Systemic Lupus Erythematosus Patients Research Articles

The relationship between vitamin D status and disease activity in Iraqi systemic lupus erythematosus patients.

To find the relationship between vitamin D deficiency and the activity of systemic lupus erythematosus in Iraqi patients. The case-control study was conducted at Baghdad Teaching Hospital, Baghdad, Iraq, from July to October 2018, and comprised systemic lupus erythematosus patients regardless of age and gender visiting the Rheumatology outpatient clinic. Serum levels of complement protein 3, complement protein 4, anti-doublestranded deoxyribonucleic acid and 25-hydroxy vitamin D were estimated. Based on disease activity scores, patients were divided into moderate activity group SLE-M and severe activity group SLE-S. Healthy subjects matched for age and gender were also enrolled as the control group. Data was analysed using Graph Pad Prism 5.0. Of the 150 subjects, 62(41.3%) were in SLE-S group, 38(25.3%) in SLE-M and 59(33.3%) in the control group. Among the patients, 97(97%) were females and 3(3%) were males, with a female-to-male ratio of 32:1. The patients' age range was <10-≥50 years, while the control group consisted of 2 (4%) males and 48 (96%) females with an age range of <10-≥50. The mean levels of serum complement protein 3, complement protein 4 and vitamin D levels were significantly lower in the patient groups compared to the controls (p<0.05). Systemic lupus erythematosus patients suffered from either vitamin D deficiency or insufficiency, and low vitamin D levels were found related to disease activity.

Evaluating treatment effect on interferon-alpha in female patients with systemic lupus erythematosus: a case-control study.

To assess the effect of treatment on interferon-alpha titer in the serum of female patients with systemic lupus erythematosus. The case-control study was conducted at the Rheumatology Unit of Baghdad Teaching Hospital from January 2022 to April 2022 and comprised female systemic lupus erythematosus patients and healthy controls matched for age. The treated patients formed group A, while the untreated patients were placed in group B, and the controls were in group C. The disease severity status was confirmed using the systemic lupus erythematosus disease activity index. The serum level of interferon-alpha was determined using the enzyme-linked immunosorbent assay. Data was analysed using SPSS 21. Of the 150 subjects, 5033.3%) were in each of the 3 groups. The mean age of patients was 32.3±9 years while that of the controls was 33.0±11 years. Erythrocyte sedimentation rate, creatinine and aspartate aminotransferase were significantly higher in patient groups compared to the controls (p<0.05), while no-significant difference was noted between the treated and untreated groups (p>0.05). Interferon-alpha level was significantly high in group B compared to group A (p=0.037). The increase was more pronounced in those aged ≤50 years (p<0.05). Interferon-alpha was significantly high in group B patients with a disease duration of <1 year compared to group A (p=0.01). Interferon-alpha showed a significant increase in group B compared to the group A in patients with inactive systemic lupus erythematosus disease activity index and with family history (p<0.05). Treatment had a direct impact on interferon-alpha protein level in the serum of female patients of systemic lupus erythematosus.

Novel endothelial progenitor cells populations as biomarkers of damage and remission in systemic lupus erythematosus

IntroductionEndothelial progenitor cells (EPCs) are essential for maintenance of vascular homeostasis and stability, key processes in the pathogenesis of systemic lupus erythematosus (SLE). However, the role and phenotypic characterization of EPCs populations in SLE have not been completely elucidated.ObjectiveTo identify EPCs specific subpopulations in patients with SLE using a novel flow cytometry tool.MethodsPeripheral blood mononuclear cells (PBMCs) were isolated from patients with SLE and healthy controls (HC). mRNA and surface protein expression were determined by quantitative PCR (qPCR) and flow cytometry. Clusters identification and characterization were performed using tSNE-CUDA dimensionality reduction algorithms.ResultstSNE-CUDA analysis identified eight different clusters in PBMCs from HC and patients with SLE. Three of these clusters had EPC-like phenotype and the expression was elevated in patients with SLE. Moreover, four SLE-associated subclusters were found mainly expressed in patients with SLE, being only present in patients in remission with SLE and significantly associated with the 2021 Definition of Remission in SLE. Importantly, we also identified specific clusters in SLE patients with organ damage, according to the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). These clusters showed an EPC-like phenotype, but the expression of angiogenic markers was lower compared to HC or patients without organ damage, suggesting an impaired angiogenic function.ConclusionOur novel approach identified clusters of EPCs in patients with SLE that are associated with remission and damage. Therefore, these clusters might be useful biomarkers to predict disease progression and severity in SLE pathogenesis.

Translating Lupus: Comparative Transcriptional Profiles of Preclinical Lupus Models and Their Relevance to Human Disease.

Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease which can present with mixed organ involvement. Kidney involvement in lupus nephritis (LN) is a severe complication and major cause of mortality in SLE patients, second only to cardiovascular disease. While mouse models have helped uncover some molecular pathways involved in SLE/LN, we need a better understanding of the connection of these pathways and the immune cells involved in disease pathogenesis to develop new and effective therapies. Furthermore, models used for studying SLE/LN in mice have a heterogeneous immune response and may not always represent disease manifestations observed in patients. Identifying models that have shared pathways with human disease would allow for better translation for developing effective SLE/LN therapies. The molecular pathways of five different SLE/LN models (MRL/lpr, poly (I:C)-induced, interferon-α-induced, bm12 GvHD, and spontaneous NZB/W F1) were compared to characterize the immune response in mouse kidneys. These models demonstrated varied magnitudes in immune responses and proportions of innate vs. adaptive cell involvement. These findings were compared to human molecular pathways and cell types from public databases, including the Accelerating Medicine Partnership-Systemic Lupus Erythematosus Program (AMP-SLE), to help corelate mechanisms involved in mouse models to human disease.

Outcomes and Predictors of Inpatient Mortality for Marantic Endocarditis Complicating Systemic Lupus Erythematosus: Contemporary Nationwide Study From the United States.

Systemic lupus erythematosus (SLE) patients are susceptible to marantic endocarditis (ME) due to a hypercoagulable state. The literature regarding the epidemiology and outcomes of ME in SLE patients is limited. All patients ≥18 years who had SLE with and without ME between 2007 and 2019 were identified from the National Inpatient Sample in the United States (US). Predictors of inpatient mortality for SLE patients with ME were analyzed. Between 2007 and 2019, there were 508,818 hospitalizations for SLE, of which 785 (0.2%) had ME. Of SLE patients with ME, 33 (4.2%) died while hospitalized over the study period. On multivariate analysis, female sex (adjusted odds ratio (aOR), 95% confidence intervals: 24.72 (3.21, 190.27)), age <34 years (aOR: 6.81 (1.80, 25.79)), anemia (aOR: 3.41 (1.12, 10.40)), antiphospholipid syndrome (aOR: 13.50 (3.83, 47.64)), stroke complicating ME (aOR: 9.64 (3.24, 28.71)), and acute kidney injury (aOR: 3.74 (1.06, 13.20)) were all associated with increased inpatient mortality among SLE patients with ME (P < .05 for all). Between 2007 to 2019, ME occurred in 0.2% of SLE hospitalizations, with a 4.2% average inpatient mortality over the study period. Female sex, antiphospholipid syndrome, and stroke were most strongly associated with increased inpatient mortality.

Correlation Between Sex Hormones (Testosterone and Prolactin) and Anti-ds-DNA Antibody Levels in Systemic Lupus Erythematosus Patients: A Gender-Specific Analysis of the Hormonal Influence on Immune Responses.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that mostly affects women and has a noteworthy gender gap. Although the precise effects of sex hormones on SLE are yet unknown, they may modulate the immune response and affect the severity of the illness. Two important sex hormones that have apossible influence on autoimmune processes are prolactin and testosterone. The objective of this study was to assess the correlation between the levels of different sex hormones (testosterone and prolactin) and anti-double-stranded DNA (anti-ds-DNA) antibody levels in Sudanese anti-ds-DNA-positive SLE patients. Methodology A prospective cross-sectional study was conducted from February to July 2017 at Al-Rayan Specialized Medical Laboratory Complex and Omdurman Military Hospital, Sudan. There were 90 SLE patients who tested positive for anti-ds-DNA. Using commercial kits, serum levels of prolactin, testosterone, and anti-ds-DNA antibodies were tested from blood samples. SPSS version 21 was used for statistical analysis to find relationships between hormone levels and antibody levels. Results In this study of 90 patients with SLE, gender distribution was 14 males (16%) and 76 females (84%). The mean prolactin levels were significantly higher in males (17.99 ng/ml) compared to females (15.73 ng/ml, P < 0.001), and testosterone levels were also significantly higher in males (1.43 ng/ml) than females (0.37 ng/ml, P = 0.001). Although males had slightly higher mean levels of anti-ds-DNA antibodies (4.51) compared to females (4.08), this difference was not statistically significant. The results of correlation analyses indicated a weak and non-significant link between anti-ds-DNA antibody levels and hormone levels, with prolactin's Spearman's rho value of -0.054 (P = 0.616) and testosterone's Spearman's rho value of 0.080 (P = 0.456). Conclusion It was found that prolactin and testosterone levels differ by gender in SLE patients and that these hormones may have an impact on how the condition is managed. But the poor associations with anti-ds-DNA antibody levels suggest that further investigation is required to fully comprehend these intricate interactions.

Mitochondrial Dysfunction in Systemic Lupus Erythematosus: Insights and Therapeutic Potential.

Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by the presence of various serum autoantibodies and multi-system effects, predominantly affecting young female patients. The pathogenesis of SLE involves a combination of genetic factors, environmental triggers, and pathogen invasions that disrupt immune cell activation, leading to the release of autoantibodies and chronic inflammation. Mitochondria, as the primary cellular powerhouses, play a crucial role in SLE development through their control of energy generation, reactive oxygen species (ROS) production, and cellular apoptotic pathways. Dysregulation of mitochondrial structure and function can contribute to the immune dysregulation, oxidative stress, and inflammation seen in SLE. Recent research has highlighted the impact of mitochondrial dysfunction on various immune cells involved in SLE pathogenesis, such as T-lymphocytes, B-lymphocytes, neutrophils, and plasmacytoid dendritic cells. Mitochondrial dysfunction in these immune cells leads to increased ROS production, disrupted mitophagy, and alterations in energy metabolism, contributing to immune dysregulation and inflammation. Moreover, genetic variations in mitochondrial DNA (mtDNA) and abnormalities in mitochondrial dynamics have been linked to the pathogenesis of SLE, exacerbating oxidative stress and immune abnormalities. Targeting mitochondrial function has emerged as a promising therapeutic approach for SLE. Drugs such as sirolimus, N-acetylcysteine, coenzyme Q10, and metformin have shown potential in restoring mitochondrial homeostasis, reducing oxidative stress, and modulating immune responses in SLE. These agents have demonstrated efficacy in preclinical models and clinical studies by improving disease activity, reducing autoantibody titers, and ameliorating organ damage in SLE patients. In conclusion, this review underscores the critical role of mitochondria in the pathogenesis of SLE and the potential of targeting mitochondrial dysfunction as a novel therapeutic strategy for improving outcomes in SLE patients. Further investigation into the mechanisms underlying mitochondrial involvement in SLE and the development of targeted mitochondrial therapies hold promise for advancing SLE treatment and enhancing patient care.

Cryptogenic Organizing Pneumonia Is Associated With Increased Mortality Risk in Hospitalizations for Systemic Lupus Erythematosus (SLE): A National Inpatient Sample Analysis.

Background This study analyzed the incidence, characteristics, and mortality risk associated with cryptogenic organizing pneumonia (COP) among hospitalizations for systemic lupus erythematosus (SLE) with lung involvement. Methods Adult hospitalizations from the 2016-2020 nationwide inpatient sample were analyzed using relevantInternational Classification of Diseases (ICD)-10 codes for SLE with lung involvement (M32.13) and COP (J84.116). We compared baseline characteristics of individuals with SLE and COP to those of other lung involvements using Chi-square tests for categorical variables and the Wilcoxon rank sum test for continuous variables. A Cox proportional hazards model was used to assess the risk of developing COP in the pooled cohort of SLE patients. The impact of COP on SLE mortality was assessed using multivariate logistic regression adjusting forillness severity, baseline risk of mortality at admission, and patient- and hospital-level covariates. Results Of 40,356 admissions for SLE, 3,175 (7.9%) were due to lung involvement, with COP identified in 570 cases (17.9%). Compared with otherlung involvement in SLE, individuals with COP were significantly older (mean age: 65 vs. 44.3 years; p<0.001), mostly female (515; 90.4% vs. 2,305 males; 88.5%; p=0.572), had a greater baseline risk of mortality [diagnosis-related groups (DRG) major or extreme likelihood of dying: 360; 63.1% vs. 1,133; 43.5%; p<0.001], and had a higher prevalence of peripheral vascular disease (25; 4.4% vs. 39; 1.5%; p<0.001), and lower prevalence of lymphocytopenia (45; 7.9% vs. 359; 13.8%; p=0.001), and hypothyroidism (44; 7.8% vs. 357; 13.7%; p=0.001). Predictors of COP included female sex [adjusted hazard ratio (AHR): 1.46; 95% confidence interval (CI): 1.12-2.96; p=0.022]; hospitalizations occurring in the third quarter of the year (AHR: 1.37; 95% CI: 1.05-2.23; p=0.038); hospital stays of six days or longer (AHR: 1.71; 95% CI: 1.06-2.77; p=0.029); undergoing five or more procedures during the same hospitalization (AHR: 1.56; 95% CI: 1.26-3.56; p=0.041); coexisting lymphocytopenia (AHR: 1.92; 95% CI: 1.16-3.19; p=0.011); need for mechanical ventilation (AHR: 1.60; 95% CI: 1.48-3.93; p=0.049), presence of another autoimmune disorder (AHR: 1.37; 95% CI: 1.15-4.29; p=0.040), and being hospitalized at private, investor-owned hospitals (AHR: 2.62; 95% CI: 1.03-6.64; p=0.043). Mortality in SLE with lung involvement was correlated with age ≥ 60 years [hazard ratio (HR) (95% CI) 1.16 (1.05-1.56); p=0.012], coexisting lupus nephritis [HR (95% CI), 2.44 (2.04-3.49); p=0.031], cancer [HR (95% CI), 3.49 (2.19-5.79); p<0.001], liver disease [HR (95% CI), 9.82 (4.79-12.57); p<0.001]; immune deficiency [HR (95% CI), 2.22 (2.02-3.11); p=0.031], hypothyroidism [HR (95% CI), 4.67 (1.47-7.75); p=0.009], and high blood pressure [HR (95% CI), 3.15 (2.83-4.51); p<0.001]. In the multivariable analysis, COP remained significantly associated with an increased risk of mortality [AHR (95% CI), 1.43 (1.16-2.74); p=0.031]. The incidence of COP did not significantly impact hospitalization costs ($US 94,772 ± 14,759 vs. 95,982 ± 32,625; p=0.954) or length of stay (mean length of hospital stay: 8.3 vs.6.8 days; p=0.147). Conclusion Cryptogenic organizing pneumoniawas associated with 1% of all hospitalizations for SLE and 18% of cases involving lung complications in SLE. The presence of COP significantly increased the risk of mortality in SLE patients with lung involvement.

