BACKGROUND Proliferative lupus nephritis(PLN)is the most severe form of lupus nephritis(LN).There are limited data available on renal outcomes of PLN from developing countries.AIM To determine the clinicopathological ...BACKGROUND Proliferative lupus nephritis(PLN)is the most severe form of lupus nephritis(LN).There are limited data available on renal outcomes of PLN from developing countries.AIM To determine the clinicopathological characteristics and long-term outcomes in terms of remission,requirement of kidney replacement therapy(KRT),and patient survival.METHODS A retrospective analysis was conducted on biopsy-proven focal or diffuse PLN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation and followed up at the renal clinic for a minimum of 5 years.All patients were induced with a combination of intravenous cyclophosphamide and corticosteroids for 6 months,followed by maintenance treatment with azathioprine(AZA)or mycophenolate mofetil(MMF).Data were analyzed using Statistical Package for the Social Sciences,version 22.0.P≤0.05 was considered statistically significant.RESULTS The mean age at the onset of systemic lupus erythematosus was 24.12 years±8.89 years,and at LN onset,26.63 years±8.61 years.There was a female predominance of 184(88.9%)cases.Among baseline characteristics,reduced estimated glomerular filtration rate,presence of hypertension,requirement of KRT,and underlying renal histology(International Society of Nephrology/Renal Pathology Society class IV than class III)were significantly associated with end-stage kidney disease(ESKD)and mortality.The renal outcomes were negatively correlated with age,duration of symptoms,and 24-hour urinary protein excretion.The overall remission rate was 89.8%at the end of induction therapy.At 5 years,141(68.11%)patients were in complete and partial remission(94[45.4%]and 47[22.7%],respectively).In total,19(9.2%)patients required KRT on presentation,and at 5 years,38(18.4%)patients developed ESKD,and 28(13.5%)patients died.Thirty-four(16.4%)patients had a renal relapse,more with AZA than MMF(30[88.2%]vs 4[11.76%],respectively;P=0.04).Renal survival at 6 months was 89.8%,while at 5 years,it was 68.11%,showing a significant improvement in patients who did not need KRT at the time of presentation(P<0.0001).CONCLUSION Baseline renal functions,requirement of KRT,and diffuse proliferative disease were the most relevant prognostic factors for kidney survival among this cohort.Short-term renal outcomes were good.Long-term outcomes were poorer with AZA-based maintenance therapy than with MMF,with more ESKD and mortality.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
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 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
Lupus nephritis(LN)is one of the most common and serious complications of systemic lupus erythematosus,which can lead to end-stage renal disease,and is an important cause of death in patients with systemic lupus eryth...Lupus nephritis(LN)is one of the most common and serious complications of systemic lupus erythematosus,which can lead to end-stage renal disease,and is an important cause of death in patients with systemic lupus erythematosus.Treatment options include glucocorticoids,immunosuppressive agents and the addition of biologics.Recently,the therapeutic role of mesenchymal stem cells(MSCs)in LN has received extensive attention worldwide.MSCs can suppress autoimmunity,alleviate proteinuria and restore renal function by modulating the functions of various immune cells and reducing the secretion of inflammatory cytokines.Several clinical trials have investigated MSC treatment in LN with promising but sometimes inconsistent outcomes.This review summarizes the sources of MSCs and mechanisms in immunoregulation.Furthermore,it examines clinical trials evaluating the efficacy,safety,and limitations of MSC therapy in LN.By highlighting advances and ongoing challenges,this review underscores the potential of MSCs for LN treatment.More large-scale randomized controlled trials are needed to support the effectiveness of this therapy and pave the way for personalized and combinatorial therapeutic approaches.展开更多
