Systemic Lupus International Collaborating Research Articles

Assessment of Interleukin 17 in Egyptian Systemic Lupus Erythematosus Patients as a Biomarker in Disease Activity.

Systemic lupus erythematosus (SLE) is a chronic idiopathic systemic autoimmune disorder with dysregulation of adaptive and innate immune systems. Interleukin (IL)-17 is the prototypical pro-inflammatory cytokine of T helper 17 (Th17) cells. Therefore, it contributes to the pathogenesis of human SLE. The aim of the research paper was the evaluation of IL-17 level as a biomarker in the SLE cohort and its relation to disease activity and analysis of IL-17 concentration in patients with lupus nephritis and non-lupus nephritis. The research enrolled 45 SLE patients according to Systemic Lupus International Collaborating Clinics Classification Criteria (SLICC), and age and sex-matched. The patients underwent full history, clinical examination, laboratory investigation, and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) calculation. The mean age ± SD of the participants equaled 32 ± 11 years, and serum IL-17 in SLE cases was statistically significantly high (p < 0.001). No statistically significant correlations were reported between disease activity according to SLEDAI and IL-17. In addition, a statistically significant positive correlation was reported between IL-17 and ESR, and a high statistically significant negative correlation was reported between IL-17 and C3 and C4 (P < 0.001). A statistically significant positive correlation was reported between IL-17 and 24-hour urinary proteins with a Pvalue of 0.01. SLE cases demonstrated higher levels of serum IL-17, contributing to SLE pathogenesis. However, no statistically significant difference was reported between IL-17 and Lupus nephritis. IL-17 and SLE activity (SLEDAI) did not correlate. A statistically significant positive relation was reported between IL-17 and 24-hour urinary proteins. Additionally, a high statistically significant negative correlation was reported between IL-17 and C3 and C4.

Blockades in the Pathway of Speciality Care in Systemic Lupus Erythematosus: A Report Based on Indian Rheumatology Association Database

Aim: The lack of knowledge among primary care physicians about autoimmune rheumatic diseases can contribute to referral delays by impeding early recognition, accurate diagnosis and timely referral to specialists. The present study is aimed at analysing the factors influencing the diagnostic and referral delay in patients with systemic lupus erythematosus (SLE) to the rheumatology speciality in India. Materials and methods: The independent, multi-centre, database-based, cross-sectional study collected data from seven centres across India through the Indian Rheumatology Association database. The patient-related factors and the referral factors were determined based on the patient narration. Based on the patient’s income, a modified version of the Prasad scale was employed to classify them according to socioeconomic status. The analysis also included the clinical presentation and system involvement. Logistic regression analysis was used to identify significant predictors of early referral based on initial symptoms. Results: The study considered 786 out of 833 patients fulfilling the Systemic Lupus International Collaborating Clinics criteria. The median delay in referral to a specialist was 1 month, with an interquartile range of 11 months, but the delay ranged from 1 to 192 months. The major causes of referral delay were the management of SLE by other specialists instead of rheumatologists (93%) and a lack of awareness of speciality care (89%). Additionally, more than 25% of primary care practitioners were unaware of rheumatology as a speciality, and a similar percentage of patients received incorrect diagnoses from their general care physician. Early referral within 6 months was associated with a lower prevalence of systemic complications, including pulmonary, gastrointestinal, peripheral vascular, central nervous system and ophthalmic involvement. Significant predictors for early referral included joint pain (49.51% vs. 19.64%; P = .013; odds ratio [OR] = 0.655), hair loss (12.76% vs. 4.76%; P = .279; OR = 0.789) and weight loss (5.05% vs. 1.12%; P = .053; OR = 0.469). Conversely, symptoms such as lung involvement (0.56% vs. 0.98%; P = .042; OR = 3.938), Raynaud’s phenomenon (0.14% vs. 0.98%; P = .002; OR = 15.847) and thrombocytopenia (0.84% vs. 1.12%; P = .035; OR = 3.002) were more likely to lead to referrals to other specialities. Conclusion: The delay in patient referral to the rheumatology speciality is primarily attributed to referrals to other specialities and a lack of knowledge about rheumatology. Educating primary care physicians and facilitating direct referrals to specialists could significantly contribute to reducing these delays.

