Down’s syndrome(DS,Trisomy 21)with a prevalence of 1:8,000 live births is considered the most common genetic chromosomal disorder,with an extra full or partial copy of chromosome 21.In addition to physical and mental...Down’s syndrome(DS,Trisomy 21)with a prevalence of 1:8,000 live births is considered the most common genetic chromosomal disorder,with an extra full or partial copy of chromosome 21.In addition to physical and mental developmental delays and disabilities being a challenge to this disorder,vulnerability of DS patients to a variety of autoimmune diseases like diabetes,thyroid disorders and celiac disease is also well established suggesting impaired immune response especially cell mediated immunity.In the last 3 decades,a few cases of Systemic Lupus Erythematosis(SLE)have been identified and reported internationally,this case report adds to this rare association and also endorses the need to carefully evaluate and investigate the DS individuals for the presence of connective tissue disorders especially if there is already an existing autoimmune disorder like diabetes,celiac or thyroid disorder.展开更多
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)an...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.展开更多
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 re...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.展开更多
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 psy...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.展开更多
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 unk...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.展开更多
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 ...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.展开更多
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 patien...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.展开更多
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 rep...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.展开更多
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 o...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.展开更多
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,...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.展开更多
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^...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.展开更多
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 describ...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.展开更多
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 biomar...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.展开更多
Introduction: Systemic lupus erythematosus (SLE) is a non-organ-specific autoimmune disease with an unknown origin. The unchanged trend of premature mortality in systemic lupus erythematosus shows the critical unmet n...Introduction: Systemic lupus erythematosus (SLE) is a non-organ-specific autoimmune disease with an unknown origin. The unchanged trend of premature mortality in systemic lupus erythematosus shows the critical unmet need for improved and optimized management of systemic lupus erythematosus. Objectives: To analyze the clinical features and the prognostic factors of death in SLE at the CNHU-HKM of Cotonou. Patients and Methods: This was a retrospective cohort study. It was conducted over the period from January 1st 2010 to December 31st 2021. The study population consisted of all patients followed for SLE in the internal medicine, rheumatology, nephrology and dermatology wards at the CNHU-HKM of Cotonou. Results: 88 cases were recorded in 12 years, i.e. an incidence of 7 cases per year. There were 80 women and 8 men with a mean age of 36.4 ± 13.1 years. The clinical picture was dominated by mucocutaneous (86.3%) and osteoarticular (71.5%) disorders. The biological abnormalities observed were anemia (78.8%), lymphopenia (43.1%) and thrombocytopenia (17.7%). 39 patients had renal damage (44.3%). The incidence of death was 17%. Factors associated with death were renal involvement, infectious complications, high initial SLEDAI score, flare, thrombocytopenia and lymphopenia. Conclusion: Mortality related to SLE remains high at the CNHU-HKM of Cotonou. Renal involvement, infectious complications, high initial SLEDAI score, flare, lymphopenia and thrombocytopenia were the factors associated with death. The presence of these factors should lead to an evaluation of the treatment.展开更多
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 ass...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.展开更多
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 ...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.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
BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active ...BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active stage of systemic lupuserythematosus (ASLE), although rare and complicated to manage, can be treatedfavorably with immunotherapies ifs used properly. Here we report such a successcase.CASE SUMMARY A 31-year-old primigravida patient, diagnosed with SLE seven years ago, wasinduced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentationwere normal. Her laboratory exam was remarkable for significant proteinuria,liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up wasnegative. The patient was diagnosed with ASLE. She was given immunosuppressiveagents (methylprednisolone, gamma globulin and azathioprine etc.)and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heartrate twice a day, and liver and renal function at least twice a week. Successfulmaternal and fetal outcomes are presented here.CONCLUSION Child-bearing in ASLE has become more promising, even for this difficult case ofASLE with multiple organ damage. Thorough antepartum counseling, cautiousmaternal-fetal monitoring, and multi-organ function monitoring bymultidisciplinary specialties are keys to favorable pregnancy outcomes.展开更多