Systemic lupus erythematosus(SLE)is a chronic autoimmune disease.Defects in the regulatory T cells(Treg cells)play a key role in breaking immune tolerance in SLE patients.This study investigates the causes of impaired...Systemic lupus erythematosus(SLE)is a chronic autoimmune disease.Defects in the regulatory T cells(Treg cells)play a key role in breaking immune tolerance in SLE patients.This study investigates the causes of impaired Treg cell function in SLE patients.Peripheral blood from 56 SLE patients and 33 healthy donors was used to assess Treg cell proportions among CD4^(+)T cells and plasma cytokine levels.Treg cells and naïve CD4^(+)T cells from healthy individuals were isolated,cultured under various conditions,and analyzed for phenotype and signal transduction mechanisms using flow cytometry,RT-qPCR,Western blotting,and calcium signaling assays.In SLE patients,the proportion of CD4^(+)CD25^(+)Foxp3^(+)and CD4^(+)Foxp3^(+)Treg cells decreased.Plasma CXCL11 levels were elevated in lupus patients.CXCL11 expression inversely correlated with Treg cell proportions and positively correlated with disease severity.CXCL11 impaired immune function and inhibited Treg cell differentiation.We present a novel pathological pathway in SLE,wherein CXCL11 impedes the immunosuppressive functions of Treg cells.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
Objective To review and synthesize preclinical and clinical evidence regarding traditional Chinese medicine(TCM)formulations and bioactive herbal compounds in lupus nephritis(LN),and to identify direction and research...Objective To review and synthesize preclinical and clinical evidence regarding traditional Chinese medicine(TCM)formulations and bioactive herbal compounds in lupus nephritis(LN),and to identify direction and research priorities for integrating TCM with professional care.Methods A narrative literature review was conducted by searching Scopus,PubMed,Web of Science,and Google Scholar for articles published between January 1,2011 and March 31,2024.Search terms combined controlled vocabulary[e.g.,medical subject headings(MeSH)]and free-text words including lupus nephritis,traditional Chinese medicine,Chinese herbal medicine,formulation,and names of specific herbs[Leigongteng(Tripterygii Wilfordii Radix et Rhizoma),Baishao(Paeoniae Radix Alba),and Yinghao(Artemisiae Annuae Herba)].Both randomized controlled trials and observational studies were included,along with mechanistic preclinical studies and pharmacologic investigations.Inclusion criteria were studies that reported renal outcomes(proteinuria and estimated glomerular filtration rate),immunemodulatory mechanisms,or safety and herb-drug interaction data.Studies without primary data,case reports,or those lacking relevance to LN were excluded.References of key articles were manually screened to identify additional eligible studies.Results TCM formulas[e.g.,Liuwei Dihuang Pills(六味地黄丸),Zhibai Dihuang Pills(知柏地黄丸),and Huanglian Jiedu Decoction(黄连解毒汤)]and herbal medicines[e.g.,extracts from Leigongteng(Tripterygii Wilfordii Radix et Rhizoma),Baishao(Paeoniae Radix Alba),and Yinghao(Artemisiae Annuae Herba)]were commonly used in the above studies.TCM formulations and their constituent compounds showed multi-modal mechanisms relevant to LN pathogenesis,encompassing immunomodulation(reduction of autoreactive B/T cell activity,regulatory T cell enhancement),inhibition of pro-inflammatory signaling pathways nuclear factor kappa-light-chain-enhancer of activated B cells(NF-κB),mitogen-activated protein kinase(MAPK),nucleotide-binding oligomerization domain(NOD),leucine-rich repeat(LRR)and pyrin domain-containing protein 3(NLRP3 inflammasome),anti-fibrotic and antioxidant effects,and direct renal-protective properties.In many studies,these indicators can reduce proteinuria and improve renal function.Clinical data,while promising,are heterogeneous in design,sample size,endpoints,and TCM formulation standardization.Safety concerns(notably with Tripterygium preparations)and potential herb-drug interactions with immunosuppressants remained important considerations.Conclusion TCM offers biologically plausible and multi-targeted adjuvant strategies for LN that may enhance therapeutic efficacy and reduce toxicity when combined with modern therapies.To translate these promises into clinical practice,future work should prioritize the standardization of TCM preparations,randomized controlled trials with clinically meaningful renal endpoints,elucidation of molecular mechanisms,and systematic evaluation of pharmacokinetic and safety interactions.Such integrative research will be essential to define the TCM’s role in evidence-based,patient-centered LN management.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
