Features Of Systemic Lupus Erythematosus Research Articles

Childhood-onset systemic lupus erythematosus: A descriptive and comparative study of clinical, laboratory, and treatment characteristics in two populations.

The aim of this study was to characterize childhood-onset systemic lupus erythematosus (SLE) in two large cohorts from Turkey and the United States. Patients diagnosed with childhood-onset SLE who fulfilled the 1997 American College of Rheumatology classification criteria for SLE from four reference centers in Turkey and the University of Pittsburgh School of Medicine in the United States were included in this study. A comparative analysis was conducted to evaluate the similarities and differences in clinical and laboratory features, damage accrual, and treatment experiences between the two populations. A total of 174 patients with childhood-onset SLE were included in this study (108 patients from Turkey and 66 patients from the United States). The female-to-male ratio was similar between the two cohorts (∼3:1, p = .73). The median age at diagnosis was 11.67years (2.19-17.93) in the Turkish cohort and 13.68years (2.74-17.93) in the U.S. cohort (p < .001). Photosensitivity (45.4% and 21.2%; p = .007) and renal involvement (41.7% and 36.4%; p = .045) were higher in the Turkish cohort. Anti-Ro/SSA (34.8% and 15.7%; p < .001), anti-Sm (59.1% and 19.4%; p < .001), and anti-RNP (47.0% and 14.8%; p < .001) positivity was more frequent in the U.S. cohort. Current use of rituximab (37.9% and 1.9%; p < .001) and belimumab (19.7% and 0%; p < .001) was more prevalent in the U.S. cohort, while the use of cyclophosphamide (often according to the low dose Euro-Lupus protocol) throughout the disease course (24.1% and 4.5%; p < .001) was more frequent in the Turkish cohort. SLICC/ACR Damage Index scores were not different between the two cohorts. This study provides detailed clinical and laboratory features of childhood-onset SLE in two independent and geographically divergent cohorts. Our findings suggest an earlier age of disease onset and a higher prevalence of kidney involvement in Turkish patients. Differences in treatment approaches were also noted. However, damage accrual related to SLE does not appear to be different between the two patient populations.

Psychological impact of life events in systemic lupus erythematosus patients - Differences between flares and remission.

Stress has been linked to worsening symptoms and increased disease activity in patients with Systemic lupus erythematosus (SLE). Life-events are individual stress points, and there is conflicting evidence regarding their role in SLE activity and disease perception. Adult SLE patients were recruited for the study. Clinical and laboratory features of SLE were recorded, and previous diagnosis of anxiety or depression were retrieved from patients' electronic charts. Flares were defined by the Systemic Lupus Erythematosus Disease Activity (SLEDAI) flare Index, and flares during the previous year were documented. During a routine visit, they completed validated Portuguese translations of the 10-item Perceived Stress Scale (PSS-10), Hospital Anxiety and Depression Scale (HADS) and Life Experience Survey (LES) for the previous year. A total of 47 female SLE patients were recruited. Ten patients (21.3%) had experienced recent flares. Patients with recent flares reported fewer life events, with lower positive, negative, and total weightings sums compared to those without recent flares. Although 42.2% of patients perceived pathological levels of stress in the previous month, 48.9% had anxiety symptoms, and 34% were at high risk for an anxiety disorder, these psychometric measures did not differ significantly between the recent flare and no-flare groups. There is a high prevalence of pathological levels of stress among SLE patients. SLE patients with recent flares report less psychological impact from life events, both positive and negative, independent of other psychological or pharmacological factors.

Digital ischemic necrosis in a patient with systematic lupus erythematosus patient after severe acute respiratory syndrome coronavirus 2 infection: a case report

BackgroundPatients with coronavirus disease 2019 have a high incidence of thrombosis that decreases after recovery. When coronavirus disease 2019 is accompanied by diseases prone to thrombosis, risk of post-infection thrombotic events may increase.Case presentationWe report a case of digital ischemic gangrene in a 24-year-old Chinese female with systemic lupus erythematosus after recovery from coronavirus disease 2019. The pathogenesis was related to clinical characteristics of systemic lupus erythematosus, hypercoagulability caused by coronavirus disease 2019, and second-hit due to viral infection.ConclusionPatients with autoimmune diseases should remain alert to autoimmune system disorders induced by severe acute respiratory syndrome coronavirus 2 and other viruses. Treatment for these patients should be strictly standardized, and appropriate anticoagulation methods should be selected to prevent thrombosis.

