SLEDAI-2K Score Research Articles

A retrospective cohort study of Epstein-Barr virus infection status and systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) and the Epstein-Barr virus (EBV) are very closely related. This study estimated the impact of EBV infection status on clinical manifestations and disease remission in patients with SLE. A retrospective study was performed using electronic health records of patients with SLE. The SLE disease activity index (SLEDAI-2K) was used to assess disease activity. VCAIgM or EAIgM positive or EBVDNA copies ≥ 50IU/mL were defined as lytic infection group, EBNA-IgG or VCAIgG-positive and who were negative for both VCAIgM and EAIgM with EBVDNA copies < 50IU/mL were defined as the latent infection group. The endpoint (disease remission) was defined as a decrease in SLEDAI-2K score of ≥ 1 grade or ≥ 4 points from baseline. The association between EBV infection status and disease remission was assessed using propensity score weighting and multivariable Cox regression models. There were 75 patients with SLE in the EBV lytic infection group and 142 patients in the latent infection group. The SLEDAI-2K score was higher in the lytic infection group (10.00 (6.25, 16.00) vs. 8.00 (5.00, 10.00), Z = 3.96, P < 0.001). There was a significant difference in the effect of EBV lytic infection on disease remission compared to latent infection (HR 0.30, 95% CI 0.19-0.49, P < 0.001). Patients with SLE with lytic EBV infection have higher disease activity and take longer to achieve remission. Our study furthers our understanding of the relationship between SLE and EBV infection and may inform better treatment practices in the future.

Reduced expressions of apoptosis-related proteins TRAIL, Bcl-2, and TNFR1 in NK cells of juvenile-onset systemic lupus erythematosus patients: relations with disease activity, nephritis, and neuropsychiatric involvement.

Lupus pathogenesis is mainly ascribed to increased production and/or impaired clearance of dead cell debris. Although self-reactive T and B lymphocytes are critically linked to lupus development, neutrophils, monocytes, and natural killer (NK) cells have also been implicated. This study assessed apoptosis-related protein expressions in NK cells of patients with juvenile-onset systemic lupus erythematosus (jSLE) and relations to disease activity parameters, nephritis, and neuropsychiatric involvement. Thirty-six patients with jSLE, 13 juvenile dermatomyositis (JDM) inflammatory controls, and nine healthy controls had Fas, FasL, TRAIL, TNFR1, Bcl-2, Bax, Bim, and caspase-3 expressions in NK cells (CD3-CD16+CD56+) simultaneously determined by flow cytometry. Disease activity parameters included Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score, erythrocyte sedimentation rate, C-reactive protein level, anti-double strain DNA antibody level, complement fractions C3 and C4 levels. Patients with jSLE had a profile of significantly reduced expression of TRAIL, Bcl-2, and TNFR1 proteins in NK cells when compared to healthy controls. Similar profile was observed in patients with jSLE with active disease, positive anti-dsDNA, nephritis, and without neuropsychiatric involvement. Patients with jSLE with positive anti-dsDNA also had reduced expression of Bax in NK cells when compared healthy controls and to those with negative anti-dsDNA. Yet, patients with jSLE with negative anti-dsDNA had reduced mean fluorescence intensity (MFI) of Bim in NK cells compared to healthy controls. Patients with jSLE with nephritis also had reduced MFI of Fas in NK cells when compared to those without nephritis. In addition, in patients with jSLE, the proportion of FasL-expressing NK cells directly correlated with the SLEDAI-2K score (rs = 0.6, p = 0.002) and inversely correlated with the C3 levels (rs = -0.5, p = 0.007). Moreover, patients with jSLE had increased NK cell percentage and caspase-3 protein expression in NK cells when compared to JDM controls. This study extends to NK cells an altered profile of TRAIL, Bcl-2, TNFR1, Fas, FasL, Bax, Bim, and caspase-3 proteins in patients with jSLE, particularly in those with active disease, positive anti-dsDNA, nephritis, and without neuropsychiatric involvement. This change in apoptosis-related protein expressions may contribute to the defective functions of NK cells and, consequently, to lupus development. The full clarification of the role of NK cells in jSLE pathogenesis may pave the way for new therapies like those of NK cell-based.