Current smoking is related to severe damage in systemic lupus erythematosus patients

Objective To assess the relationship between smoking exposure and organ damage accrual measured by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus score (SLICC-SDI) in consecutive patients with systemic lupus erythematosus (SLE) from Argentina. Methods 623 consecutive SLE patients (fulfilling ≥4, 1997 ACR criteria) were included in this cross-sectional study. Sociodemographic and disease related variables including SLICC-SDI score and smoking status were collected. Patients currently smoking were considered “smokers”, and “non-smokers” those who never smoked and former smokers. SLICC-SDI was divided into two categories: &lt;3 and ≥3 was defined as severe damage. Results Six hundred and 23 patients were included in the analysis, 89% women. Eighty-four per cent were non-smokers and 16 % were current smokers 83 percent of patients had SLICC-SDI &lt;3 and 17 % had SLICC-SDI ≥3. Twenty one percent of patients with SLICC-SDI ≥3 and 15% with &lt;3 SLICC-SDI were current smokers ( p 0.081). In the multiple regression analysis, current smoking (OR 1.82, CI 95% 1.01-3.31, p 0.046), older age (OR 1.04, CI 95% 1.00-1.05, p 0.034), disease duration (OR 1.03, CI 95% 1.00-1.07, p 0.021) and cyclophosphamide exposure (OR 2.97, CI 95% 1.49-5.88, p 0.002) were related to SLICC-SDI ≥3. Conclusion In our sample of patients, current smoking, older age, disease duration and cyclophosphamide were related to severe damage (SLICC-SDI ≥3).

Disease activity at the onset of diagnosis as a predictor of disease outcomes in a cohort of patients with systemic lupus erythematosus: A post hoc retrospective analysis of the COMOSLE-EGYPT study.

Systemic lupus erythematosus (SLE) has a non-uniform course directly reflected in changes in disease activity and anticipation of damage. To determine the impact of disease activity at the onset of disease diagnosis, measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) disease activity score, on different disease parameters and outcomes. This multicentre, retrospective cohort study included 823 SLE patients. Disease damage was measured by the Systemic Lupus International Collaborating Clinics Damage Index (SLICC), and comorbidities were measured by the Charlson Comorbidity Index (CCI). According to the mean SLEDAI at onset of disease diagnosis, patients were classified into two groups: I included patients with a mean SLEDAI ≤ 10 (non-severe disease activity), and II included patients with a mean SLEDAI > 10 (severe disease activity). SLEDAI at onset of disease diagnosis was a predictor of damage and comorbidities. A higher SLEDAI score at onset of disease diagnosis was associated with damage accrual. Patients who are younger at disease onset are more likely to have more severe disease. Severe disease activity at the onset of disease diagnosis was also associated with future comorbidity occurrences, but it was not significantly associated with mortality. SLEDAI at the onset of disease diagnosis could be a prognostic marker predicting the damage, which may help in the identification of patients who are at higher risk of adverse outcomes. Special care should be directed towards patients who are younger at disease onset as they may have a higher disease activity at diagnosis. Key Points • This is a unique study as it is the first to focus on the impact of SLE disease activity at the onset of disease diagnosis measured by SLEDAI disease activity score on different disease parameters and outcomes. • Previous studies, though these are scarce, have highlighted the impact of disease activity throughout the disease course and not specifically at the beginning of the SLE disease.

Clinical Presentation, Care Pathways, and Delays in Access to Specialized Care in Patients With Systemic Lupus Erythematosus: A Study From Lupus Midwest Network (LUMEN).