Rice Husk Silica Liquid Enhances Autophagy and Reduces Overactive Immune Responses via TLR-7 Signaling in Lupus-Prone Models.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by widespread inflammation and multi-organ damage. Toll-like receptor 7 (TLR-7) and autophagy have been implicated in SLE pathogenesis. Rice husk silica liquid (RHSL) has shown potential for modulating inflammatory responses, but its effects on SLE have not been thoroughly investigated. This study aims to evaluate the impact of RHSL on immune responses and autophagy in cell culture experiments, focusing on its effects on TLR-7 signaling, cytokine production, and autophagy modulation. RAW264.7 cells and human peripheral blood mononuclear cells (PBMCs) from healthy donors and SLE patients were used. Cells were stimulated with LPS or TLR-7 agonists and treated with RHSL. Cell viability was assessed, and cytokine levels (TNF-α and IL-6) were measured by ELISA. Autophagy-related proteins (LC3II, ATG5-ATG12) were analyzed by Western blotting. The effect of autophagy inhibition was studied using 3-methyladenine (3-MA). A concentration of 100 μg/mL RHSL did not affect cell viability but significantly reduced the TNF-α production in TLR-7 agonist-stimulated RAW264.7 cells (compared to TLR-7 alone, 3.41 ± 0.54 vs. 6.72 ± 0.07 folds) and PBMCs (compared to TLR-7 alone, 0.97 ± 0.19 vs. 1.40 ± 0.33 folds). RHSL enhanced autophagy, as evidenced by increased LC3II (4.35 ± 1.08 folds) and ATG5-ATG12 (7.07 ± 1.30 folds) conjugation in both RAW264.7 cells and SLE patient-derived PBMCs. The reduction in TNF-α production by RHSL was attenuated by 3-MA, indicating that autophagy plays a role in this process. RHSL also inhibited the translocation of phosphorylated NF-κB into the nucleus, suggesting a mechanism for its anti-inflammatory effects. RHSL exhibits potential as an immunomodulatory agent in SLE by enhancing autophagy and modulating TLR-7 signaling pathways. These findings suggest that RHSL could offer therapeutic benefits for managing inflammatory responses in SLE and warrant further investigation into its clinical applications.

Prevalencia de las manifestaciones neuropsiquiátricas en lupus eritematoso sistémico

Introduction: neuropsychiatric (NP) manifestations in systemic lupus erythematosus (SLE) are common and have gained interest due to their increasing prevalence. Their clinical recognition is difficult because of their diverse and variable presentation. This study is focused on highlighting the forms of NP involvement for their recognition and treatment. Objective: to determine NPSLE manifestations prevalence in SLE patients aged 18 years or older. Materials and methods: a systematic review of the scientific literature in various databases, selecting cross-sectional and prevalence studies evaluating NPSLE manifestations. Results: 26 articles which met the inclusion criteria were selected, the most common manifestations being headache, seizures, cognitive dysfunction, depression, and anxiety. Other less frequent manifestations include myasthenia gravis, Miller Fisher syndrome, mononeuritis multiplex and concentration loss. Conclusion: both neurological and psychiatric manifestations studied, have a high prevalence in the total population, and are not always recognized by clinicians, lacking a timely diagnosis to determine whether they are caused by SLE disease activity on the nervous system or other end-organ dysfunctions.

Circulating extracellular vesicles as novel biomarkers for pulmonary arterial hypertension in patients with systemic lupus erythematosus.