Lupus Low Disease Activity State and organ damage in relation to quality of life in systemic lupus erythematosus: a cohort study with up to 11 years of follow-up.

Beyond prevention of organ damage, treatment goals in systemic lupus erythematosus (SLE) include optimisation of health-related quality of life (HRQoL). The Lupus Low Disease Activity State (LLDAS) has received increasing attention as a goal whenever remission cannot be achieved. How SLE disease activity, organ damage, and LLDAS attainment relate to patient-reported outcomes (PROs) is not fully explored, which formed the scope of this investigation. We included 327 patients with SLE from a tertiary referral centre. Longitudinal registrations of disease activity using SLEDAI-2K and physician global assessment (PhGA), organ damage using the SLICC/ACR damage index (SDI), pharmacotherapies, EQ-5D-3L data, as well as visual analogue scale (VAS) scores for fatigue, pain, and overall SLE-related health state over a median follow-up time of 8.5 years were analysed. In the overall population, as well as subgroups of patients with recent-onset SLE and those with clinically active, autoantibody-positive disease, LLDAS attainment, lower PhGA, and lower clinical SLEDAI-2K scores were associated with favourable HRQoL by EQ-5D-3L and VAS assessments, while increasing SDI scores were associated with poor PROs yet not fatigue in the overall population. PROs were further enhanced by being in LLDAS sustainedly. In fully adjusted models of the entire study population, LLDAS attainment and lower disease activity were associated with favourable PROs, irrespective of SDI. In one of the longest to date observational studies, we demonstrated that low disease activity and being sustainedly in LLDAS were coupled with favourable HRQoL, pain, fatigue, and overall health experience, irrespective of organ damage.

Adolescent Health Care Needs and Relationship to Disease in Patients With Childhood-Onset Systemic Lupus Erythematosus.

Our objective was to characterize adolescent health and psychosocial issues in patients with childhood-onset systemic lupus erythematosus (cSLE) and evaluate demographic and disease characteristics associated with adolescent health. We retrospectively examined adolescents aged 12 to 18 years with cSLE seen at the Hospital for Sick Children meeting the American College of Rheumatology/Systemic Lupus International Collaborating Clinics classification criteria, assessed by adolescent medicine in the cSLE clinic between 2018 and 2020. Adolescent health issues were characterized using the Home, Education/Employment, Activities, Diet/Drugs, Sexuality, Suicide/mood (HEADDSS) framework. Issues were classified as presenting and/or identified; adolescent health burden was tabulated as the number of distinct adolescent issues per patient. Multiple Poisson regression models examined associations between patient and disease characteristics (age, sex, material deprivation, disease activity, disease damage, and high-dose glucocorticoid exposure) and adolescent health issues. A total of 108 (60%) of 181 adolescents with cSLE were seen by adolescent medicine, with a median of 2 (interquartile range [IQR] 1-3) visits and a median of 2 (IQR 1-5) adolescent health issues during the study period. Common issues were mood (presenting in 21% vs identified in 50%), sleep (27% vs 2%), school and education (26% vs 1%), and nonadherence (23% vs 8%). Psychoeducation was provided by adolescent medicine to 54% of patients. High-dose glucocorticoids (risk ratio [RR] 1.82, 95% confidence interval [CI] 1.41-2.35, P < 0.001), material deprivation (RR 1.17, 95% CI 1.04-1.30, P = 0.007), and lower SLE Disease Activity Index scores (RR 0.95, 95% CI 0.92-0.98, P = 0.004) were associated with higher adolescent health burden. Adolescents with cSLE experience many adolescent issues, especially low mood. High-dose glucocorticoids and social marginalization are associated with greater adolescent health burden. This study highlights the importance of addressing adolescent health needs as part of routine care.

Association of disease activity with depression and anxiety in systemic lupus erythematosus: A comparison of SLEDAI-2K and SLE-DAS.