We aimed to characterize presentation and care pathways of patients with systemic lupus erythematosus (SLE), and delays in access to SLE-specialized care. We included patients with incident SLE from the Lupus Midwest Network registry. Time from the first medical encounter for SLE clinical manifestation to access to SLE-specialized care, physician diagnosis, and treatment was estimated. Delays were defined as ≥6 months to access specialized care. We compared SLE manifestations, disease activity, and Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage indexes (SDI) between patients with and without delays. Logistic regression models assessed associations with delays. The study included 373 patients with SLE. The median time to access SLE-specialized care was 1.1 (95% confidence interval [CI] 0.9-1.5) months, time to diagnosis was 30.6 (95% CI 18.9-48.1) months, and time to treatment initiation was 4.7 (95% CI 3.9-8.4) months. Approximately 25% of patients (93 out of 373) experienced delays accessing specialized care, which were associated with fewer SLE manifestations at first SLE-related encounter (fewer than two SLE domains; 92% vs 72%, P < 0.001). Patients with mucocutaneous or musculoskeletal manifestations were less likely to experience delays, whereas hematologic (odds ratio [OR] 1.71, 95% CI 1.03-2.84) or antiphospholipid antibodies domains (OR 6.05, 95% CI 2.46-14.88) were associated with delays. Delays were associated with damage at first access to SLE-specialized care (SDI ≥1; 30% vs 7%, P < 0.001). Patients follow a heterogeneous pathway to receive care. One-fourth of patients experienced delays accessing SLE-specialized care, which was associated with disease-related damage. Fewer manifestations, hematologic manifestations, or antiphospholipidantibodies were associated with delays.

Identifying distinct phenotypes of patients with juvenile systemic lupus erythematosus: results from a cluster analysis by the Egyptian college of rheumatology (ECR) study group

PurposeJuvenile systemic lupus erythematosus (J-SLE) is a complex, heterogeneous disease affecting multiple organs. However, the classification of its subgroups is still debated. Therefore, we investigated the aggregated clinical features in patients with J-SLE using cluster analysis. Methods: Patients (≤ 16 years) diagnosed using the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria were identified from the clinical database of the Egyptian College of Rheumatology (ECR) SLE study group. Demographic data, clinical characteristics, laboratory features, and current therapies were selected. A cluster analysis was performed to identify different clinical phenotypes. Results: Overall, 404 patients, of whom 355 (87.9%) were female, had a mean age at diagnosis of 11.2 years and a mean disease duration of 2.3 years. We identified four distinct subsets of patients. Patients in cluster 1 (n = 103, 25.5%) were characterized predominantly by mucocutaneous and neurologic manifestations. Patients in cluster 2 (n = 101, 25%) were more likely to have arthritis and pulmonary manifestations. Cluster 3 (n = 71, 17.6%) had the lowest prevalence of arthritis and lupus nephritis (LN), indicative of mild disease intensity. Patients in cluster 4 (n = 129, 31.9%) have the highest frequency of arthritis, vasculitis, and LN. Cluster 1 and 4 patients had the highest disease activity index score and were less likely to use low-dose aspirin (LDA). The SLE damage index was comparable across clusters. Conclusions: Four identified J-SLE clusters express distinct clinical phenotypes. Attention should be paid to including LDA in the therapeutic regimen for J-SLE. Further work is needed to replicate and clarify the phenotype patterns in J-SLE.

Systemic lupus international collaborating clinics-2012 and European league against rheumatism/American college of rheumatology-2019 classification criteria for systemic lupus erythematosus associated with childhood-onset auto-immune cytopenia.

Systemic Lupus Erythematosus (SLE) can be diagnosed using the 2012 criteria of the Systemic Lupus International Collaborating Clinics (SLICC) and, more recently, the 2019 criteria of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR). Hematological involvement is scored differently by these classifications. Our objective was to compare both criteria in a cohort of children with autoimmune cytopenia (AIC)-associated SLE. We included 79 patients with childhood-onset AIC as the first manifestations of SLE. The median age at SLE diagnosis was 14.5years (1.1-21.4years). The SLICC criteria were fulfilled by 76/79 (96%) patients and the EULAR/ACR criteria by 72/79 (91%) patients during follow-up. The SLICC and EULAR/ACR criteria were discordant (not concomitantly fulfilled) in 25/79 (32%) patients. Non-hematological clinical manifestations were more frequently observed in SLE diagnosis when the criteria were concordant (30/54, 56%) than when they were not (5/25, 20%) (p = 0.004). In 16/25 (64%) discordant patients, the SLICC criteria allowed earlier diagnosis of SLE. Finally, the attribution of a maximum weight of 6 to the hematological involvement of the EULAR/ACR criteria increased the sensitivity thereof from 63/79 (80%) to 76/79 (96%) in our population. The SLICC 2012 and EULAR/ACR 2019 criteria do not effectively diagnose SLE in children when AIC is the predominant feature. The SLICC criteria appear to be more effective in this population of SLE patients. An increase in the maximum weight of hematological involvement to 6 increases the sensitivity of the EULAR/ACR criteria for SLE diagnosis in children.