Pediatric lupus nephritis(LN)is a challenging and severe condition in pediatrics.Traditional Chinese medicine(TCM)has shown significant advantages in improving immune disorders,reducing recurrence rates,and mitigating...Pediatric lupus nephritis(LN)is a challenging and severe condition in pediatrics.Traditional Chinese medicine(TCM)has shown significant advantages in improving immune disorders,reducing recurrence rates,and mitigating the toxic side effects of Western medications.However,it faces challenges such as the lack of a unified TCM syndrome differentiation system,insufficient standardization of dynamic syndrome differentiation,an incomplete efficacy evaluation system,and a lack of precise intervention methods.This study focuses on the clinical advantages of TCM.On 1 September 2024,the 35th Clinical Advantage Disease Series Salon was held in Zhengzhou,discussing the advantages of TCM and integrated Chinese-Western medicine in treating pediatric LN.Experts in TCM and integrated Chinese-Western medicine,along with interdisciplinary researchers,conducted extensive and in-depth discussions.They proposed specific recommendations for TCM and integrated Chinese-Western medicine in treating pediatric LN and reached a consensus.Based on this,the study analyzes the challenges in treating pediatric LN from the perspective of its development patterns,and summarizes three key areas and six research directions to highlight the advantages of TCM and integrated Chinese-Western medicine in treating pediatric LN.It focuses on three key areas:The construction of a TCM system for pediatric LN,the prevention and treatment of complications,and chronic disease management.And it proposes six research directions:(1)Constructing a syndrome differentiation system for pediatric LN;(2)Formulating TCM and integrated Chinese-Western medicine guidelines for pediatric LN;(3)Researching the mechanisms of enhancing efficacy and reducing toxicity in integrated Chinese-Western medicine for pediatric LN;(4)Preventing and treating complications in pediatric LN;(5)Developing and researching TCM regimens for preventing and treating the recurrence of pediatric LN;(6)Strategies for the full-cycle chronic disease management of pediatric LN.Finally,the study summarizes and generalizes the technological layout and research directions for pediatric LN.Therefore,based on the series of salons on traditional Chinese medicine advantages for children LN diseases,this paper puts forward a research paradigm of scientific and technological breakthroughs for children LN,in order to provide reference for the construction and research direction of children LN diagnosis and treatment system with traditional Chinese medicine characteristics.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
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
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
Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura （TTP）-like syndrome suggest a survival beneft to early treatment with plasma exchange. The following is a report of the eighth su...Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura （TTP）-like syndrome suggest a survival beneft to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-year-old lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady’s TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.展开更多
Membranous lupus nephritis(MLN),class V,is a distinct LN characterized by immune complex deposition on subepithelial kidney biopsy.MLN is often associated with nephrotic syndrome.The histology of MLN is very similar t...Membranous lupus nephritis(MLN),class V,is a distinct LN characterized by immune complex deposition on subepithelial kidney biopsy.MLN is often associated with nephrotic syndrome.The histology of MLN is very similar to idiopathic(primary)membranous nephropathy(pMN).However,MLN usually has abundant mesa-glomerular deposits absent in primary membranous nephropathy.The clinical manifestations,management,and prognosis of MLN differ from other types of LN(type III,IV,or mixed type III/IV+V).Although immunosuppressive therapy is often necessary for MLN,the optimal treatment regimen is yet to be determined.This review summarizes the progress in the diagnosis and treatment of MLN and discusses the selection of immunosuppressants for MLN.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
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