To explore the association of disease activity, as evaluated by both the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) and the Systemic Lupus Erythematosus Disease Activity Index-2000 (SLEDAI-2K), with depression and anxiety in patients with systemic lupus erythematosus (SLE). A cross-sectional study was conducted among 85 Chinese patients with SLE. Disease activity was measured using SLEDAI-2K and SLE-DAS scoring systems. Depression and anxiety were assessed using Patient Health Questionnaire-9 (PHQ-9) and Generalized Anxiety Disorder Scale-7 (GAD-7), respectively. Multivariate logistic regression analysis was performed to evaluate the association of disease activity scores, as well as specific clinical and laboratory items, with depression and anxiety. There was a robust correlation between SLEDAI-2K and SLE-DAS scores in overall patients (Spearman's r = 0.764, 95% confidence interval (CI) 0.655-0.842; p< 0.001) and those with moderate-to-high disease activity (Spearman's r = 0.792, 95%CI 0.616-0.892; p< 0.0001). However, the correlation weakened for patients with mild disease activity or remission (Spearman's r = 0.450, 95%CI 0.188-0.652; p= 0.001). Multivariate logistic regression analysis did not show a significant correlation between SLEDAI-2K and SLE-DAS scores and depression/anxiety. The presence of mucosal ulcer/serositis significantly increased the risk of depression (OR = 4.472, 95%CI 1.035-19.328, p= 0.045) and anxiety (OR = 3.978, 95%CI 1.051-15.049, p= 0.042). The SLE-DAS scoring system demonstrated a comparable ability to assess disease activity in SLE compared with SLEDAI-2K. Though neither scoring system showed significant associations with depression and anxiety, the presence of mucosal ulcer/serositis markedly heightened the risk of both among SLE patients.

Children systemic lupus erythematosus-associated pancreatitis

ObjectiveTo early recognise and improve the prognosis of children systemic lupus erythematosus (cSLE)-associated pancreatitis by summarising and analysing clinical features and prognosis data from 12 cases.MethodsRetrospective analysis of clinical data from 12 cases of cSLE-associated pancreatitis diagnosed and treated from January 2016 to December 2021 at hospitals such as Children’s Hospital of Capital Institute of Paediatrics.ResultsThe median SLEDAI-2K score for disease activity was 18.00 (range 12.25–21.00) in the case group and 10.00 (range 7.00–18.00) in the control group, with a statistically significant difference (P < 0.05) between the two groups. The case group had a higher proportion of abdominal pain, vomiting, abdominal distension, pleural effusion, Raynaud’s phenomenon (RP), splenic infarction, and concurrent macrophage activation syndrome (MAS) than the control group, with a statistically significant difference (P < 0.05). Serum ferritin (SF), alanine transaminase (ALT), aspartate transaminase (AST), lactate dehydrogenase (LDH), amylase, and increased 24-h urine protein levels were statistically different between the two groups (P < 0.05); platelet counts (PLT) reduction was also statistically different (P < 0.05). The case group had a higher proportion of methylprednisolone pulse therapy, cyclophosphamide pulse therapy during remission induction, and therapeutic plasma exchange than the control group, with a statistically significant difference (P < 0.05) between the two.ConclusionCSLE-associated pancreatitis has a high fatality rate. The presence of RP, splenic infarction, pleural effusion, and MAS warrants attention from clinicians regarding the possibility of pancreatitis. Once pancreatitis is detected, the primary disease needs active treatment for better prognosis.

Association of Mitochondrial Pyruvate Carrier with the Clinical and Histological Features in Lupus Nephritis.

Mounting evidence suggests that mitochondrial dysfunction contributes to lupus nephritis (LN) pathogenesis. Mitochondrial pyruvate carrier 1 (MPC1) and mitochondrial pyruvate carrier 2 (MPC2) mediating pyruvate transport from the cytoplasm to the mitochondrial matrix, determines the cell survival and cellular energy supply. Here, we aimed to investigate the association of mitochondrial pyruvate carrier expression with the clinical and histological features in LN. Patients with biopsy-proven proliferative LN (class III and class IV, n=18) and membranous LN (class V, n=18) were included. Expression of MPC1 and MPC2 were examined by immunohistochemistry. MPC protein levels in the two groups were evaluated by the Student's t-test. Correlation analysis between MPC levels and clinicopathological features was performed by Spearman's rank correlation. Both MPC1 and MPC2 were exclusively expressed in renal tubules of enrolled LN. Significantly lower MPC1 and MPC2 were observed in patients with proliferative LN compared to membranous LN. In addition, the MPC1 and MPC2 were negatively correlated with SLEDAI-2K score, renal function, and renal pathology activity index. Both MPC1 and MPC2 were localized in renal tubules, and decreased MPC content was more pronounced in proliferative LN than membranous LN. MPC levels were significantly correlated with renal functions and renal pathology activity.