Tumid lupus erythematosus in C1-inhibitor deficiency.

A case of a 28-year-old woman with known C1-inhibitor deficiency (functional, Type II) with persistent bilateral non-pruritic, mildly photosensitive facial rash for 10 months following delivery of her second child is presented. Histology of the skin was suggestive of tumid lupus erythematosus (LE), but no other features of systemic LE (ANA, dsDNA negative) were evident. She had stopped danazol which was controlling the underlying disease, and once this was restarted and treatment for tumid lupus was started, she improved. More rigorous control preventing all C1-inhibitor deficiency-related attacks proved successful. The hypothesis that uncontrolled classical pathway complement activation that led to the lupus-like skin lesions is being presented as a clinical case, highlighting the complex interrelationships between immunodeficiency and autoimmunity in inborn errors in immunity.

Regulatory T cells expressing CD19-targeted chimeric antigen receptor restore homeostasis in Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is a progressive disease leading to immune-mediated tissue damage, associated with an alteration of lymphoid organs. Therapeutic strategies involving regulatory T (Treg) lymphocytes, which physiologically quench autoimmunity and support long-term immune tolerance, are considered, as conventional treatment often fails. We describe here a therapeutic strategy based on Tregs overexpressing FoxP3 and harboring anti-CD19 CAR (Fox19CAR-Tregs). Fox19CAR-Tregs efficiently suppress proliferation and activity of B cells in vitro, which are relevant for SLE pathogenesis. In an humanized mouse model of SLE, a single infusion of Fox19CAR-Tregs restricts autoantibody generation, delay lymphopenia (a key feature of SLE) and restore the human immune system composition in lymphoid organs, without detectable toxicity. Although a short survival, SLE target organs appear to be protected. In summary, Fox19CAR-Tregs can break the vicious cycle leading to autoimmunity and persistent tissue damage, representing an efficacious and safe strategy allowing restoration of homeostasis in SLE.

Exploring the Perspectives of Patients Living With Lupus: Retrospective Social Listening Study.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease affecting various organs with a wide range of clinical manifestations. Cutaneous lupus erythematosus (CLE) can manifest as a feature of SLE or an independent skin ailment. Health-related quality of life (HRQoL) is frequently compromised in individuals living with lupus. Understanding patients' perspectives when living with a disease is crucial for effectively meeting their unmet needs. Social listening is a promising new method that can provide insights into the experiences of patients living with their disease (lupus) and leverage these insights to inform drug development strategies for addressing their unmet needs. The objective of this study is to explore the experience of patients living with SLE and CLE, including their disease and treatment experiences, HRQoL, and unmet needs, as discussed in web-based social media platforms such as blogs and forums. A retrospective exploratory social listening study was conducted across 13 publicly available English-language social media platforms from October 2019 to January 2022. Data were processed using natural language processing and knowledge graph tagging technology to clean, format, anonymize, and annotate them algorithmically before feeding them to Pharos, a Semalytix proprietary data visualization and analysis platform, for further analysis. Pharos was used to generate descriptive data statistics, providing insights into the magnitude of individual patient experience variables, their differences in the magnitude of variables, and the associations between algorithmically tagged variables. A total of 45,554 posts from 3834 individuals who were algorithmically identified as patients with lupus were included in this study. Among them, 1925 (authoring 5636 posts) and 106 (authoring 243 posts) patients were identified as having SLE and CLE, respectively. Patients frequently mentioned various symptoms in relation to SLE and CLE including pain, fatigue, and rashes; pain and fatigue were identified as the main drivers of HRQoL impairment. The most affected aspects of HRQoL included "mobility," "cognitive capabilities," "recreation and leisure," and "sleep and rest." Existing pharmacological interventions poorly managed the most burdensome symptoms of lupus. Conversely, nonpharmacological treatments, such as exercise and meditation, were frequently associated with HRQoL improvement. Patients with lupus reported a complex interplay of symptoms and HRQoL aspects that negatively influenced one another. This study demonstrates that social listening is an effective method to gather insights into patients' experiences, preferences, and unmet needs, which can be considered during the drug development process to develop effective therapies and improve disease management.