Factors affecting discrepancies in disease activity evaluation between patients and physicians in systemic lupus erythematosus-The importance of symptoms such as fatigue.

There are often discrepancies in the evaluation of disease activity between patients and physicians in systemic lupus erythematosus (SLE). In this study, we examined the factors that affect those evaluations. Physician visual analogue scale (Ph-VAS), patient VAS (Pt-VAS), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2k), glucocorticoid (GC) usage and dose, age, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and three patient-reported outcomes (SLE symptom checklist [SSC], short-form 36 questionnaire [SF-36], and LupusPRO) were obtained from a study performed in 2019 using 225 SLE outpatients of the Kyoto Lupus Cohort at Kyoto University Hospital. Correlations among Ph-VAS, Pt-VAS, or dif (Pt-VAS-Ph-VAS) (Pt-VAS minus Ph-VAS) and other factors were examined. We found a significant discrepancy between Pt-VAS (median 38.0mm) and Ph-VAS (median 18.7mm) scores (p < 0.001). SSC score showed a significant correlation with Pt-VAS and dif (Pt-VAS-Ph-VAS) (p < 0.001). Among SSC items, fatigue showed the most significant correlation with dif (Pt-VAS-Ph-VAS). We also showed that higher dif (Pt-VAS-Ph-VAS) was associated with lower quality of life (QOL) evaluated by SF-36 and LupusPRO. Pt-VAS scores tended to be higher than Ph-VAS scores, and the discrepancy was influenced mainly by fatigue. Higher dif (Pt-VAS-Ph-VAS) was associated with lower patient QOL.

Remission and low disease activity are associated with lower healthcare costs: results from the Systemic Lupus International Collaborating Clinics (SLICC) inception cohort

ObjectivesThis study aims to determine the independent impact of definitions of remission/low disease activity (LDA) on direct/indirect costs (DCs, ICs) in a multicentre inception cohort.MethodsPatients from 31 centres in 10...

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.

Efficacy, safety, and optimal intervention of belimumab for proliferative lupus nephritis patients in real-world settings: LOOPS registry.

This study investigated the efficacy, safety, and predictive factors of belimumab (BEL) in induction therapy for patients with proliferative lupus nephritis (LN) in real-world settings. Patients with biopsy-proven ISN/RPS class III or IV LN, with or without coexisting class V LN, who underwent standard of care (SoC), glucocorticoid (GC), and either mycophenolate mofetil or cyclophosphamide treatments were included. Participants were treated with SoC (SoC group, n = 32) or BEL and SoC (BEL+SoC group, n = 30). The primary end point was complete renal response (CRR) at 52 weeks. Baseline patient characteristics were not significantly different between the two groups. The 52-week retention rate of BEL was 90.0%. The BEL+SoC group showed significantly higher CRR and primary efficacy renal response achievement at 52 weeks and significantly lower GC dosage, adverse events, and Systemic Lupus International Collaborating Clinics damage index scores. Multivariate analysis of CRR achievement at 52 weeks revealed that the lack of estimated glomerular filtration rate (eGFR) improvement at 4 weeks was associated with CRR failure in the SoC group. A shorter duration (cut-off of 42 days) between the start of induction therapy and addition of BEL was also related to the CRR in the BEL+SoC group. BEL, in addition to SoC, controls disease activity, reduces GC use, and suppresses organ damage in case of proliferative LN. Earlier BEL induction within 6 weeks may help achieve CRR in treatment-resistant cases without eGFR improvement at 4 weeks after induction therapy.