GPX4 Downregulation Induces Platelet Ferroptosis in Systemic Lupus Erythematosus

Background: Ferroptosis is a recently discovered type of regulated necrosis and glutathione peroxidase 4 (GPX4) has been recognized as a key enzyme that protects against ferroptosis. However, the role of platelet GPX4 in systemic lupus erythematosus (SLE) has not been explored. In this study, GPX4 levels in platelets were measured and the correlation with clinical features were analyzed. Methods: Platelet GPX4 protein expression was detected by Western blot and immunofluorescence in 37 SLE patients and 23 healthy controls. Clinical data were recorded at time points of blood sampling. Platelet activation was analyzed by flow cytometry, LDH release by LDH release assay and plasma oxidized DNA levels by a general 8-OHdG ELISA kit. Results: Patients with SLE had significantly decreased expression of platelet GPX4 and increased percentage of platelet activation as compared with healthy controls. Furthermore, levels of platelet GPX4 were negatively correlated with SLEDAI-2K score, 24-hour urine protein and oxidized DNA. Low GPX4 expression were associated with skin and joint involvements of SLE patients. Interestingly, deferoxamine (DFO), a ferroptosis inhibitor, could protect SLE platelets from death. Conclusions: These findings confirmed that downregulated GPX4 protein in platelets of SLE patients negatively correlated with plasma oxidized DNA, SLE disease activity and 24-hour urine protein, suggesting the potential role of platelet ferroptosis in oxidized DNA release and the pathogenesis of SLE.

Role of Interleukin-17 in Predicting Activity of Rheumatoid Arthritis and Systemic Lupus Erythematosus.

Although high serum levels of interleukin (IL)-17 and its producing cells have been found in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) in earlier research, it is still unclear how these findings relate to disease activity. This study examines the link between serum levels of IL-17 and the activity of both RA and SLE. This pilot case-control study included 100 patients with RA, 100 with SLE, and 100 healthy controls. The Disease Activity Score-28 (DAS28) scores assessed the activity of RA, whereas the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores assessed SLE activity. All participants' data were compared and correlated. Serum levels of IL-17 were significantly higher in RA and SLE patients compared with the controls (P < .001) and showed significantly positive correlations (P < .001) with rheumatoid factor titer, anti-cyclic citrullinated peptide (anti-CCP) and DAS28 score among the RA patients. Although among SLE patients, they were significantly positively correlated (P < .001) with anti-double-stranded DNA (anti-ds DNA) levels and the SLEDAI-2K scores, the best cut-off value of IL-17 for predicting moderate and high disease activity was > 175 pg/mL among RA patients and > 95 pg/mL among SLE patients. There is a significant correlation between RA and SLE activity and serum levels of IL-17. This discovery emphasizes IL-17 as a potential therapeutic target.

Real-World Effectiveness of Belimumab in Patients with Active Lupus.