Neuropsychological profiles comparison between Multiple Sclerosis patients and Multiple Sclerosis patients with overlapping features of systemic lupus erythematosus

AimMultiple sclerosis (MS) and Systemic Lupus Erythematosus (SLE) are autoimmune disorders that may lead to cognitive impairment. This study aimed to compare the neuropsychological profiles of patients with MS, and MS and coexisting SLE features. MethodsWe included a total of 90 participants, divided into 3 groups: 30 patients with clinically definite relapsing remitting MS, 30 with coexisting MS and incomplete SLE (overlap group) and 30 healthy controls (HC). All participants underwent neuropsychological assessment with the Montreal Cognitive Assessment (MoCA), Symbol Digit Modalities Test (SDMT), Paced Auditory Serial Addition Test (PASAT), and Selective Reminding Test (SRT). ResultsBoth groups scored lower on the MoCA compared to the HC (p < .001). The overlap group showed the lowest performance on the SDMT and PASAT compared to the other two groups (p < .01), while the MS group scored similarly to the HC in the PASAT (p > .05). Regarding the learning rate and long-term recall, the overlap group had lower scores compared to both the MS and HC (p < .001), but it outperformed both groups in the retention efficacy score (p < .001). The MS group did not differ significantly from the HC in these memory domains (p > .05). ConclusionThe overlap group exhibited a broader range of impairments, including slower processing speed, decreased working memory, reduced learning rate, and long-term retrieval deficits. Their retention ability remained intact. The coexistence of MS with SLE pathology had additive impacts on cognitive function.

Genetically predicted 486 blood metabolites concerning risk of systemic lupus erythematosus: a Mendelian randomization study

Metabolic abnormalities constitute a significant characteristic of systemic lupus erythematosus (SLE). We utilised a two-sample Mendelian randomisation (MR) study to evaluate the potential causal association between 486 blood metabolites and SLE. Exposure data at the metabolite level were extracted from 7824 European Genome-wide association studies (GWAS). Preliminary analysis utilised SLE GWAS data from FinnGen. The primary method for causal analysis relied on random inverse variance weighting (IVW). To ensure robustness, sensitivity analyses included the Cochran Q test, MR-Egger intercept test, MR-PRESSO, and leave-one-out analysis. Steiger testing and linkage disequilibrium score regression were employed to validate the identified metabolites. This study identified 12 metabolites, comprising six known chemical structures: 1,5-anhydroglucitol(1,5-AG) [odds ratio (OR) = 0.100, 95% confidence interval (CI): 0.015–0.773, P = 0.027), gamma-glutamylthreonine (OR = 0.077, 95% CI: 0.010–0.574, P = 0.012), 5-dodecenoate(12:1n7) (OR = 0.205, 95% CI: 0.061–0.685, P = 0.010), linoleoylglycerophosphoethanolamine * (OR = 0.159, 95% CI: 0.027–0.933, P = 0.044), erythrose (OR = 88.331,95% CI:1.098–63.214, P = 0.040) and 1-, adrenate (22:4n6) (OR = 9.876, 95% CI: 1.753–55.639, P = 0.001)]. Additionally, we found associations between SLE and six unknown chemical structures: X-06351 (OR = 0.071, 95% CI: 0.006–0.817, P = 0.034), X-10810 (OR = 4.268 95% CI: 1.260–14.459, P = 0.020), X-11412 (OR = 5.418 95% CI: 1.068–27.487, P = 0.041), X-11905 (OR = 0.551, 95%CI: 0.304–0.997, P = 0.049), X-12038 (OR = 0.178 95%CI: 0.032–0.988, P = 0.045), X-12217 (OR = 0.174 95%CI: 0.044–0.680, P = 0.014). This study offers evidence supporting a causal relationship between SLE and 12 circulating metabolites, six of which have known chemical structures and six that remain unidentified. These findings introduce a new perspective for further exploration of SLE mechanisms.