Performance of the Systemic Lupus Erythematosus Risk Probability Index (SLERPI) in a cohort of Colombian population

ObjectiveTo evaluate the performance of the Systemic Lupus Erythematosus Risk Probability Index (SLERPI) in Colombian patients with systemic lupus erythematosus (SLE).MethodsThe Colombian cohort included 435 SLE patients and 430 controls with other autoimmune diseases (ADs). Clinical and serological data were collected, and SLE was indicated by SLERPI scores > 7. The American College of Rheumatology (ACR)-1997, Systemic Lupus International Collaborating Clinics (SLICC)-2012, and European League Against Rheumatism (EULAR)/ACR-2019 criteria were used as reference standards. The impact of overt polyautoimmunity (PolyA) on SLERPI performance was assessed. Additionally, multivariate lineal regression analysis was performed to evaluate the contribution of SLERPI features to the overall SLERPI score.ResultsSLE patients had higher SLERPI scores (P < 0.0001), with almost 90% meeting "definite" lupus criteria. Main factors influencing SLERPI included immunological disorder (β:44.75, P < 0.0001), malar/maculopapular rash (β:18.43, P < 0.0001), and anti-nuclear antibody positivity (β:15.65, P < 0.0001). In contrast, subacute cutaneous lupus erythematosus/discoid lupus erythematosus (β:2.40, P > 0.05) and interstitial lung disease (β:-21.58, P > 0.05) were not significant factors to the overall SLERPI score. SLERPI demonstrated high sensitivity for SLE, both for the overall SLE group and for those without overt PolyA (95.4% and 94.6%, respectively), but had relatively low specificity (92.8% and 93.7%, respectively). The model showed high sensitivity for hematological lupus (98.8%) and lupus nephritis (96.0%), but low sensitivity for neuropsychiatric lupus (93.2%). Compared to the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria, SLERPI yielded the highest sensitivity and lowest specificity.ConclusionSLERPI efficiently identified SLE patients in a Colombian cohort, showing high sensitivity but low specificity. The model effectively distinguishes SLE patients, even in the presence of concurrent overt PolyA.Key Points•SLERPI has a high sensitivity, but low specificity compared to ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria in the Colombian population.•Within the SLERPI score, immunological disorder, malar/maculopapular rash, and anti-nuclear antibody positivity are the strongest predictors of SLE.•SLERPI model can efficiently distinguish patients with SLE, regardless of concomitant overt PolyA.•SLERPI demonstrates high sensitivity in identifying hematological and nephritic subphenotypes of SLE.

Clinical Profile and Outcomes of Patients With Systemic Lupus Erythematosus.