This study evaluated the real-world effectiveness of belimumab (BLM) in the treatment of systemic lupus erythematosus (SLE) patients with moderate to high disease activity. This retrospective cohort study enrolled 129 Japanese patients with moderate to high SLE disease activity who received BLM between January 2013 and March 2023. The clinical outcomes, including the flare-free survival, SLE Disease Activity Index 2000 (SLEDAI-2K) score, and prednisone-equivalent dose, in the BLM and mycophenolate mofetil (MMF) treatment groups were compared before and after treatment. Safety data for BLM were collected. Additionally, we compared the effectiveness of BLM and intravenous cyclophosphamide (IV-CY) treatment using the stabilized inverse probability of treatment weighting (IPTW) method based on the propensity scores. This observational study enrolled 129 patients with moderate/severe SLE: 48 patients received belimumab, 45 received IV-CY, and 36 received MMF and prednisolone for remission induction therapy. The median follow-up for the BLM group was 17.0 months. Among them, 19 received BLM plus MMF. BLM significantly reduced the mean SLEDAI-2K (from mean baseline to 52 weeks: 49.2% reduction from 12.8 to 6.5) and prednisone daily dose (from mean baseline to 52 weeks: 21.9% reduction from 12.8 to 10.0 mg/day). The flare-free survival at 52 weeks was not significantly different between the BLM and MMF groups. There was no significant difference in the flare-free survival rates or reduction rates of the SLEDAI-2K between the patients treated with BLM and those treated with BLM plus MMF. In the propensity score-matched comparative analyses, there was no significant difference in the flare-free survival rates or an estimated decline in the SLEDAI-2K scores between the patients with lupus treated with BLM and IV-CY. BLM may be a promising alternative treatment option for lupus patients with moderate or high disease activity who do not respond to conventional treatments.

The efficacy of rituximab plus belimumab or telitacicept in refractory lupus nephritis.

Lupus nephritis is a severe and common complication of systemic lupus erythematosus (SLE). The pathogenesis of lupus nephritis is characterized by B-cell activation and autoantibody formation. Rituximab and belimumab, as well as telitacicept, target B cells through different mechanisms, potentially exerting a synergistic effect in the treatment of lupus nephritis. This study aims to investigate the efficacy and safety of treatment with rituximab followed by belimumab or telitacicept in the management of refractory lupus nephritis. We conducted a single-center, open-label, retrospective study, including 25 patients with refractory lupus nephritis. All patients received combination therapy with rituximab in individualized dosages to achieve peripheral B-cell depletion, and then followed by belimumab or telitacicept. The follow-up period was at least 12 months, and the primary end point was renal remission rate at the last follow-up. During a median follow-up of 19 (13, 29) months, 20 of 25 (80%) patients achieved objective remission (OR), including 19 (76%) patients achieved complete renal response (CRR). After rituximab (712 ± 416mg in average), 18 patients received belimumab and seven patients received telitacicept. In the rituximab plus telitacicept group, all patients achieved CRR; while in the rituximab plus belimumab group, 12 (66.7%) patients achieved CRR and 13 (72.2%) patients achieved OR. The mean SLEDAI-2K score decreased from 15 ± 6 to 6 ± 6, representing an average reduction of 60%. At the last follow-up, 18/25 (72%) had prednisone ≤ 5mg/d or even discontinued prednisone use. Adverse effects were mainly immunoglobulin deficiency, respiratory tract infection, urinary tract infections, and rash. No death occurred. Rituximab followed by belimumab or telitacicept may be effective in inducing remission in refractory lupus nephritis, with tolerable adverse effects.

The Role of Sinopharm BIBP COVID-19 Vaccine Immunization in Systemic Lupus Erythematous Flare-up

Background: This study aimed to investigate the incidence and clinical characteristics of flare-ups in patients with systemic lupus erythematosus (SLE) following immunization with inactivated SARS-CoV-2 vaccines. Methods: In this cross-sectional study at Imam Hossein Hospital's Rheumatology Clinic (Iran), we investigated 72 SLE patients in remission who received the Sinopharm BIBP inactivated COVID-19 vaccine. Their post-vaccination status was monitored for 3 months using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) checklist by an internal medicine specialist. Results: Fourteen patients (19.44%) experienced symptom flare-ups after vaccination. The most common symptoms were arthritis (64.29%) and skin rash (21.43%). Age, sex, organ involvement, and treatment regimen did not significantly differ between those with and without symptom recurrence (P &gt; 0.05). The second vaccine dose led to more flare-ups compared to the first dose (12.12% vs. 8.33%, P &lt; 0.001). However, the severity of symptom recurrence, measured by the SLEDAI-2K score (P = 0.763), and the interval from vaccination to symptom recurrence (P = 0.075) did not significantly differ between the 2 groups. Except for 2 patients, none of the participants required hospitalization, and flare-up symptoms were effectively managed by prednisolone dosage adjustments. For these 2 patients, the treatment regimen was changed, and the steroid dose was increased; one of them was admitted to the hospital, and the other one was managed on an outpatient basis. Conclusions: The incidence of flare-ups in SLE patients in remission following COVID-19 vaccination with Sinopharm BIBP vaccine was low; most of them were mild and did not require hospitalization, except for 1 patient who was hospitalized after the first dose of vaccination and received rituximab due to vasculitis flare. These findings highlight vaccine safety and underscore the importance of close monitoring, especially after the second dose.