Recent advances in the management of lupus nephritis, together with earlier renal biopsy and selective use of aggressive immunosuppressive therapy, have contribut-ed to a favorable outcome in children and adolescents ...Recent advances in the management of lupus nephritis, together with earlier renal biopsy and selective use of aggressive immunosuppressive therapy, have contribut-ed to a favorable outcome in children and adolescents with systemic lupus erythematosus （SLE）. Neverthe-less, we believe that a more effective and less toxic treatment is needed to attain an optimal control of the activity of lupus nephritis. Recent published papers and our experiences regarding treatment of young patients with lupus nephritis using calcineurin inhibitors are re-viewed. Although it has been reported that intermittent monthly pulses of intravenous cyclophosphamide （IVCY） are effective for preserving renal function in adult pa-tients, CPA is a potent immunosuppressive agent thatinduces severe toxicity, including myelo- and gonadal toxicity, and increases the risk of secondary malig-nancy. Thus, treatment for controlling lupus nephritis activity, especially in children and adolescents, remains challenging. Cyclosporine A （CsA） and tacrolimus （Tac） are T-cell-specific calcineurin inhibitors that prevent the activation of helper T cells, thereby inhibiting thetranscription of the early activation genes of interleu-kin （IL）-2 and suppressing T cell-induced activation of tumor necrosis factor-α, IL-1β and IL-6. Therefore, both drugs, which we believe may be less cytotoxic, are attractive therapeutic options for young patients with lupus nephritis. Recently, a multidrug regimen of prednisolone （PDN）, Tac, and mycophenolate mofetile （MMF） has been found effective and relatively safe in adult lupus nephritis. Since the mechanisms of action of MMF and Tac are probably complementary, multidrug therapy for lupus nephritis may be useful. We propose as an alternative to IVCY, a multidrug therapy with mizoribine, which acts very similarly to MMF, and Tac, which has a different mode of action, combined with PDN for pediatric-onset lupus nephritis. We also believe that a multidrug therapy including CsA and Tac may bean attractive option for young patients with SLE and lupus nephritis展开更多
BACKGROUND Fulminant lupus myocarditis is a rare but fatal manifestation of systemic lupus erythematosus.Aggressive immunosuppressive treatments are important in its successful management.However,they can significantl...BACKGROUND Fulminant lupus myocarditis is a rare but fatal manifestation of systemic lupus erythematosus.Aggressive immunosuppressive treatments are important in its successful management.However,they can significantly damage the immunity and are associated with a considerable risk of infection development and spread.We present a rare and complicated case of a 20-year-old female diagnosed with fulminant lupus myocarditis accompanied by pneumonia.The patient was successfully treated with plasma exchange(PE)for fulminant lupus myocarditis.CASE SUMMARY A 20-year-old Chinese woman presented to the Hematology Department complaining of fatigue and knee pain.Blood test showed anemia and thrombocytopenia.On the second day of hospitalization,she was transferred to the ICU due to dyspnea and hypotension.Autoimmune profiles showed hypocomplementemia and positive antinuclear antibodies.Computer tomography showed an enlarged heart and pneumonia.Ultrasound revealed an enlarged heart with a low left ventricular ejection fraction.Fulminant lupus myocarditis with cardiogenic shock was initially considered.Due to the accompanying pneumonia,aggressive immunosuppression was contraindicated.Her cardiac function remained critical after the initial therapy of intravenous immunoglobulin and corticosteroids at a conventional dose,but she responded well to later PE therapy plus corticosteroids administration.The patient fully recovered with normal cardiac function.CONCLUSION This case indicates that PE is a valuable treatment choice without adverse effects of immunosuppression in patients with fulminant lupus myocarditis and coexisting infection.展开更多
BACKGROUND Complement overactivation is a major driver of lupus nephritis(LN).Impaired interactions of C-reactive protein(CRP)with complement factor H(CFH)have been shown as a pathogenic mechanism that contributes to ...BACKGROUND Complement overactivation is a major driver of lupus nephritis(LN).Impaired interactions of C-reactive protein(CRP)with complement factor H(CFH)have been shown as a pathogenic mechanism that contributes to the overactivation of complement in LN.However,genetic variations of neither CRP nor CFH show consistent influences on the risk of LN.AIM To examine whether genetic variations of CRP and CFH in combination can improve the risk stratification in Chinese population.METHODS We genotyped