Background Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by relapsing-remitting immune system activation, affecting multiple organ systems. Despite significant advances in understanding SLE's pathogenesis, there remains a need for comprehensive clinical profiling at the time of diagnosis to improve early detection and management. This study addresses this gap by providing a detailed analysis of the clinical presentation, disease activity, and patient outcomes using the Systemic Lupus International Collaborating Clinics (SLICC) criteria and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) index. Methodology This cross-sectional observational study included 80 patients diagnosed with SLEusing the 2012 SLICC criteria. Patients were recruited from the Rheumatology department and other wards of Byramjee JeejeebhoyGovernment Medical Collegeand Sassoon General Hospital, Pune, India. All participants provided informed consent and institutional ethical approval was obtained. Data were collected through detailed clinical history, physical examinations, and standard tests such aschest X-rays, CBC, RFT, LFT, urine microscopy, creatine phosphokinase, ANA, AntiDsDNA, complement consumption, and Coombs' tests, with 2D echocardiography performed as needed. Follow-ups every three months over 1.5 years assessed disease activity using SLEDAI criteria. Patients aged 12 and above who met the SLICC criteria were included and those with other connective tissue disorders were excluded. Associations between clinical symptoms and organ involvement were analyzed using thechi-square testwith a p-value of <0.05 considered significant. Results The study evaluated 80 patients with SLE, revealing a predominantly female cohort (80%) with a mean age of 29.4 years and a standard deviation of 8.3 years, skewed towards younger age groups. Clinical manifestations were diverse; the most common symptoms were (83.75%), oral ulcers (98.75%), and alopecia (95%). Anemia (66.25%) was the most prevalent abnormality, followed by albuminuria and renal abnormalities. Organ involvement was highest in the renal system (50%) and mucocutaneous features, with lower incidences in cardiac, gastrointestinal, and vascular systems. Gender-specific analyses indicated significant differences in SLE nephritis (p=0.048) and autoimmune hemolytic anemia (p=0.046). Autoantibody profiles showed high positivity for ANA (98.8%) and DsDNA (61.3%). Clinical outcomes demonstrated that 68.8% of patients achieved remission and 16.3% experienced organ damage. The SLEDAI scores significantly improved over time, with substantial reductions from baseline to nine months (p<0.001). Conclusion In conclusion, this study provides a detailed examination of SLE, revealing that it predominantly affects young adults and is characterized by diverse manifestations including mucocutaneous symptoms, significant renal involvement, and notable autoantibody profiles. The high prevalence of anti-nucleosome and anti-dsDNA antibodies underscores their diagnostic and prognostic value. Clinically, the findings highlight the necessity for early detection and targeted management of SLE, particularly in addressing renal and mucocutaneous symptoms. Future research should focus on longitudinal studies to track disease progression, explore genetic and environmental influences, and investigate regional variations to enhance treatment strategies and patient outcomes.

Belimumab in the treatment of systemic lupus erythematosus with juvenile onset: Results of a single-center retrospective study

The treatment of systemic lupus erythematosus with juvenile onset (jSLE) remains a difficult task, taking into account the more aggressive course of the disease, requiring the appointment of various therapy regimens, including mainly a combination of high doses of glucocorticoids (GC) with immunosuppressive drugs, which on the one hand improves control by the course of the disease, but on the other hand leads to an increase in serious adverse effects from therapy. Modern therapy capabilities have improved significantly with the advent of the belimumab – first and alone registered biologics for children with SLE.The aim of the study – based on an open single-center retrospective study, to analyze the efficacy and safety of belimumab in children with SLE.Material and methods. The study included all patients with jSLE who were observed in the pediatric department of V.A. Na sonova Research Institute of Rheumatology and received at least 1 infusion of belimumab. Diagnosis of SLE based on 2012 SLICC (Systemic Lupus Erythematosus International Collaborating Clinics) criteria. The efficacy of therapy was evaluated among patients who received belimumab for 6 months or more, and safety in all who received at least 1 infusion.Results. The study included 31 patients, 24 girls/7 boys. The median (Me) age at onset of the disease was 12.6 [10.18; 13.5] years, the Me duration of the disease at the time of initiation of belimumab therapy was 2.15 [0.9; 4.4] years. The Me activity on the SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) at the time of diagnosis verification was 12 [9; 17.5], at the time of start of belimumab – 8 [6; 12], 35.5% patients had severe activity, 51.6% – moderate, 12.9% – mild activity. The dose of GC per os at start of belimumab was 15 [10; 21.25] mg/day, 32.26% of patients received a high dose of GC, 54.84% – moderate dose, 12.9% – low dose. 9 patients had SDI (Systemic Lupus International Collaborating Clinics Damage Index) ≥1, Me – 1 [1; 2]. After 6 months of therapy, the Me of disease activity according to SLEDAI was 4 [2; 6], the dose of GC per os was reduced to 10 [8.25; 17.5] mg/day. In 15 patients, a decrease in antiDNA was recorded (57.7% of those who initially had elevated values of antiDNA), in 9 the level of complement was normalized (50% of those who initially had hypocomplementemia). After 12 months of therapy, the Me of SLEDAI was 4 [2; 4] (p=0.034), the dose of GC per os was 5 [5; 8.125] mg/day (p=0.012). 5 patients completed therapy within 12 months or more: 1 patient – remission, 4 patients – secondary inefficiency. Belimumab treatment was well tolerated, with the exception of three cases of serious adverse reactions (9.7%): prolonged diarrheal syndrome (after the 1st infusion), Lyell’s syndrome (after the 2nd infusion), infusion reaction (during the 2nd infusion). During the therapy of belimumab, no new damage were recorded; in 2 patients there was a decrease in the SDI.Conclusion. Belimumab therapy in patients with jSLE demonstrated high efficacy with a decrease in the activity of the disease according to SLEDAI, normalization of antiDNA and complement, the possibility of a significant reduction the dose of GC, the absence of progression of the SDI with a good safety profile in the vast majority of patients.