Pulmonary arterial hypertension (PAH) is a serious complication of systemic lupus erythematosus (SLE) with increased mortality. A prothrombotic state may contribute to pathogenesis of SLE-PAH. Extracellular vesicles (EVs) are known to be associated with thrombosis. Here, we investigated circulating EVs and their associations with SLE-PAH. Eighteen SLE-PAH patients, 36 SLE-non-PAH patients, and 36 healthy controls (HCs) were enrolled. Flow cytometry was used to analyze circulating EVs from leukocytes (LEVs), red blood cells (REVs), platelets (PEVs), endothelial cells (EEVs), and Annexin V+ EVs with membrane phosphatidylserine (PS) exposure. Plasma levels of all EV subgroups were elevated in SLE patients with or without PAH compared to HCs. Furthermore, plasma Annexin V+ EVs, LEVs, PEVs, REVs, EEVs, and Annexin V+ REVs were significantly elevated in SLE-PAH patients compared to SLE-non-PAH patients. Additionally, PAH patients with moderate/high SLE showed a significant increase in LEVs, PEVs, REVs, Annexin V+ EVs, and Annexin V+ REVs compared to SLE-non-PAH patients. However, PAH patients with inactive/mild SLE only exhibited elevations in Annexin V+ EVs, REVs, and Annexin V+ REVs. In the SLE-PAH patients, EEVs were positively correlated with pulmonary arterial systolic pressure, while PEVs and EEVs were positively correlated with right ventricular diameter. Moreover, the receiver operating characteristic curve indicated that Annexin V+ EVs, LEVs, PEVs, REVs, EEVs and Annexin V+ REVs could predict the presence of PAH in SLE patients. Importantly, multivariate logistic regression analysis showed that circulating levels of LEVs or REVs, anti-nRNP antibody, and serositis were independent risk factors for PAH in SLE patients. Findings reveal that specific subgroups of circulating EVs contribute to the hypercoagulation state and the severity of SLE-PAH. Higher plasma levels of LEVs or REVs may serve as biomarkers for SLE-PAH.

Effectiveness of Comprehensive Lifestyle Interventions on Disease Activity and Quality of Life in Patients with Systemic Lupus Erythematosus in Pakistan

Background: Systemic lupus erythematosus (SLE), is a chronic autoimmune illness that significantly lowers quality of life in its victims. Although pharmaceutical therapies are the norm, more focus is being paid to lifestyle changes as a means of controlling disease activity. Objectives: The impact of a comprehensive lifestyle intervention program on patients' quality of life and disease activity with systemic lupus erythematosus was assessed in this research. Methodology: Total 100 individuals with systemic lupus erythematosus were enrolled in a randomized controlled experiment and split into two groups: the intervention group (n = 50) and the control group (n = 50). Over a six-month period, the intervention comprised stress management, consistent exercise, and dietary adjustments. Changes in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) Scores were the primary endpoint; quality of life, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were the secondary objectives. Results: The SLEDAI scores of the intervention group were significantly lower than those of the control group (mean change: -4.2 vs. -1.3, p&lt;0.01). Improvements in the SF-36 survey's physical and mental components showed a substantial increase in quality of life. Furthermore, there were higher drops in blood serum CRP and ESR levels in the intervention group (CRP: -3.5 mg/L vs. -1.2 mg/L, p≤0.05; ESR: -10 mm/hr vs. -3 mm/hr, p≤0.05). Conclusion: Diet, exercise, and stress management are examples of lifestyle therapies that have been shown to dramatically lower disease activity and enhance quality of life in SLE patients. The inclusion of lifestyle adjustments in standard SLE treatment procedures is supported by these findings.

Use of belimumab in treating patients with systemic lupus erythematosus: a single-center, real-world retrospective study

ObjectiveTo investigate the efficacy and safety of belimumab in the treatment of systemic lupus erythematosus (SLE) in a real-world setting and provide a valuable reference for clinical treatment.MethodsIn this retrospective study, 101 patients with SLE who came to our hospital from March 2020 to September 2022, 56 of whom with lupus nephritis (LN), were selected. All patients received belimumab in combination with standard of care(SoC)therapy regimen for more than 52 weeks and their clinical/laboratory data, assessment of disease activity, glucocorticoids dosage and occurrence of adverse events were recorded. Lupus Low Disease Activity State (LLDAS) and DORIS remission as a primary goal in the treatment of SLE. The groups were classified according to the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K): SLEDAI-2 K < 6 was categorized as the mild group (mild activity) and SLEDAI-2 K ≥ 6 was categorized as the active group (moderate-severe activity). The disease of the two groups mentioned above were assessed using the SELENA-SLEDAI Flare Index (SFI) and the SLE Responder Index-4 (SRI-4), respectively. Furthermore, we used complete remission (CR) and partial remission (PR) in the kidney as the standard for efficacy evaluation for LN patients.ResultsAfter 52 weeks of treatment with belimumab, patients’ complement levels increased significantly (p < 0.05); Other indicators such as 24-hour urine protein quantification and daily glucocorticoids dose decreased compared to pretreatment (p < 0.05). At 52 weeks, (i) after evaluation, the whole group of patients showed significant improvement in their condition; (ii) 55.4% of patients achieved LLDAS and 23.8% achieved DORIS remission; (iii) 73.2% of patients with LN achieved CR, 16.1% achieved PR. Adverse reactions were observed in 15 patients (14.9%), all of which normalized after symptomatic treatment.ConclusionsIn general, during treatment with belimumab, immunological and biochemical indices improved in SLE patients, urinary protein levels were reduced in LN patients, and the rate of renal function remission was effectively increased; At the same time, the use of belimumab is associated with a low frequency of side effects, good overall tolerability and a favorable safety profile.