six CRP single nucleotide polymorphisms(SNPs)(rs1205,rs3093062,rs2794521,rs1800947,rs3093077,and rs1130864)and three CFH SNPs(rs482934,rs1061170,and rs1061147)in 270 LN patients and 303 healthy subjects.RESULTS No linkage was found among CRP and CFH SNPs,indicating lack of genetic interactions between the two genes.Moreover,CRP and CFH SNPs,neither individually nor in combination,are associated with the risk or clinical manifestations of LN.Given the unambiguous pathogenic roles of the two genes.CONCLUSION These findings suggest that the biological effects of most genetic variations of CRP and CFH on their expressions or activities are not sufficient to influence the disease course of LN.展开更多
文摘BACKGROUND Proliferative lupus nephritis(PLN)is the most severe form of lupus nephritis(LN).There are limited data available on renal outcomes of PLN from developing countries.AIM To determine the clinicopathological characteristics and long-term outcomes in terms of remission,requirement of kidney replacement therapy(KRT),and patient survival.METHODS A retrospective analysis was conducted on biopsy-proven focal or diffuse PLN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation and followed up at the renal clinic for a minimum of 5 years.All patients were induced with a combination of intravenous cyclophosphamide and corticosteroids for 6 months,followed by maintenance treatment with azathioprine(AZA)or mycophenolate mofetil(MMF).Data were analyzed using Statistical Package for the Social Sciences,version 22.0.P≤0.05 was considered statistically significant.RESULTS The mean age at the onset of systemic lupus erythematosus was 24.12 years±8.89 years,and at LN onset,26.63 years±8.61 years.There was a female predominance of 184(88.9%)cases.Among baseline characteristics,reduced estimated glomerular filtration rate,presence of hypertension,requirement of KRT,and underlying renal histology(International Society of Nephrology/Renal Pathology Society class IV than class III)were significantly associated with end-stage kidney disease(ESKD)and mortality.The renal outcomes were negatively correlated with age,duration of symptoms,and 24-hour urinary protein excretion.The overall remission rate was 89.8%at the end of induction therapy.At 5 years,141(68.11%)patients were in complete and partial remission(94[45.4%]and 47[22.7%],respectively).In total,19(9.2%)patients required KRT on presentation,and at 5 years,38(18.4%)patients developed ESKD,and 28(13.5%)patients died.Thirty-four(16.4%)patients had a renal relapse,more with AZA than MMF(30[88.2%]vs 4[11.76%],respectively;P=0.04).Renal survival at 6 months was 89.8%,while at 5 years,it was 68.11%,showing a significant improvement in patients who did not need KRT at the time of presentation(P<0.0001).CONCLUSION Baseline renal functions,requirement of KRT,and diffuse proliferative disease were the most relevant prognostic factors for kidney survival among this cohort.Short-term renal outcomes were good.Long-term outcomes were poorer with AZA-based maintenance therapy than with MMF,with more ESKD and mortality.
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.
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Supported by Natural Science Foundation of Zhejiang Province,No.LY23H050005Zhejiang Medical Technology Project,No.2020KY439,No.2022RC009,No.2024KY645,and No.2024KY697.
文摘Lupus nephritis(LN)is one of the most common and serious complications of systemic lupus erythematosus,which can lead to end-stage renal disease,and is an important cause of death in patients with systemic lupus erythematosus.Treatment options include glucocorticoids,immunosuppressive agents and the addition of biologics.Recently,the therapeutic role of mesenchymal stem cells(MSCs)in LN has received extensive attention worldwide.MSCs can suppress autoimmunity,alleviate proteinuria and restore renal function by modulating the functions of various immune cells and reducing the secretion of inflammatory cytokines.Several clinical trials have investigated MSC treatment in LN with promising but sometimes inconsistent outcomes.This review summarizes the sources of MSCs and mechanisms in immunoregulation.Furthermore,it examines clinical trials evaluating the efficacy,safety,and limitations of MSC therapy in LN.By highlighting advances and ongoing challenges,this review underscores the potential of MSCs for LN treatment.More large-scale randomized controlled trials are needed to support the effectiveness of this therapy and pave the way for personalized and combinatorial therapeutic approaches.