Summary: The role of CD80/CD86 and CTLA-4 in the pathogenesis of systemic lupus erythematosus and their clinical significance was investigated. By using RT-PCR technique, the expression of CD80/CD86 and CTLA-4 mRNA in...Summary: The role of CD80/CD86 and CTLA-4 in the pathogenesis of systemic lupus erythematosus and their clinical significance was investigated. By using RT-PCR technique, the expression of CD80/CD86 and CTLA-4 mRNA in peripheral blood mononuclear cells (PBMC) were semiquantitatively detected in 32 patients with active SLE. The results showed that the percentage of positive CD86 expression in active SLE was increased significantly as compared with normal controls (90.63 % vs 60.00 %, P<0.01). The mean level of CD86 mRNA expression in active SLE group was markedly higher than in the normal controls (0.6410+0.0174 vs 0.4510+0.0402, P<0.001). Compared with normal controls, the percentage of positive CTLA-4 expression and the mean level of CTLA-4 mRNA expression in active SLE group were both increased significantly (both P<0.01). There was no statistical differences in positive rate of CD80 and the average level of CD80 mRNA between the two groups (both P>0.05). It was concluded that the aberrant expression of CD86 and CTLA-4 might play an important role in the activity and development of SLE.展开更多
Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has b...Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has been used to treat SLE, while its mechanism remains to be fully elucidated. In this study, to investigate the expression of LTβR in the T cells of SLE patients and its roles in the pathogenesis of SLE, we isolated the peripheral blood T cells of SLE patients and normal controls to detect expression of LTβR by flow cytometry and RNA assay. T cells were also stimulated with LIGHT, a ligand of LTβR, and then detected for their LTβR expressions and apoptosis by flow cytometry. Also, their expressions of inflammatory factors and receptors were determined by RNA assay. The results showed that LTβR positive cells were 22.75%±6.98% in CD3~+ cells of SLE patients, while there were almost no LTβR positive cells in CD3~+ cells of normal persons. Moreover, LTβR expression was remarkably higher in CD3,CD4 and CD8 positive T cells of active SLE patients than non/low active patients(all P〈0.05), and positively correlated with increased Ig level, decreased complement level and renal damage. Moreover, the stimulation of SLE T cells with LIGHT promoted higher expression of LTβR, IL-23 R and IL-17 A, and apoptosis of T cells. In conclusion,we demonstrated a high expression of LTβR in the T cells of SLE patients which may be associated with pathogenesis of SLE.展开更多
文摘Down’s syndrome(DS,Trisomy 21)with a prevalence of 1:8,000 live births is considered the most common genetic chromosomal disorder,with an extra full or partial copy of chromosome 21.In addition to physical and mental developmental delays and disabilities being a challenge to this disorder,vulnerability of DS patients to a variety of autoimmune diseases like diabetes,thyroid disorders and celiac disease is also well established suggesting impaired immune response especially cell mediated immunity.In the last 3 decades,a few cases of Systemic Lupus Erythematosis(SLE)have been identified and reported internationally,this case report adds to this rare association and also endorses the need to carefully evaluate and investigate the DS individuals for the presence of connective tissue disorders especially if there is already an existing autoimmune disorder like diabetes,celiac or thyroid disorder.
文摘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.
文摘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.
基金Supported by National Natural Science Foundation of China,No.81760840 and No.82160874Natural Science Foundation of Hainan Province,No.2019RC206.
文摘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.
文摘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.
基金Supported by the Ministry of Science and Higher Education of the Russian Federation,No.075-15-2022-301the Russian Science Foundation,No.22-45-08004.
文摘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.
基金Supported by the National Natural Science Foundation of China,No.82271843Key Project supported by Medical Science and Technology Development Foundation,Nanjing Department of Health,No.ZKX20019.
文摘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.
基金This study was conducted with the approval of the ethics committee of Chongqing Hospital of Traditional Chinese Medicine(approval No.2023-GAKY-KS-XYQ).The patient gave explicit informed consent to report her clinical case.
文摘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.
文摘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.
文摘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.
基金supported by the Natural Science Foundation of Sichuan Province (Grant No.2022NSFSC1415)the Special Project of Sichuan Province Traditional Chinese Medicine Administration (Grant No. 2020JC0124)+1 种基金the Management Project of General Hospital of Western Theater Command (Grants No. 2021-XZYG-C22 and 2021-XZYG-C21)the Spark Young Innovative Talent Project of General Hospital of Western Theater Command。
文摘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.
文摘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.
基金the Department of Research and Technology at Golestan University of Medical Sciences,No.113017.
文摘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.