Clinical Features and Survival Analysis of Lupus Nephritis among Patients with Systemic Lupus Erythematosus: A Three-Decade-Long Retrospective Cohort Study.

Background/Objectives: Lupus nephritis (LN) is one of the most severe organ manifestations of systemic lupus erythematosus (SLE). The aim of our retrospective cohort study was to compare the clinical characteristics, therapy, survival, causes of death, and prognostic factors of LN and non-LN lupus patients. Moreover, we compared a wide spectrum of clinical data of LN patients diagnosed before and since 2005 to determine any changes in disease course and outcomes. Methods: We assessed the clinical and laboratory data of 384 SLE patients, out of whom, 127 patients were diagnosed with LN between 1990 and 2020. Results: Based on our observations, discoid LE, subacute cutaneous LE, antiphospholipid syndrome, Sjögren's syndrome, and rheumatoid arthritis were more common in non-LN patients, while anemia and anti-RNP positivity were more frequent in LN patients. Development of LN did not affect survival rates; male sex and presence of APS were negative prognostic parameters in the non-LN group while achieving remission was a positive prognostic factor in both groups. Death caused by sepsis was more prevalent in the LN group. Serositis and neurological manifestations occurred less frequently in LN patients diagnosed after 2005. The use of mycophenolate mofetil became more common, and the cumulative corticosteroid dose decreased. The SLICC Damage Index score also decreased. Conclusions: Our study demonstrated that the disease course has changed in recent years, and the main therapeutic goal in both SLE and lupus nephritis should be to achieve remission because this significantly improves long-term prognosis and patient survival.

Genetic susceptibility and clinical features of CYP2D6 associated with systemic lupus erythematosus in a Chinese population.

This study aims to explore possible susceptibility genes and clinical features for systemic lupus erythematosus (SLE) patients in a Chinese population. Expanding on the results of a prior single-center observational study involving 60 systemic lupus erythematosus patients, a subsequent single-center prospective observational study was conducted on SLE patients undergoing treatment at Nanfang Hospital Affiliated to Southern Medical University from 2021 to 2023. The identification process for drug-related target genes entailed an extensive search across PharmGKB (https://www.pharmgkb.org/), the Clinical Pharmacogenetics Implementation Consortium (CPIC),and PubMed literature databases, to pinpoint common drugs and target single nucleotide polymorphisms(SNPs)for SLE. Blood samples were individually collected and genotyped using MassARRAY® high-throughput nucleic acid mass spectrometry. Genotype frequency differences were assessed through Chi-square tests against both the larger East Asian population as well as kidney transplant recipients. Data collection relied on electronic medical records, encompassing demographic details(age, gender),medication regimens(hormones, NSAIDs, hydroxychloroquine, DMARDs, biologic agents, stomach medications, calcitriol, etc.),laboratory indicators(RF, Anti-CCP antibody, ESR, CRP, anti-ANA antibodies, dsDNA antibodies, anti-SM antibodies, S m. RNP antibodies, A LT, ALB, CR, UA, WBC, PLT, HGB, Ca, K, Glu, CHOL, TG, LDL-C, HDL-C) and lupus activity scores(SLEDAI-2K). Possible disease susceptibility genes were categorized, and SPSS26 software facilitated statistical analyses. The research encompassed a total of 137 SLE patients along with 50 SNPs. After conducting statistical analyses, it emerged that there existed significant disparities in CYP2D6 gene (rs1065852) distribution when compared against allele mutation rates within both East Asian populations (p < .05) and kidney transplant patients(p < .05). Wild-type gene (GG) constituted 14% of cases while mutant gene (GA + AA) constituted 86%. Allele mutation rate (A63.6%) was significantly higher among SLE patients (RR = 0.802; p = .0355). Furthermore, the variant rs1065852 genotype (GA + AA) demonstrated significant associations with lower CRP levels, higher HGB levels, and higher HDL-C levels (p < 0 0.05). The metabolic enzyme CYP2D6 may be used as susceptibility gene for predicting systemic lupus erythematosus and are correlated with CRP and other indicators.

Systematic analysis of serum peptidase inhibitor 3 in psoriasis diagnosis and treatment.

