser,C24:1 sphingolipid,and protoporphyrin IX,which exhibited AUC values of 0.998,0.992 and 0.969 respectively,for distinguishing between the SLE and HC groups,with a combined AUC of 1.0.In distinguishing between the LN and SLE groups,the AUC values for these metabolites were 0.920,0.893 and 0.865,respectively,with a combined AUC of 0.931,demonstrating excellent diagnostic performance.Spearman correlation analysis revealed that Pro-Asn-Gln-Met-Ser and protoporphyrin IX were positively correlated with the SLE Disease Activity Index(SLEDAI)scores,urinary protein/creatinine ratio(ACR)and urinary protein levels,while C24:1 sphingolipid exhibited a negative correlation.Conclusions This study provides the first comprehensive characterization of the exosome-derived metabolites in SLE and established a promising prediction model for SLE and LN discrimination.The correlation between exosome-derived metabolites and key clinical parameters strongly indicated their potential role in SLE pathological progression.
文摘BACKGROUND Systemic lupus erythematosus(SLE)patients are admitted to the intensive care unit(ICU)for disease flares and infections,both of which carry a high mortality risk.Studies characterizing the outcome predictors of SLE are few in the Asian continent.This study characterized the clinical profile,treatment,and outcome predictors of ICU admissions with SLE.AIM To ascertain the reasons for ICU admission among SLE patients and to explore outcome predictors in these patients.The primary outcome was ICU mortality.Secondary outcomes included need for ventilation,inotropes,renal replacement therapy,and length of ICU and hospital stay.METHODS A retrospective study of 77 SLE patients was conducted in the medical ICU of a tertiary care teaching hospital in India.Clinical features,treatment,and outcomes of patients admitted between January 2018 and December 2022 were recorded.Factors associated with mortality were explored using bivariate and multivariate logistic regression analysis and reported as adjusted odds ratios with 95%confidence intervals.RESULTS The mean(SD)age was 31.1(10.3)years;83.1%were female.The median(interquartile)duration of SLE before admission was 12(1-60)months;SLE was newly diagnosed in the current admission in 23.4%.The median Acute Physiology and Chronic Health Evaluation II score was 16.3(14.5-18.2)and similar among survivors and nonsurvivors;32 had evidence of disease flare,44 had an infection,and one patient had an intracranial bleed.ICU admission was for respiratory failure(46.7%),hemodynamic instability(32.5%),and status epilepticus(14.3%).Twenty-nine patients(37.7%)had autoimmune hemolytic anemia,and 11(14.3%)had diffuse alveolar hemorrhage.Immunomodulation included corticosteroids(96.1%),cyclophosphamide(33.8%),mycophenolate(23.4%),plasma exchange(13%),and immunoglobulins(11.7%).All patients received broad-spectrum antibiotics.Respiratory support,inotropes,and renal replacement therapy were required in 93.5%,51.7%,and 32.5%,respectively.ICU mortality was 50.7%(95%confidence interval:39%-62%).The mean±SD hospital length of stay was 18.9±14.3 days.On multivariate analysis,only shock(P=0.004)was independently associated with mortality.CONCLUSION Intercurrent infection and disease flare are common reasons for ICU admission in SLE patients.Despite multimodal therapy,mortality is high.Shock was independently associated with mortality.
基金Supported by STI2030-Major Projects,No.2021ZD0202001Capital Funds for Health Improvement and Research,No.CFH 2022-2-4012.
文摘BACKGROUND Systemic lupus erythematosus(SLE)can affect multiple organs or systems.The involvement of the central nervous system can result in the manifestation of epilepsy,an acute confusional state,and other rare neuropsychiatric presentations,such as catatonia.CASE SUMMARY We present a case of an adolescent male patient with first-onset SLE who presented with neuropsychiatric symptoms including epilepsy and delirium.The initial utilization of olanzapine to alleviate symptoms of agitation precipitated the emergence of catatonia,which was mitigated by discontinuing olanzapine and supplementing with lorazepam.In this case,whether the catatonia was secondary to the utilization of antipsychotics or to an organic disease is a question that warrants differential diagnosis.CONCLUSION Multidisciplinary collaborative management is the cornerstone for the successful management of severe cases of SLE.