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.

Serum 25(OH)D levels are associated with disease activity and renal involvement in initial-onset childhood systemic lupus erythematosus.

Vitamin D deficiency is common in patients with systemic lupus erythematosus (SLE) and may affect their disease activity and severity. This study aims to assess the vitamin D status in patients with initial-onset SLE during childhood and its association with the clinical and laboratory markers of disease activity. This is a retrospective study that includes 168 patients with initial-onset SLE during childhood and 109 healthy children as controls. Clinical and laboratory data were recorded. The area under the curve (AUC) method was used to evaluate the efficacy of double-stranded deoxyribonucleic acid (dsDNA), lower 25(OH)D and complement 3 (C3) alone and in combination to diagnose the presence of renal damage in children with SLE. Compared with the controls (25.53 ± 7.02 ng/ml), patients with initial-onset SLE during childhood have lower serum 25(OH)D levels (18.63 ± 5.32 ng/ml) (P < 0.05). Among patients with initial-onset SLE during childhood, SLEDAI-2K scores are significantly higher in the vitamin D insufficiency (median = 14.5) and vitamin D deficiency (median = 14.0) groups than in the vitamin D sufficiency group (median = 9.0) (P < 0.05). Patients with initial-onset SLE during childhood with lower 25(OH)D levels are more likely to have lupus nephritis (LN) and a higher SDI score (P < 0.05). Compared with patients with other types of LN (16.69 ± 3.90 ng/ml), patients with type V LN have lower levels of 25(OH)D (12.27 ± 3.53 ng/ml) (P < 0.05). The AUC was 0.803 when dsDNA antibody, 25(OH)D level and C3 were used in combination to diagnose LN in patients with SLE. Vitamin D deficiency and insufficiency are closely related to an increase in SLEDAI and SDI scores. Significant decrease in vitamin D level is a risk factor for LN.

Burden of systemic lupus erythematosus in clinical practice: baseline data from the SLE Prospective Observational Cohort Study (SPOCS) by interferon gene signature

ObjectiveThe longitudinal Systemic Lupus Erythematosus Prospective Observational Cohort Study (SPOCS) aims to assess SLE disease course overall and according to type I interferon 4 gene signature (IFNGS). Here, we describe...

Safety and immunogenicity of influenza A(H3N2) component vaccine in juvenile systemic lupus erythematosus