Psoriasis is a chronic inflammatory skin disease. To date, there are no serum biomarkers for psoriasis that have been validated to diagnose or treat psoriasis. Peptidase inhibitor 3 (PI3) levels in serum were measured using chemiluminescence immunoassay (CLIA) in two independent cohorts including healthy controls (HC) and patients diagnosed with chronic urticaria (CU), chronic eczema (CE), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), psoriatic arthritis (PsA), or psoriasis vulgaris (PV). Receiver operating characteristic (ROC) curve analysis determined the diagnostic performance of PI3 in patients with psoriasis. The correlation between PI3 levels and the Psoriasis Area Severity Index (PASI) score was analyzed using the Spearman correlation method. Additionally, the study evaluated PI3 expression and treatment response of PV patients 12weeks before and after topical treatment with calcipotriol betamethasone and calcipotriol ointment (T#1) or topical therapy plus PSORI-CM01 granules (T#2). In cohort #1, PI3 levels effectively discriminate PV patients from HC and CU patients, with AUCs of 0.909 and 0.840, respectively. In cohort #2, AUCs for detecting PV patients among HC, CU, CE, SLE, and RA patients were 0.940, 0.926, 0.802, 0.989, and 0.951, respectively. For PsA patients, AUCs were 0.989, 0.986, 0.910, 1.000, and 0.984 compared to HC, CU, CE, SLE, and RA patients, respectively. In both cohorts, PI3 levels correlated significantly with PASI scores in PV patients (cohort #1, r = 0.433; cohort #2, r = 0.634) and PsA patients (cohort #2, r = 0.718). Moreover, univariate logistic regression analyses revealed that PV patients with higher PI3 expression had a significantly higher risk of treatment resistance, with an odds ratio of 3.45 [95% confidence interval (CI) 1.54, 7.74, p = 0.003]. Finally, PI3 levels decreased nearly 35-fold more in the responder than in the non-responder group before and after treatment. Serological PI3 is a reliable biomarker for PV diagnosis and may have the potential to predict and monitor the progression of PV before and after treatment. Key Points • This study validated PI3's diagnostic performance in two independent psoriasis cohorts using CLIA. • PI3 expression is significantly correlated with the psoriasis severity and with patients who benefited from the treatments. • Serological PI3 is a reliable biomarker for psoriasis diagnosis and may have the potential to monitor the psoriasis progression with and without treatments.

Impact of perception of illness on quality of life in juvenile systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs, notably the skin, joints, and kidneys. The primary goal in managing SLE is to enhance patients' quality of life (QoL). Illness perception can influence QoL in patients with chronic disease. We assessed illness perception in juvenile SLE (jSLE) patients and its effect on patient's and parental QoL. Patients diagnosed with jSLE according to the SLICC 2012 criteria between January and November 2023 were included. Patients' illness perceptions were gaged using the brief illness perception questionnaire (B-IPQ), while patient's and parental QoL were evaluated using PedsQL and WHOQOL- BREF, respectively. The study comprised 32 patients and 32 parents, predominantly female (78.1%). Musculoskeletal involvement was the most common (65.6%), followed by mucocutaneous (59.4%), renal, and hematological involvement (50% each). Neuropsychiatric involvement was absent. The median SLEDAI-2K score at the last outpatient clinic visit, which was documented in the patient's file was 2 (0-18) and was not correlated with the B-IPQ score (r = 0.121, p = .51). A significant negative correlation was found between B-IPQ and patient QoL, indicating poorer QoL in patients with negative illness perceptions (r = -0.576, p < .001). No correlation was observed between parental QoL and B-IPQ (p => .05). Of note, 56.3% of patients had poor QoL, scoring below the PedsQL cut-off, while 43.8% of parents had poor QoL for general health, scoring below the WHOQOL-BREF cut-off for general health. Although disease perception did not correlate with disease activation in jSLE, it significantly impacted patient QoL. Enhancing patients' perceptions of jSLE may improve their overall QoL.

Clinical and laboratory pattern of patients with systemic lupus erythematosus seropositive for rheumatoid factor.