Autoimmune Haemolytic Anaemia as Initial and Only Presentation of Systemic Lupus Erythematosus: A Case Report

Autoimmune haemolytic anaemia (AIHA) is an acquired disorder resulting in the presence of antibodies against red blood cell antigens which leads to intravascular haemolysis. It can occur as the initial and sometimes the only presenting feature of Systemic Lupus Erythematosus (SLE). In this report, we describe the diagnostic evaluation and ongoing management of a 13-year-old female with AIHA as the first clinical feature of SLE. She presented with paleness of the body and yellowish discoloration of the eyes. Initial examination showed severe pallor with moderate jaundice. Investigations revealed severe anaemia with slightly elevated reticulocyte count and positive direct coombs test(DCT). Peripheral blood film showed microcytic, hypochromic anaemia. Bone marrow aspiration showed a hypocellular marrow for the age of the child. Immunological screening results were positive for antinuclear antibody (ANA) and anti-double stranded DNA (Anti-ds DNA). Patient is currently on Prednisolone, hydroxychloroquine and mycophenolate mofetil(MMF). The diagnosis of SLE with AIHA requires a good history, physical examination, high index of suspicion and extensive investigations. Management entails multidisciplinary approach, supportive care, initiation of treatment with steroids and immunosuppressive drugs if necessary to minimize morbidity and mortality.

Patients with systemic lupus erythematosus who are underweight have distinct disease characteristics.

This study aimed to detail the disease characteristics of systemic lupus erythematosus (SLE) in individuals who are underweight and assess whether underweight status is associated with SLE disease activity. This was a retrospective cohort study involving 218 patients newly diagnosed with SLE. Patients were categorized as underweight (body mass index [BMI] <18.5kg/m2) or not underweight (BMI ≥18.5kg/m2). We reviewed disease characteristics including the SLE Disease Activity Index 2000 (SLEDAI-2K) at diagnosis. High disease activity was defined as SLEDAI-2K ≥10. Disease characteristics were compared between those who were underweight and not underweight. We used multivariable logistic regression analysis to determine whether underweight status is associated with high disease activity. Out of the 218 patients, 35 (16.1%) were underweight and 183 (83.9%) were not. Underweight patients had less renal involvement (5.7% vs 20.2%, p = .040), lower C-reactive protein levels (1.0 [0.3-2.3] mg/L vs 1.2 [0.8-5.0] mg/L, p = .028), and lower SLEDAI-2K scores (6.7 ± 4.6 vs 9.1 ± 5.7, p = .009), and were less likely to be at high disease activity status (22.9% vs 42.6%, p = .028), compared with those who were not underweight. Following adjustment for multiple covariates, being underweight was inversely associated with high disease activity status (adjusted odds ratio = 0.38, 95% confidence interval = 0.16 to 0.92, p = .031). Patients with SLE who were underweight showed less renal involvement and lower SLEDAI-2K scores compared with those who were not underweight. Moreover, those with SLE who were underweight had a 60% lower risk of exhibiting high disease activity.

Особливості ювенільного Rhupus-синдрому з ураженням нирок: аналіз літератури та власний досвід

Rhupus syndrome is a rare coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), characterized by symmetrical erosive polyarthritis and permanent deformities in addition to the clinical and serological features of SLE. Its prognosis is further complicated by neurological, and/or hematological, nephrological lesions, which sharply reduce the quality of life of patients. Clinical signs and consequences of Rhupus syndrome may differ in children and adults. Purpose - to acquaint doctors with the peculiarities of diagnosis, treatment, and observation of children with Rhupus syndrome, the importance of early diagnosis of complicated courses and disabilities. Clinical case. The authors present a clinical case of a 15-year-old girl with severe arthritis, which manifested simultaneously with clinical and serological signs of systemic lupus and developed secondary nephropathy at an early stage. JIA with the presence of specific autoantibodies to SLE in this case confirms the diagnosis of Rhupus. The child was treated with a disease-modifying antirheumatic drug (Methotrexate), steroids (Methylprednisolone), an angiotensin II receptor blocker, and other drugs in the treatment complex. Conclusions. Accurate diagnosis is the key to the correct diagnosis and choice of treatment strategy, as Rhupus syndrome is a very rare disorder with a very limited number of reported cases, but with the early realization of possible complications, such as kidney damage. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Diagnostic Values of METTL1-Related Genes and Immune Characteristics in Systemic Lupus Erythematosus.