基金supported by the National Natural Science Foundation of China(No.82071838)the Key Medical Discipline Construction Unit of Jiangsu Province(No.JSDW202205)+1 种基金the Research Hospital of Jiangsu Province(No.YJXYY202204)Natural Science Foundation of Nantong City(No.JC2023053).
文摘Systemic lupus erythematosus(SLE)is a chronic autoimmune disease.Defects in the regulatory T cells(Treg cells)play a key role in breaking immune tolerance in SLE patients.This study investigates the causes of impaired Treg cell function in SLE patients.Peripheral blood from 56 SLE patients and 33 healthy donors was used to assess Treg cell proportions among CD4^(+)T cells and plasma cytokine levels.Treg cells and naïve CD4^(+)T cells from healthy individuals were isolated,cultured under various conditions,and analyzed for phenotype and signal transduction mechanisms using flow cytometry,RT-qPCR,Western blotting,and calcium signaling assays.In SLE patients,the proportion of CD4^(+)CD25^(+)Foxp3^(+)and CD4^(+)Foxp3^(+)Treg cells decreased.Plasma CXCL11 levels were elevated in lupus patients.CXCL11 expression inversely correlated with Treg cell proportions and positively correlated with disease severity.CXCL11 impaired immune function and inhibited Treg cell differentiation.We present a novel pathological pathway in SLE,wherein CXCL11 impedes the immunosuppressive functions of Treg cells.
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Objective To review and synthesize preclinical and clinical evidence regarding traditional Chinese medicine(TCM)formulations and bioactive herbal compounds in lupus nephritis(LN),and to identify direction and research priorities for integrating TCM with professional care.Methods A narrative literature review was conducted by searching Scopus,PubMed,Web of Science,and Google Scholar for articles published between January 1,2011 and March 31,2024.Search terms combined controlled vocabulary[e.g.,medical subject headings(MeSH)]and free-text words including lupus nephritis,traditional Chinese medicine,Chinese herbal medicine,formulation,and names of specific herbs[Leigongteng(Tripterygii Wilfordii Radix et Rhizoma),Baishao(Paeoniae Radix Alba),and Yinghao(Artemisiae Annuae Herba)].Both randomized controlled trials and observational studies were included,along with mechanistic preclinical studies and pharmacologic investigations.Inclusion criteria were studies that reported renal outcomes(proteinuria and estimated glomerular filtration rate),immunemodulatory mechanisms,or safety and herb-drug interaction data.Studies without primary data,case reports,or those lacking relevance to LN were excluded.References of key articles were manually screened to identify additional eligible studies.Results TCM formulas[e.g.,Liuwei Dihuang Pills(六味地黄丸),Zhibai Dihuang Pills(知柏地黄丸),and Huanglian Jiedu Decoction(黄连解毒汤)]and herbal medicines[e.g.,extracts from Leigongteng(Tripterygii Wilfordii Radix et Rhizoma),Baishao(Paeoniae Radix Alba),and Yinghao(Artemisiae Annuae Herba)]were commonly used in the above studies.TCM formulations and their constituent compounds showed multi-modal mechanisms relevant to LN pathogenesis,encompassing immunomodulation(reduction of autoreactive B/T cell activity,regulatory T cell enhancement),inhibition of pro-inflammatory signaling pathways nuclear factor kappa-light-chain-enhancer of activated B cells(NF-κB),mitogen-activated protein kinase(MAPK),nucleotide-binding oligomerization domain(NOD),leucine-rich repeat(LRR)and pyrin domain-containing protein 3(NLRP3 inflammasome),anti-fibrotic and antioxidant effects,and direct renal-protective properties.In many studies,these indicators can reduce proteinuria and improve renal function.Clinical data,while promising,are heterogeneous in design,sample size,endpoints,and TCM formulation standardization.Safety concerns(notably with Tripterygium preparations)and potential herb-drug interactions with immunosuppressants remained important considerations.Conclusion TCM offers biologically plausible and multi-targeted adjuvant strategies for LN that may enhance therapeutic efficacy and reduce toxicity when combined with modern therapies.To translate these promises into clinical practice,future work should prioritize the standardization of TCM preparations,randomized controlled trials with clinically meaningful renal endpoints,elucidation of molecular mechanisms,and systematic evaluation of pharmacokinetic and safety interactions.Such integrative research will be essential to define the TCM’s role in evidence-based,patient-centered LN management.