IntroductionSeasonal influenza A (H3N2) virus is an important cause of morbidity and mortality in the last 50 years in population that is greater than the impact of H1N1. Data assessing immunogenicity and safety of this virus component in juvenile systemic lupus erythematosus (JSLE) is lacking in the literature.ObjectiveTo evaluate short-term immunogenicity and safety of influenza A/Singapore (H3N2) vaccine in JSLE.Methods24 consecutive JSLE patients and 29 healthy controls (HC) were vaccinated with influenza A/Singapore/INFIMH-16-0019/2016(H3N2)-like virus. Influenza A (H3N2) seroprotection (SP), seroconversion (SC), geometric mean titers (GMT), factor increase in GMT (FI-GMT) titers were assessed before and 4 weeks post-vaccination. Disease activity, therapies and adverse events (AE) were also evaluated.ResultsJSLE patients and controls were comparable in current age [14.5 (10.1–18.3) vs. 14 (9–18.4) years, p = 0.448] and female sex [21 (87.5%) vs. 19 (65.5%), p = 0.108]. Before vaccination, JSLE and HC had comparable SP rates [22 (91.7%) vs. 25 (86.2%), p = 0.678] and GMT titers [102.3 (95% CI 75.0–139.4) vs. 109.6 (95% CI 68.2–176.2), p = 0.231]. At D30, JSLE and HC had similar immune response, since no differences were observed in SP [24 (100%) vs. 28 (96.6%), p = 1.000)], SC [4 (16.7%) vs. 9 (31.0%), p = 0.338), GMT [162.3 (132.9–198.3) vs. 208.1 (150.5–287.8), p = 0.143] and factor increase in GMT [1.6 (1.2–2.1) vs. 1.9 (1.4–2.5), p = 0.574]. SLEDAI-2K scores [2 (0–17) vs. 2 (0–17), p = 0.765] and therapies remained stable throughout the study. Further analysis of possible factors influencing vaccine immune response among JSLE patients demonstrated similar GMT between patients with SLEDAI < 4 compared to SLEDAI ≥ 4 (p = 0.713), as well as between patients with and without current use of prednisone (p = 0.420), azathioprine (p = 1.0), mycophenolate mofetil (p = 0.185), and methotrexate (p = 0.095). No serious AE were reported in both groups and most of them were asymptomatic (58.3% vs. 44.8%, p = 0.958). Local and systemic AE were alike in both groups (p > 0.05).ConclusionThis is the first study that identified adequate immune protection against H3N2-influenza strain with additional vaccine-induced increment of immune response and an adequate safety profile in JSLE. (www.clinicaltrials.gov, NCT03540823).

Risk factors associated with multiple organ damage in childhood-onset systemic lupus erythematosus

ObjectivePrevious studies have shown that approximately 39%–65% of patients with childhood-onset systemic lupus erythematosus (cSLE) have damage in at least one organ. Data on risk factors for organ damage in cSLE remain limited, especially in Asian populations. This study was conducted to evaluate the incidence of cSLE and identify the risk factors for accumulated organ damage in patients with cSLE.MethodsThis was a retrospective study. Patients aged &amp;lt;18 years who were diagnosed with cSLE between 2008 and 2020 were enrolled. Information on baseline characteristics, treatment, and disease activity assessed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was collected from diagnosis until the most recent visits were reviewed from medical records. Disease damage was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI).ResultsA total of 134 patients with a mean age at diagnosis of 11.2 ± 2.9 years were enrolled. The median duration of treatment was 4.7 (interquartile range 2.8–7.1) years. Forty patients (29.9%) had irreversible organ damage (SDI &amp;gt; 1) with an incidence rate of 5.7 events per 100 person-years. The most frequent type of organ damage was ocular (11.1%), followed by musculoskeletal (8.9%) and neurological (7.4%). High disease activity at diagnosis (SLEDAI-2K ≥ 12) (odds ratio [OR] 3.19, 95% confidence interval [CI] 1.32–7.68), infection (OR 3.73, 95% CI 1.60–8.67), and mycophenolate mofetil use (OR 3.62, 95% CI 1.45–9.03) were predictors of organ damage. The median time to disease damage in patients with SLEDAI-2K scores ≥12 at diagnosis was 6.5 years (95% CI 5.77–7.36; P = 0.004).ConclusionPhysicians should be aware of organ damage in patients with cSLE, particularly those with high disease activity at initial presentation, those who are receiving mycophenolate mofetil therapy, and those with an infection.

1147. A study of high-dose influenza vaccination responses compared to standard-dose in lupus patients: an open- labeled, randomized controlled study