文摘AIM:To summarize and quantitatively evaluate vasculature alteration of foveal zone in systemic lupus erythematosus(SLE)patients by secondary literature analysis.METHODS:A systematic search of PubMed,Embase,Web of Science,Cochrane Library,CBM,CNKI WanFang Data and VIP was conducted.Studies were about retinal vessel density in SLE patients from January 2000 to April 2023 and valid data were extracted.The Joanna Briggs Institute(JBI)critical appraisal checklist was used to evaluate the cross-sectional studies and prospective studies.The measurement data for combined effect size were weighted mean difference(WMD)and 95%confidence interval(CI).The heterogeneity was evaluated by I2 test.The fixed-effect model was adopted when P>0.1 or I2<50%,and random-effect model was adopted in the contrary.Subgroup and sensitivity analysis were utilized to analyze the sources of heterogeneity.The publication bias was evaluated by Egger tests and funnel plots.RESULTS:A total of 14 studies with 445 subjects and 441 healthy controls from 9 countries were enrolled and 11 studies were included in Meta-analysis.The JBI scores of studies were no less than 14 points.The Metaanalysis results indicated that mean parafoveal superficial vessel density(SVD;WMD=-1.22,95%CI:-1.67,-0.76),mean perifoveal SVD(WMD=-1.42,95%CI:-1.95,-0.89),mean whole SVD(WMD=-1.66,95%CI:-2.53,-0.79),mean parafoveal deep vessel density(WMD=-1.67,95%CI:-2.75,-0.59)and mean whole deep vessel density(WMD=-4.09,95%CI:-7.67,-0.52)was significantly lower than the control,while mean foveal SVD(WMD=-1.71,95%CI:-4.65,1.24),mean foveal avascular zone(FAZ)area(WMD=0.04,95%CI:-0.01,0.09)and mean acircularity index(AI;WMD=0.00,95%CI:-0.02,0.02)were not different between SLE patients and controls.Subgroup analysis indicated that the heterogeneity in SVD was partially due to the scanning area.Ocellus or binoculus data contributed partially to the heterogeneity in parafoveal deep vessel density and FAZ area.Sensitivity analysis indicated that the results were robust after changing the analysis model except for foveal SVD and FAZ area.There was no bias in included studies except whole SVD.CONCLUSION:Parafoveal superficial and deep vessel density are significantly lower in SLE patients while FAZ area and AI are not different between SLE patients and the control.
文摘INTRODUCTION Cardiovascular system involvement is an important determinant of long-term prognosis in patients with systemic lupus erythematosus(SLE).Aneurysmal dilatation of the aortic root combined with Stanford type A aortic dissection(TAAD)is a highly catastrophic complication in these patients.
文摘AIM:To evaluate the choroidopathy in patients with systemic lupus erythematosus(SLE)using enhanced depth imaging spectral domain optical coherence tomography(EDI SD-OCT)and optical coherence tomography angiography(OCTA).METHODS:A total of 74 patients with SLE and 40 healthy volunteers were included in this cross-sectional study.SLE patients were further divided into three subgroups based on clinical and blood biochemistry findings.Ocular parameters obtained on ophthalmologic examination and optical imaging(EDI SD-OCT and OCTA)included the best corrected distance visual acuity(logMAR CDVA),subfoveal choroidal thickness(SCT),choroidal vascularity index(CVI)and vessel density(VD)of superficial capillary plexus(SCP)and deep capillary plexus(DCP).RESULTS:SLE patients had significantly lower values for CVI and VD of DCP(DVD)than control subjects.Amongst SLE patients,gender and chloroquine dose were found to be independent determinants of CVI while age predicted SCT.Steroid dose was a significant predictor for foveal VD of SCP(SVD),chloroquine dose for parafoveal SVD,gender for total DVD,and gender and steroid dose for perifoveal DVD.No correlation of logMAR CDVA and SCT was noted between SLE patients and control subjects.No correlation of SCT was noted with disease duration,Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score,hydroxychloroquine(HCQ)dose or steroid dose.No correlation of CVI was noted with patient age,disease duration,SLEDAI score,HCQ dose or steroid dose.No significant difference was noted between SLE subgroups in terms of any of the ocular parameters studied.CONCLUSION:The findings reveal the presence of ocular findings suggestive of early onset choroidopathy on EDI SD-OCT and OCTA in SLE patients,in the absence of ocular manifestations or active disease.