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Pediatric lupus nephritis(LN)is a challenging and severe condition in pediatrics.Traditional Chinese medicine(TCM)has shown significant advantages in improving immune disorders,reducing recurrence rates,and mitigating the toxic side effects of Western medications.However,it faces challenges such as the lack of a unified TCM syndrome differentiation system,insufficient standardization of dynamic syndrome differentiation,an incomplete efficacy evaluation system,and a lack of precise intervention methods.This study focuses on the clinical advantages of TCM.On 1 September 2024,the 35th Clinical Advantage Disease Series Salon was held in Zhengzhou,discussing the advantages of TCM and integrated Chinese-Western medicine in treating pediatric LN.Experts in TCM and integrated Chinese-Western medicine,along with interdisciplinary researchers,conducted extensive and in-depth discussions.They proposed specific recommendations for TCM and integrated Chinese-Western medicine in treating pediatric LN and reached a consensus.Based on this,the study analyzes the challenges in treating pediatric LN from the perspective of its development patterns,and summarizes three key areas and six research directions to highlight the advantages of TCM and integrated Chinese-Western medicine in treating pediatric LN.It focuses on three key areas:The construction of a TCM system for pediatric LN,the prevention and treatment of complications,and chronic disease management.And it proposes six research directions:(1)Constructing a syndrome differentiation system for pediatric LN;(2)Formulating TCM and integrated Chinese-Western medicine guidelines for pediatric LN;(3)Researching the mechanisms of enhancing efficacy and reducing toxicity in integrated Chinese-Western medicine for pediatric LN;(4)Preventing and treating complications in pediatric LN;(5)Developing and researching TCM regimens for preventing and treating the recurrence of pediatric LN;(6)Strategies for the full-cycle chronic disease management of pediatric LN.Finally,the study summarizes and generalizes the technological layout and research directions for pediatric LN.Therefore,based on the series of salons on traditional Chinese medicine advantages for children LN diseases,this paper puts forward a research paradigm of scientific and technological breakthroughs for children LN,in order to provide reference for the construction and research direction of children LN diagnosis and treatment system with traditional Chinese medicine characteristics.
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Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura （TTP）-like syndrome suggest a survival beneft to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-year-old lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady’s TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.
文摘Membranous lupus nephritis(MLN),class V,is a distinct LN characterized by immune complex deposition on subepithelial kidney biopsy.MLN is often associated with nephrotic syndrome.The histology of MLN is very similar to idiopathic(primary)membranous nephropathy(pMN).However,MLN usually has abundant mesa-glomerular deposits absent in primary membranous nephropathy.The clinical manifestations,management,and prognosis of MLN differ from other types of LN(type III,IV,or mixed type III/IV+V).Although immunosuppressive therapy is often necessary for MLN,the optimal treatment regimen is yet to be determined.This review summarizes the progress in the diagnosis and treatment of MLN and discusses the selection of immunosuppressants for MLN.
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.