The aim of the study was to investigate the associations between the presence and level of rheumatoid factor (RF) in the blood serum and the clinical and laboratory characteristics of patients with systemic lupus erythematosus (SLE). This retrospective tricentric cross-sectional study analyzed a Ukrainian contingent of SLE patients. Medical records of 495 patients were evaluated. Rheumatoid factor serum concentration was tested in 206 of them (41.6%) using turbidimetry technique. Clinical manifestations, routine laboratory parameters, specific immunological tests, disease activity (SLEDAI-2K), and damage indices (SLICC/ACR DI) were evaluated. Our study revealed that RF was elevated in 27.7% of patients. The RF-positive patients experienced a longer delay in SLE diagnosis (2.0 vs. 0.5 years, p = 0.046), less frequent kidney involvement (42.1% vs. 59.4%, p = 0.045) and fever (42.1% vs. 59.2%, p = 0.046), and more frequent lymphadenopathy (59.6% vs. 42.3%, p = 0.039) compared to RF-negative patients. Patients with RF positivity had higher levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer, and were more frequently positive for antibodies to Ro/SSA and La/SSB. Rheumatoid factor concentration directly correlated with CRP (r = 0.318; p < 0.01) and ESR (r = 0.228; p = 0.04) levels. However, no associations were found between RF levels and SLEDAI-2K, joint involvement frequency, SLICC/ACR DI or drug therapy content. Univariate logistic regression analysis showed that RF positivity was independently associated with lymphadenopathy, presence of anti-Ro/SSA and anti-La/SSB antibodies, and negatively associated with kidney involvement. In RF-seropositive SLE patients (approximately 28%), the diagnosis is established later compared to RF-seronegative ones; kidney involvement and fever are less common, while lymphadenopathy develops more frequently. Rheumatoid factor seropositivity is associated with higher levels of ESR, CRP, ANA, and the presence of antibodies to Ro/SSA and La/SSB. According to the results of univariate logistic regression analysis, an independent association with RF positivity was confirmed only for kidney involvement, lymphadenopathy, and antibodies to Ro/SSA and La/SSB.

IRF5 Variants Are Risk Factors for Systemic Lupus Erythematosus in Two Mexican Populations.

Interferon regulatory factor 5 ( IRF5 ) is one of the pivotal genes implicated in systemic lupus erythematosus (SLE) among diverse ethnic groups, including Europeans, Asians, Hispanics, and Africans. Notably, its significance appears particularly pronounced among Hispanic populations. Previous studies have identified several single-nucleotide variants within IRF5 , such as rs2004640G/T, rs2070197T/C, and rs10954213G/A, as associated with susceptibility to SLE among patients from Mexico City. However, the population of Yucatan, located in the Southeast of Mexico and characterized by a greater Amerindian genetic component, remains largely unexplored in this regard. Our study aimed to replicate the observed association between IRF5 variants and susceptibility to SLE among patients from Central Mexico and Yucatan. Furthermore, we investigated the impact of IRF5 rs59110799G/T, a variant that has not been previously studied in SLE individuals. Our study included 204 SLE patients and 160 controls from Central Mexico, as well as 184 SLE patients and 184 controls from Yucatan. All participants were females 18 years and older. We employed a TaqMan assay to detect the presence of the following single-nucleotide variants: rs2004640G/T, rs2070197T/C, rs10954213G/A, and rs59110799G/T. Furthermore, we utilized 2 distinct web tools and databases to predict the potential functional implications of IRF5 variants. In SLE patients from Central Mexico, several IRF5 alleles showed significant associations with the disease following adjustment by the Bonferroni test: the rs2070197C allele (odds ratio [OR], 2.08), the rs10954213A allele (OR, 1.59), and the rs59110799G allele (OR, 1.71). Conversely, among patients from Yucatan, the following alleles showed associations: rs2004640T (OR, 1.51), rs2070197C (OR, 1.62), rs10954213A (OR, 1.67), and rs59110799G (OR, 1.44). Our findings highlight genetic variations between Mexican populations and emphasize the role of IRF5 as a risk factor in SLE patients from both Central Mexico and Yucatan.

Incidence and risk of arterial and venous thrombotic events in Systemic Lupus Erythematosus patients: a population-based study.

Current data on arterial and venous thrombotic events (ATE & VTE) and cardiovascular (CV) risk management in European systemic lupus erythematosus (SLE) population are limited. This study aimed to investigate the incidence and risk of thrombotic events and all-cause death in an Italian SLE cohort over the past decade, along with its pharmacotherapy. Incident SLE cases between 2010 and 2019 were identified using administrative health databases of the Lombardy Region. The association between SLE and outcomes, compared with age- and sex-matched controls, was reported as incidence rate per 1000 person-years and as adjusted hazard ratios with 95% confidence intervals. Overall, 2133 SLE patients and 21 283 no-SLE individuals were included. A higher incidence rate of ATE (4.22 vs 2.26 1000 PY), VTE (1.85 vs 0.67 1000 PY,) and all-cause death (15.18 vs 6.22 1000 PY) was reported in SLE patients than in those without (p< 0.0001) as well as an increased risk of ATE (HR, 1.65; 95% CI, 1.20-2.26), VTE (HR, 2.25; 95% CI, 1.35-3.74), and all-cause death (HR, 1.81; 95% CI, 1.52-2.15). After SLE diagnoses, hydroxychloroquine and glucocorticoids were prescribed for at least 60% of patients. Additionally, a higher exposure to cardiovascular medications was also seen in SLE patients. Our findings confirmed higher risks of ATE, VTE and all-cause death in SLE patients. While increased CV medications use after SLE diagnoses suggests heightened awareness to CV risk profile, more attention is required to balance SLE disease activity with minimizing exposure to drugs associated with exacerbating CV risk.