文摘In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> 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.
文摘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.
文摘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.
文摘Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by pro-tein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroen-teropathies. Most of these complications have good therapeutic responses to corticosteroids and immu-nosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and proki-netic medications are helpful in facilitating functional recovery and improving the outcome.
文摘Objective:To review the efficacy and safety of rituximab therapy for systemic lupus erythematosus(SLE).Methods:We searched for randomized controlled trails and observational studies that evaluated the effect of rituximab based on the systemic lupus erythematosus disease activity index(SLEDAI),British Isles lupus assessment group index(BILAG),urine protein levels,and the prednisolone dose,and had adequate data to calculate the mean,standard deviation(SD),and 95% confidence intervals,and to systematically review and meta-analyze observational studies with fixed effects model or random effects model.Results:We included 2 randomized controlled studies and 19 observational clinical studies.We summarized the data from the 19 observational studies,analyzed the heterogeneity of the literature,and then used fixed effect model or random effect model for statistical analysis.The SLEDAI,BILAG,and urine protein levels and the prednisolone dosage were decreased after rituximab treatment,and the decreases in the BILAG,urine protein levels,and the prednisolone dose were found to be significant(P<0.05),when compared with baseline level.Rituximab's adverse effects generally could be controlled with an effective dosing regimen.Conclusions:Although there are still controversies about rituximab's treatment on SLE,but our study had showed that rituximab had favorable effects on refractory lupus.The long-term efficacy and safety of rituximab require further study.
文摘BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.
文摘The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique to investigate the immunophenotyping expression of lymphocytes T and B in the peripheral blood of active SLE patients and its clinical value. It was showed that, compared with normal controls, the expression of CD + 3, CD + 4 and the ratio of CD + 4/CD + 8 in the peripheral blood of these patients were decreased , while the expression of CD + 8, CD + 20 was significantly increased . It was suggested that both T and B cells in patients with active SLE involved in immunoregulation, were activated. The abnormal expression of lymphocyte immunophenotyping could influence the immune reaction in SLE patients, which might be one of the important pathogenesis factors in SLE.
文摘I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus (SLE). In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, but is rarely confirmed histologically. We described a case of a 32-year-old man with increased activity of SLE, who presented with hematochezia and abdominal pain due to ischemic colitis with small vessel vasculitis which was proven by sigmoidoscopic biopsy. The clinical course of the patient was improved after steroid and conservative management.
基金a grant from the Natural Sciences Foundation of Hubei, China (No. 2007ABA110).
文摘CD4＋CD25＋ regulatory T cells （Tregs） and the expression of their molecular markers （GITR, Foxp3） in peripheral blood of the patients with systemic lupus erythematosus （SLE） were investigated in order to reveal the pathogenesis of SLE on the cellular and molecular levels. The level of Tregs in peripheral blood was detected by flow cytometry. The expression levels of GITR and Foxp3 mRNA in peripheral blood mononuclear cells （PBMCs） were assayed by reverse transcriptase-polymerase chain reaction （RT-PCR）. The level of IL-6 in the plasma was measured by ELISA. Comparisons were made among 3 groups： the active SLE group, the inactive SLE group, and normal control group. The level of Tregs in the active SLE group and the inactive SLE group was significantly lower than in the normal control group （P〈0.01）. The level of Tregs in the active group was lower than in the inactive group with the difference being not significant （P〉0.05）. The level of Tregs in SLE patients was significantly negatively correlated with the disease active index in SLE （SLEDAI） （r=-0.81, P〈0.01）. The expression levels of GITR mRNA in PBMCs of the active SLE group and the inactive SLE group were significantly higher than in the normal control group （P〈0.05）, and those of Foxp3 mRNA in SLE patients of both active and inactive SLE groups were significantly lower than in the normal control group （P〈0.05）. There was no significant difference in the expression of GITR and Foxp3 mRNA between the active SLE group and inactive SLE group （P〉0.05）. The plasma levels of IL-6 in both the inactive SLE group and active SLE group were significantly higher than in the normal control group （P〈0.01）. The plasma level of IL-6 in the active SLE group was sig- nificantly increased as compared with that in the inactive SLE group （P〈0.05）, and the plasma level of IL-6 in SLE was significantly positively correlated with SLEDAI scores （r=0.58, P〈0.01） and significantly negatively correlated with the ratio of CD4＋CD25＋ cells/CD4＋ cells （r=-0.389, P〈0.05）. It was concluded that the levels of Tregs and Foxp3 mRNA in peripheral blood of SLE patients were decreased and the levels of GITR mRNA and plasma IL-6 were increased. The Tregs and their molecular markers GITR, Foxp3 as well as the plasma IL-6 might play an important role in the pathogenesis of SLE.