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Recent advances in the management of lupus nephritis, together with earlier renal biopsy and selective use of aggressive immunosuppressive therapy, have contribut-ed to a favorable outcome in children and adolescents with systemic lupus erythematosus （SLE）. Neverthe-less, we believe that a more effective and less toxic treatment is needed to attain an optimal control of the activity of lupus nephritis. Recent published papers and our experiences regarding treatment of young patients with lupus nephritis using calcineurin inhibitors are re-viewed. Although it has been reported that intermittent monthly pulses of intravenous cyclophosphamide （IVCY） are effective for preserving renal function in adult pa-tients, CPA is a potent immunosuppressive agent thatinduces severe toxicity, including myelo- and gonadal toxicity, and increases the risk of secondary malig-nancy. Thus, treatment for controlling lupus nephritis activity, especially in children and adolescents, remains challenging. Cyclosporine A （CsA） and tacrolimus （Tac） are T-cell-specific calcineurin inhibitors that prevent the activation of helper T cells, thereby inhibiting thetranscription of the early activation genes of interleu-kin （IL）-2 and suppressing T cell-induced activation of tumor necrosis factor-α, IL-1β and IL-6. Therefore, both drugs, which we believe may be less cytotoxic, are attractive therapeutic options for young patients with lupus nephritis. Recently, a multidrug regimen of prednisolone （PDN）, Tac, and mycophenolate mofetile （MMF） has been found effective and relatively safe in adult lupus nephritis. Since the mechanisms of action of MMF and Tac are probably complementary, multidrug therapy for lupus nephritis may be useful. We propose as an alternative to IVCY, a multidrug therapy with mizoribine, which acts very similarly to MMF, and Tac, which has a different mode of action, combined with PDN for pediatric-onset lupus nephritis. We also believe that a multidrug therapy including CsA and Tac may bean attractive option for young patients with SLE and lupus nephritis
文摘BACKGROUND Fulminant lupus myocarditis is a rare but fatal manifestation of systemic lupus erythematosus.Aggressive immunosuppressive treatments are important in its successful management.However,they can significantly damage the immunity and are associated with a considerable risk of infection development and spread.We present a rare and complicated case of a 20-year-old female diagnosed with fulminant lupus myocarditis accompanied by pneumonia.The patient was successfully treated with plasma exchange(PE)for fulminant lupus myocarditis.CASE SUMMARY A 20-year-old Chinese woman presented to the Hematology Department complaining of fatigue and knee pain.Blood test showed anemia and thrombocytopenia.On the second day of hospitalization,she was transferred to the ICU due to dyspnea and hypotension.Autoimmune profiles showed hypocomplementemia and positive antinuclear antibodies.Computer tomography showed an enlarged heart and pneumonia.Ultrasound revealed an enlarged heart with a low left ventricular ejection fraction.Fulminant lupus myocarditis with cardiogenic shock was initially considered.Due to the accompanying pneumonia,aggressive immunosuppression was contraindicated.Her cardiac function remained critical after the initial therapy of intravenous immunoglobulin and corticosteroids at a conventional dose,but she responded well to later PE therapy plus corticosteroids administration.The patient fully recovered with normal cardiac function.CONCLUSION This case indicates that PE is a valuable treatment choice without adverse effects of immunosuppression in patients with fulminant lupus myocarditis and coexisting infection.
文摘BACKGROUND Complement overactivation is a major driver of lupus nephritis(LN).Impaired interactions of C-reactive protein(CRP)with complement factor H(CFH)have been shown as a pathogenic mechanism that contributes to the overactivation of complement in LN.However,genetic variations of neither CRP nor CFH show consistent influences on the risk of LN.AIM To examine whether genetic variations of CRP and CFH in combination can improve the risk stratification in Chinese population.METHODS We genotyped six CRP single nucleotide polymorphisms(SNPs)(rs1205,rs3093062,rs2794521,rs1800947,rs3093077,and rs1130864)and three CFH SNPs(rs482934,rs1061170,and rs1061147)in 270 LN patients and 303 healthy subjects.RESULTS No linkage was found among CRP and CFH SNPs,indicating lack of genetic interactions between the two genes.Moreover,CRP and CFH SNPs,neither individually nor in combination,are associated with the risk or clinical manifestations of LN.Given the unambiguous pathogenic roles of the two genes.CONCLUSION These findings suggest that the biological effects of most genetic variations of CRP and CFH on their expressions or activities are not sufficient to influence the disease course of LN.