Immunophenotypes of systemic lupus erythematosus – features of clinical and laboratory disorders

The aim – to evaluate subpopulations of B lymphocytes and features of interferon (IFN) status in patients with systemic lupus erythematosus (SLE), to clarify the relationship of immunological parameters with clinical manifestations of the diseaseMaterial and methods. 139 patients (123 (88%) women and 16 (12%) men) with a definite diagnosis of SLE were included in the analysis. The disease duration was 3.0 [0.3; 12.0] years, SLEDAI-2K (Systemic Lupus Erythematosus Disease Activity Index 2000) – 7 [4; 11] points, SDI (Systemic Lupus International Collaborating Clinics Damage Index) – 0 [0; 1] points. Immunophenotyping of peripheral blood lymphocytes, including determination of B cells, the general population of memory B cells, non-switched and switched memory B cells, naive, transient B cells, and plasmablasts was carried out using multicolor flow cytometry. IFN status was assessed by the expression of IFN-stimulated genes (MX1, RSAD2, EPSTI1) using real-time polymerase chain reactionResults. Two immunological “patterns” were identified – the prevailing immunological mechanism of the pathogenesis of the disease – SLE – with predominant activation of type I IFN and with predominant activation of the B cell component of the immune system. The immunological phenotype with activation of type I IFN was associated with high immunological activity, predominant skin damage, leukopenia, and the phenotype with predominant activation of the B cell link was associated with damage to the kidneys and nervous system.Conclusion. The results of the work suggest a wide variety of immune mechanisms underlying the pathogenesis of SLE. It is possible to identify a number of leading molecular “patterns” of the pathogenesis of the disease, which must be taken into account to select an effective “targeted” drug.

Frailty is independently associated with subclinical cardiovascular disease in patients with systemic lupus erythematosus

ObjectivesCardiovascular disease is a leading cause of mortality in systemic lupus erythematosus (SLE). Frailty has been associated with an increased cardiovascular disease risk (CVR) in the general population. We aimed...

Exploring gastrointestinal manifestations in childhood onset systemic lupus erythematosus - Insights from a multicenter study.

Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE. The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology. All participants met the Systemic Lupus International Collaborating Clinics criteria. Out of 123 patients, 78 (63.4%) exhibited GIS involvement at the initial SLE diagnosis, whereas the remaining 45 (36.6%) developed GI symptoms after a median duration of 12 (3-140) months. Eighty-two (66.7%) individuals experienced symptoms related to the GI tract, whereas the remaining patients received a diagnosis of GI involvement through laboratory assessments. The predominant initial GIS involvement symptom was abdominal pain, observed in 77 (62.6%) patients, followed by elevated hepatic transaminases in 70 (56.9%), hepatomegaly in 40 (32.5%), diarrhea in 26 (21.1%), and jaundice in 11 (8.9%) patients. The GIS involvement was associated with SLE in 82 (78.6%), while it resulted from drug-related adverse events in 35 (28.5%) patients or comorbidities in 6 (0.5%) patients. GIS involvement should be considered in all childhood onset-SLE patients, especially in the presence of suggestive symptoms or elevated hepatic transaminases. It is also crucial to consider SLE in the differential diagnosis of GIS manifestations in children. Apart from GIS involvement directly associated with SLE, adverse events of drugs should be kept in mind.