Methyltransferase like 1 (METTL1) regulates epitranscriptomes via the m7G modification in mammalian mRNA and microRNA. Systemic lupus erythematosus (SLE) is caused by abnormal immune reactivity and has diverse clinical manifestations. RNA methylation as a mechanism to regulate gene expression is widely implicated in immune regulation. However, the role of m7G in immune response of SLE has not been extensively studied. Expression of METTL1 was identified in the public dataset GSE122459 and validated in an independent cohort of SLE patients. We investigated the association between METTL1-expression and clinical manifestations of SLE. Subsequently, differentially expressed genes (DEG) that were correlated with METTL1-expression in GSE122459 were used for functional enrichment analysis. The correlation between infiltrating immune cells and METTL1, as well as candidate biomarkers identified to be correlated with either METTL1 or immune cell infiltration were assessed by single-sample GSEA. Potential mechanisms were explored with Gene ontology and KEGG pathway enrichment. Diagnostic performances of candidate biomarkers in SLE were analyzed. The mRNA and protein expression of METTL1 in SLE patients were significantly decreased in both datasets. METTL1-coexpressed DEGs were enriched in several key immune-related pathways. Activated CD8 T cells, activated CD4 T cells, memory B cells and type 2 helper T cells were different between patients with high and low METTL1 expression. Further, activated CD8 T-cells, activated CD4 T-cells, memory B-cells were correlated with METTL1. The genes of LAMP3, CD83, PDCD1LG2, IGKVD3D-20, IGKV5-2, IGKV2D-30, IGLV3-19 and IGLV4-60 were identified as candidate targets that were correlated with immune cell proportion. Moreover, LAMP3, CD83, and PDCD1LG2 expression were of diagnostic value in SLE as indicated by ROC analysis. Our findings suggested that METTL1 and its candidate targets LAMP3, CD83, PDCD1LG2 may be used for diagnosing SLE and could be explored for developing targeted molecular therapy for SLE.

Abstract 13 — Comparison of Clinical and Serological Features in Juvenile and Adult-Onset Systemic Lupus Erythematosus

Background Systemic lupus erythematosus (SLE) has varying clinical and serological features in juvenile and adult-onset disease groups, depending on the ethnicity. Objectives: To compare the disease characteristics of patients with adult-onset (ASLE) and juvenile-onset systemic lupus erythematosus (JSLE). Methods It is a cross-sectional study, carried out at Rheumatology department Fauji Foundation Hospital. 184 SLE patients were included. They were divided into adult (n=152) and juvenile (n=32) group. The clinical and serological features at the time of disease onset were compared between the two groups. Statistical analysis was done using chi square and t-test. Results The mean age was 36.22 ± 11.48 in adults and 19.94 ± 7.17 in the juvenile group (t = 7.71, p&lt;0.001). The duration of disease was 46.05 ± 45.74 months(adult) and 62.43 ± 84.00 months (juvenile). There was no significant difference in disease activity, SLE disease activity index (SLEDAI) was 13 in adult lupus patients and 14.5 in the juvenile, t-value=1.1 and p-value=0.39. Sicca symptoms were significant in the ASLE (p=0.004). Neuropsychiatric manifestations (p=0.03), thrombocytopenia (p=0.048) and low complement levels (0.02) were significantly higher in JSLE. Serological profiles did not differ significantly in the two groups. Conclusion The features of SLE differ in adult and juvenile patients. Neurological involvement and thrombocytopenia were more prevalent in JSLE, whereas sicca symptoms were more frequently found in ASLE.

COX5A as a potential biomarker for disease activity and organ damage in lupus.