文摘Introduction: Systemic lupus erythematosus (SLE) is a non-organ-specific autoimmune disease with an unknown origin. The unchanged trend of premature mortality in systemic lupus erythematosus shows the critical unmet need for improved and optimized management of systemic lupus erythematosus. Objectives: To analyze the clinical features and the prognostic factors of death in SLE at the CNHU-HKM of Cotonou. Patients and Methods: This was a retrospective cohort study. It was conducted over the period from January 1st 2010 to December 31st 2021. The study population consisted of all patients followed for SLE in the internal medicine, rheumatology, nephrology and dermatology wards at the CNHU-HKM of Cotonou. Results: 88 cases were recorded in 12 years, i.e. an incidence of 7 cases per year. There were 80 women and 8 men with a mean age of 36.4 ± 13.1 years. The clinical picture was dominated by mucocutaneous (86.3%) and osteoarticular (71.5%) disorders. The biological abnormalities observed were anemia (78.8%), lymphopenia (43.1%) and thrombocytopenia (17.7%). 39 patients had renal damage (44.3%). The incidence of death was 17%. Factors associated with death were renal involvement, infectious complications, high initial SLEDAI score, flare, thrombocytopenia and lymphopenia. Conclusion: Mortality related to SLE remains high at the CNHU-HKM of Cotonou. Renal involvement, infectious complications, high initial SLEDAI score, flare, lymphopenia and thrombocytopenia were the factors associated with death. The presence of these factors should lead to an evaluation of the treatment.
文摘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.
文摘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.
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.
文摘BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active stage of systemic lupuserythematosus (ASLE), although rare and complicated to manage, can be treatedfavorably with immunotherapies ifs used properly. Here we report such a successcase.CASE SUMMARY A 31-year-old primigravida patient, diagnosed with SLE seven years ago, wasinduced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentationwere normal. Her laboratory exam was remarkable for significant proteinuria,liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up wasnegative. The patient was diagnosed with ASLE. She was given immunosuppressiveagents (methylprednisolone, gamma globulin and azathioprine etc.)and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heartrate twice a day, and liver and renal function at least twice a week. Successfulmaternal and fetal outcomes are presented here.CONCLUSION Child-bearing in ASLE has become more promising, even for this difficult case ofASLE with multiple organ damage. Thorough antepartum counseling, cautiousmaternal-fetal monitoring, and multi-organ function monitoring bymultidisciplinary specialties are keys to favorable pregnancy outcomes.
文摘Summary: The role of CD80/CD86 and CTLA-4 in the pathogenesis of systemic lupus erythematosus and their clinical significance was investigated. By using RT-PCR technique, the expression of CD80/CD86 and CTLA-4 mRNA in peripheral blood mononuclear cells (PBMC) were semiquantitatively detected in 32 patients with active SLE. The results showed that the percentage of positive CD86 expression in active SLE was increased significantly as compared with normal controls (90.63 % vs 60.00 %, P<0.01). The mean level of CD86 mRNA expression in active SLE group was markedly higher than in the normal controls (0.6410+0.0174 vs 0.4510+0.0402, P<0.001). Compared with normal controls, the percentage of positive CTLA-4 expression and the mean level of CTLA-4 mRNA expression in active SLE group were both increased significantly (both P<0.01). There was no statistical differences in positive rate of CD80 and the average level of CD80 mRNA between the two groups (both P>0.05). It was concluded that the aberrant expression of CD86 and CTLA-4 might play an important role in the activity and development of SLE.
文摘Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has been used to treat SLE, while its mechanism remains to be fully elucidated. In this study, to investigate the expression of LTβR in the T cells of SLE patients and its roles in the pathogenesis of SLE, we isolated the peripheral blood T cells of SLE patients and normal controls to detect expression of LTβR by flow cytometry and RNA assay. T cells were also stimulated with LIGHT, a ligand of LTβR, and then detected for their LTβR expressions and apoptosis by flow cytometry. Also, their expressions of inflammatory factors and receptors were determined by RNA assay. The results showed that LTβR positive cells were 22.75%±6.98% in CD3~+ cells of SLE patients, while there were almost no LTβR positive cells in CD3~+ cells of normal persons. Moreover, LTβR expression was remarkably higher in CD3,CD4 and CD8 positive T cells of active SLE patients than non/low active patients(all P〈0.05), and positively correlated with increased Ig level, decreased complement level and renal damage. Moreover, the stimulation of SLE T cells with LIGHT promoted higher expression of LTβR, IL-23 R and IL-17 A, and apoptosis of T cells. In conclusion,we demonstrated a high expression of LTβR in the T cells of SLE patients which may be associated with pathogenesis of SLE.