Bilateral vocal cords palsy in adolescent Systemic Lupus Erythematosus patient

Background: Executive functions such as speech are uncommonly involved in systemic lupus erythematosus (SLE) presentation. We report a case of SLE who presented with a seizure followed by an inability to speak. Case description: A 17-year-old girl was presented with a sudden loss of voice for 2 days after a seizure following fever for 4 months. The patient was aware and could understand words but could not execute verbal sounds; the sound she produced was muffled. The generalized seizure occurred without any trigger, lasted 2 minutes, and stopped spontaneously. Fever occurred primarily at night which subsided without treatment. She also complained of severe hair loss. Patient with leucopenia, lymphopenia, anemia hemolytic, thrombocytopenia, proteinuria, hematuria, brain atrophy from magnetic resonance imaging (MRI) examination, and echocardiography revealed mild carditis and mild circumferential pericardial effusion. Immunological assay showed antinuclear antibodies (ANA) &gt;1:1000 with nuclear homogenous, low C3 complement, and positive Anti-dsDNA. Systemic Lupus International Collaborating Clinics (SLICC) 2015 total score was 14, and the European League Against Rheumatism (EULAR/ACR) 2019 total score was 31, confirmed patient for SLE diagnosis. Further exploration from fiberoptic laryngoscopy confirmed bilateral vocal cord palsy due to SLE. Management included high dose methylprednisolone, cyclophosphamide, and speech therapy which results in gradual improvement from producing mumble sounds, then words followed by sentences with nasal sounds. Laryngeal involvement is uncommonly reported as an SLE manifestation. Management includes steroids and immunosuppressive for pharmacological and speech therapy as an exercise for vocal cord palsy. Conclusion: The patient must be continuously monitored for complete resolution of the palsy.

Clinical and Immunological Profile of Pediatric-Onset Systemic Lupus Erythematosus in Eastern India: A Prospective Observational Study.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with a strong female predisposition. pSLE often results in a worse prognosis compared to adult SLE. Studies on pSLE from the Indian subcontinent are scarce. This study aims to describe the clinical manifestations, laboratory and serological parameters, management, and outcomes of pSLE patients from a premier tertiary care institute in Eastern India. This prospective observational study was conducted at Kalinga Institute of Medical Sciences, Bhubaneswar, from September 2020 to October 2023. Children aged 1-14 years fulfilling the Systemic Lupus International Collaborating Clinics criteria for SLE were included. A detailed history, clinical examination, and laboratory investigations were performed. Data on complications, treatment, and outcomes were collected. Statistical analysis was done using SPSS Statistics version 21 (IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.). Out of 114,009 patients (outdoor and indoor), 40 were diagnosed with pSLE, resulting in an incidence of 0.35 per 1000 children. The female-to-male ratio was 7:1. The mean age of presentation was 11.67 ± 2.37 years. Among the predominant symptoms observed, mucocutaneous manifestations were seen in 39 (97.5%), followed by pallor in 36 (90%), and fever in 33 (82.5%). The most common organ system involved was mucocutaneous, observed in 39 (97.5%) patients, followed by hematological in 36 (90%) and renal in 19 (47.5%). Lupus nephritis was observed in 19 (47.5%) patients, with class IV being the most common. Anti-nuclear antibody and anti-double-stranded DNA were positive in 39 (97.5%) and 27 (68%) of children, respectively. Complete remission was achieved in 14 (35%), improvement in 16 (40%), and flare-ups in 3 (7.5%) patients. pSLE is an uncommon but severe autoimmune disease with significant multi-system involvement. Early identification and prompt treatment are crucial to minimizing adverse outcomes. This study provides detailed insights into the clinical and immunological profile of pSLE in Eastern India, underscoring the need for larger multicentric studies with long-term follow-ups.

Damage in a large systemic lupus erythematosus cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER) with emphasis on the cardiovascular system: a longitudinal analysis

ObjectiveTo assess organ damage, with emphasis on the cardiovascular system, over the different stages of the disease in a large SLE cohort.MethodsMulticentre, longitudinal study of a cohort of 4219 patients...