文摘Systemic lupus erythematosus(SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows:(1) immunological comorbilities(overlap syndromes);(2) non-immunological comorbilities associated to SLE; and(3) a putative liver damage induced by SLE itself, referred to as "lupus hepatitis". In the first group, liver injury can be ascribed to overlapping hepatopathies triggered by autoimmune mechanisms other than SLE occurring with higher incidence in the context of lupus(e.g., autoimmune hepatitis, primary biliary cirrhosis). The second group includes non-autoimmune liver diseases, such as esteatosis, hepatitis C, hypercoagulation state-related liver lesions, hyperplasic parenchymal and vascular lesions, porphyria cutanea tarda, and drug-induced hepatotoxicity. Finally, the data in the literature to support the existence of a hepatic disease produced by SLE itself, or the occurrence of a SLE-associated prone condition that increases susceptibility to acquire other liver diseases, is critically discussed. The pathological mechanisms underlying each of these liver disorders are also reviewed. Despite the high heterogeneity in the literature regarding the prevalence of SLE-associated liver diseases and, in most cases, lack of histopathological evidence or clinical studies large enough to support their existence, it is becoming increasingly apparent that liver is an important target of SLE. Consequently, biochemical liver tests should be routinely carried out in SLE patients to discard liver disorders, particularly in those patients chronically exposed to potentially hepatotoxic drugs. Diagnosing liver disease in SLE patients is always challenging, and the systematization of the current information carried out in this review is expected to be of help both to attain a better understanding of pathogenesis and to build an appropriate work-up for diagnosis.
基金Supported by the National Natural Science Foundation of China,No.81670801Medical Association of Sichuan Province,No.S16027+1 种基金Health and Family Planning Commission of Sichuan Province,No.17PJ059Science and Technology Department of Sichuan Province,No.2018JY0498
文摘A 48 year-old Chinese woman suffering from polyarthritis,irregular fever and trichomadesis was admitted to the hospital.A diagnosis of systemic lupus erythematosus(SLE)was made based on polyarthritis,pancytopenia,reduced complement 3,multiple positive autoantibodies,a positive Coomb’s test and protein in her urine.In addition,splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging,indicating that the patient had SLE and portal hypertension.Further negative investigations ruled out the possibility of cirrhosis.The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension(NCPH)without liver histopathology,due to the patient’s refusal for liver biopsy.Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone,hydroxychloroquine and metoprolol tartrate.To date,SLE complicated by NCPH has rarely been reported,as it is under-recognized clinically as well as pathologically.Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics,which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.
基金supported by grants from Science Research Foundation of Ministry of Education of China (No. 205057)Foundation of Jiangsu Province Natural Science (No. 2004148)
文摘The role played by cytokines, other than interferon （IFN）-a, in the differentiation and function of dendritic cells （DCs） in systemic lupus erythematosus （SLE）, remains unclear. Serum interleukin-10 （IL-10） levels are generally elevated in SLE patients, which might modulate the differentiation of DCs. In this study, DCs were induced from monocytes either by transendothelial trafficking or by culture with granulocyte-macrophage colony-stimulating factor （GM-CSF） ＋ IL-4 ＋ tumor necrosis factor （TNF）-a. Both systems were used to investigate the effects of elevated serum IL-10 level on DC differentiation in SLE patients. The results showed that monocyte-derived DCs induced by either SLE serum or exogenous IL-10 reduced the expression of human leukocyte antigen （HLA）-DR and CD80, decreased IL-12p40 level, and increased IL-10 level, and exhibited an impaired capacity to stimulate allogenic T-cell proliferation. These results indicate that serum IL-10 may be involved in the pathogenesis of SLE by modulating the differentiation and function of DCs.