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with limited therapeutic targets or clinical outcome predictors. This study aimed to gain more insights into the underlying immunological pathways and prognostic biomarkers of SLE. Integrated analyses of RNA-seq data from 64 SLE and 62 healthy controls, examining 27 immune cell types to explore the key pathways and driver genes in SLE pathogenesis. Single-cell RNA sequencing data from the skin and kidney were used to determine the association of COX5A expression with organ damage. The associations of COX5A with SLE phenotypes were further evaluated in two independent cohorts, and receiver operating characteristic (ROC) curves were constructed to assess the value of COX5A as a biomarker for disease activity and organ damage in SLE. We found that oxidative phosphorylation (OXPHOS) is the most significantly altered metabolic pathway in SLE, especially in effector T cells. Notably, we identified an OXPHOS-related enzyme, COX5A, whose expression was significantly higher in effector T cells than in naïve T cells and showed associations with disease activity, organ damage, and steroid treatment of SLE. Furthermore, ROC curves showed that COX5A is a robust biomarker for disease activity, kidney involvement, and new-onset skin lesions, with the area under the curve (AUC) values of 0.880, 0.801, and 0.805, respectively. Our results identified the OXPHOS signature as a prominent feature in SLE T cells, and COX5A as a potential candidate biomarker for disease activity and organ damage in SLE.

Mononuclear cells from patients with rhupus are influenced by TNF in their production of gp130/sIL-6Rb and APRIL

ObjectiveRhupus is a rare disease that shares characteristics of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). While several studies have explored the clinical and immunological profiles of patients with...

In vitro evaluation of the potential immunosuppressive effect of panobinostat on cultured lymphocytes retrieved from childhood systemic lupus erythematosus patients

Although many drugs are available for childhood systemic lupus erythematosus (SLE) treatment, the adverse effects and poor response in some cases make it crucial to find new drugs targeting various pathways in disease pathogenesis to improve overall outcomes. This study aimed to (i) investigate the effect of Panobinostat on cultured lymphocytes obtained from children with active SLE and (ii) to compare that effect with standard drugs used in SLE, such as Prednisone and hydroxychloroquine. The study included 24 SLE active patients, divided into four equal groups. Lymphocytes were isolated from blood samples of the study patients. According to the study group, cells were treated with either Panobinostat, Prednisolone, hydroxychloroquine, or not treated (control group). After cell culture, the response of lymphocytes upon drug treatment was analyzed in terms of the production of anti-dsDNA antibodies and levels of apoptosis as detected by flow cytometry using annexin V and propidium iodide (PI) staining. The Panobinostat group showed a significant decrease in the viable cell count (p&lt;0.001). Both Prednisone and hydroxychloroquine decreased anti-dsDNA expression more than the Panobinostat and control groups (p&lt;0.001 for both). PI was higher in the Prednisone group, and Annexin V was higher in the Panobinostat group compared to other groups; however, their increase did not reach statistically significant levels (p= 0.12 and 0.85, respectively). This is the first study of the Panobinostat effect on cultured lymphocytes of SLE. In conclusion, Panobinostat could be a prospective treatment for B-cell-driven autoimmune diseases such as SLE. However, its effect on autoantibodies levels and different clinical features of SLE still need a thorough evaluation.

Relationship between Disease Characteristics and Circulating Interleukin 6 in a Well-Characterized Cohort of Patients with Systemic Lupus Erythematosus.

Interleukin-6 (IL-6) is a proinflammatory cytokine that mediates pleiotropic functions in immune responses and inflammatory diseases. The literature lacks studies, with a clinical perspective, on the relationship between IL-6 serum levels and the characteristics of the disease in patients with systemic lupus erythematosus (SLE). In the present work, we aimed to analyze the association between circulating IL-6 and disease manifestations in a well-characterized series of patients with SLE. Serum IL-6 levels and disease activity (SLEDAI-2K), severity (Katz) and damage index (SLICC-DI), complete lipid profile, and subclinical carotid atherosclerosis were evaluated in 284 patients with SLE. In addition, a complete characterization of the complement system was performed in samples from patients with SLE. A multivariate linear regression analysis was carried out to study the relationship between clinical and laboratory characteristics of the disease and IL-6 levels. Age (beta coef. 0.07 [95%CI 0.01-0.1] pg/mL, p = 0.014), C-reactive protein (beta coef. 0.21 [95%CI 0.16-0.25] pg/mL, p < 0.01), and male gender (beta coef. 2 [95%CI 0.3-0.5] pg/mL, p = 0.024), were positively associated with higher IL-6 levels in SLE patients. Most disease characteristics and damage and activity indices did not show significant relationships with IL-6. However, after multivariate analysis, IL-6 was associated with lower serum levels of HDL cholesterol (beta coef. -0.04 [95%CI -0.08-(-0.1)] pg/mL, p = 0.011), and apolipoprotein A1 (beta coef. -0.02 [95%CI -0.04-(-0.001)] pg/mL, p = 0.035). In contrast, the alternative complement cascade, C1inh, and C3a were all positively and independently associated with higher serum levels of IL-6. Moreover, stratification of the Systematic Coronary Risk Assessment 2 (SCORE2) results according to different categories of cardiovascular risk was associated with higher circulating serum IL-6 levels (beta coef. 0.2 [95%CI 0.02-0.4], pg/mL, p = 0.028). In conclusion, in a large series of SLE patients, IL-6 was not associated with disease-related features of SLE, including damage, severity, or activity indices. However, an association was found between serum IL-6 levels and circulating C3a and cardiovascular risk. Our study emphasizes the importance that IL-6 could have in cardiovascular disease and complement system disruption of SLE patients. Therapies targeting IL-6 could have a role in these two clinical manifestations of patients with SLE.

Autoantibody status according to multiparametric assay accurately estimates connective tissue disease classification and identifies clinically relevant disease clusters

ObjectiveAssessment of circulating autoantibodies represents one of the earliest diagnostic procedures in patients with suspected connective tissue disease (CTD), providing important information for disease diagnosis, identification and prediction of potential...

Systemic lupus erythematosus masquerading as gangrene − an infrequent presentation

Systemic lupus erythematosus (SLE) is an auto-immune disease affecting multiple organs. Among the cutaneous features, digital gangrene is considered to be very rare, occurring in only 1.3% of SLE patients. It is considered to be due to compromised perfusion of digits secondary to vasculitis, vasospasm and thomboembolism. This is the case report of a 35-year-old female who presented with acute blackish discoloration of toes of both feet associated with pain. She had no other features of SLE. But serology came out positive for SLE. The patient responded well to steroids, hydroxychloroquine, antiplatelet and anticoagulant medications and is currently under follow-up. This case report basically shows us that SLE should be considered as a possible differential diagnosis whenever there is a young patient presenting with digital gangrene without any other medical history.

Clinical and immunologic features of the phenotype of systemic lupus erythematosus combined with Sjögren's syndrome

The problem of clinical and immunological heterogeneity of systemic lupus erythematosus (SLE) is of great interest, especially the combination of SLE and Sjögren's syndrome (SjS) determines a more favorable prognosis and specifics of therapy.Objective: to characterize the clinical and immunological features of SLE combined with SjS.Material and methods. The retrospective study included 44 patients with SLE combined with SjS and 356 patients with SLE without SjS, hospitalized at the V.A. Nasonova Research Institute of Rheumatology between 2013 and 2021. The two groups of patients were compared in terms of life expectancy, clinical manifestations, laboratory parameters and therapy.Results and discussion. The following phenotypic features of the clinical and immunological variant of SLE combined with SjS were found: significantly longer duration of SLE (p&lt;0.01); higher incidence of polyarthritis (p=0.01) and Raynaud's syndrome (p&lt;0.003) at disease onset. Subacute cutaneous lupus erythematosus, Raynaud's syndrome, peripheral nervous system involvement (sensory polyneuropathy and distal sensory-motor polyneuropathy) were found more frequently in SLE combined with SjS than in SLE without SjS, p&lt;0.0001. Among laboratory abnormalities in SLE patients with SjS, leukopenia (p&lt;0.0001), antibodies to SSA/Ro, SSB/La, and rheumatoid factor (p&lt;0.0001) were observed in a greater number of cases. In the group of SLE combined with SjS, the chronic variant of SLE course according to the classification of V.A. Nasonova, and lower activity were observed, which didn't not require therapy with high doses of glucocorticoids; in this group, rituximab (p&lt;0.01), cyclophosphamide and methotrexate were used significantly more often.Conclusion. Thus, significant clinical and laboratory differences were found between SLE with SjS and without SjS. They determine prognosis, approaches to pathogenetic therapy and monitoring.