Abstract Background Annual influenza vaccination at standard dosing in the general population is suggested. However, there may be more suitable for lupus patients. Objectives To assess the immune responses among lupus patients receiving high dose (HD) and standard (SD) quadrivalent inactivated influenza vaccine, evaluate the adverse events of the HD, and monitor the incidence of influenza infection. Methods A randomized parallel design, controlled trial was conducted from March 2021 to May 2022 at Ramathibodi Hospital. All lupus patients were stratified into two groups depending on the depth of immunosuppressive therapy received and randomized to receive either HD or SD in a 1:1 ratio. Patient demographics data and relevant information were retrieved. Blood samples were obtained for hemagglutination inhibition assay (HAI) before vaccination and 4-12 weeks after completion of the vaccination series to determine seroprotection and seroconversion rates. Results One hundred twenty-eight lupus patients were enrolled, and 109 individuals completed the HAI analysis. 54/109 patients were in the high- (HI) with mean age (mean ± SD) of 34.3 (25.2-43.4) years, and 55/109 were in the low-immunosuppressive (LI) groups with mean age (mean ± SD) of 38.2 (30.2-46.2) (p=0.018). The majority of patients had a SLEDAI-2K score of 4. The seroprotection rates against H1N1, H3N2/Hongkong, H3N2/Cambodia, B/Victoria, and B/Yamagata strains in HI and LI groups at baseline after the first and second injections were outlined as follows 1.) HI; 74.1%, 82.1%, 76.9%, 75.9%, 79.6%; 85.2%, 89.3%, 88.5%, 85.2%, 88.9%; 89.5%, 89.3%, 88.5%, 93.9%, 88.9%, and 2.) LI; 76.4%, 84.6%, 93.1%, 74.5%, 89.1%; 92.7%, 100%, 100%, 94.5%, 96.4%; 92.7%, 100%, 100%, 94.5%, 94.5%. There was one documented influenza infection during the 9-month follow-up period. The patient was in the LI group and received the SD vaccine. 19/109 (17.4%) patients had grade 1 adverse events with no difference between SD and HD. Conclusion Lupus patients’ immune response, especially those taking HI, may not be similar to healthy individuals. A higher dosage of influenza vaccination may provide a better seroprotection rate. However, real-world clinical effectiveness is yet to demonstrate, and further studies are required. Disclosures All Authors: No reported disclosures

Cardiac Abnormalities in Relation to the Disease Activity Index Among Systemic Lupus Erythematosus Patients in a Tertiary Hospital: A Cross-Sectional Study.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune connective tissue disorder involving multiple organs and systems. Cardiovascular involvement in SLE patients is a major cause of morbidity and mortality. Although subclinical cardiac abnormalities exist among SLEpatients, they are rarely screened for. Echocardiography has been demonstrated to be a useful tool for the early diagnosis of cardiac abnormalities in SLE patients, many of which are clinically silent. Early recognition of cardiovascular abnormalities is vital for the prompt initiation of the appropriate management.This study aims to determine the prevalence of various structural and functional cardiac abnormalities among SLE patients and to determine its association with the modified SLE Disease Activity Index 2000 (modified SLEDAI-2K). The study was a cross-sectional study of SLE patients at the Korle-Bu Teaching Hospital (KBTH), Accra, Ghana, from June to December 2021. The setting was the rheumatology outpatient clinic of the KBTH and included adult men and women, 18 years and above, diagnosed with SLE with no known cardiac abnormalities. The baseline demographic and clinical characteristics of the participants were determined. A detailed transthoracic echocardiogramwas performed for all patients. The frequency of common cardiac pathologies was determined and compared between those with a high modified SLEDAI-2K and those with a low modified SLEDAI-2K. Ninety-nine SLE patients participated in the study with a mean age of 35.12 years. Females formed the majority (90.9%) of the participants. The mean age at diagnosis of SLE was 28.7 years and the mean disease duration was 4.6 years. All of the participants were on at least two disease-modifying medications. The mean modified SLEDAI-2K score was 9.1. Thirty-five percent (35%) of the patients had mild to moderately active disease and 39% had severely active disease. Sixty-six (66%) out of the severely active disease group had abnormal echocardiographic findings, while 28% of those with mild to moderate disease had abnormal echocardiographic findings. Echocardiographic abnormalities were found in 56 patients (47%), out of which 8.7% had valvular involvement, 15.7% had diastolic dysfunction, 5.2% had left ventricular hypertrophy (LVH), and 0.9% had left ventricular systolic dysfunction (LVSD). About 12% of the participants had pulmonary hypertension and 1% had pericardial involvement. The odds of echocardiographic abnormalities were 13.7 times higher in SLE patients with high disease activity compared to those with low disease activity (odds ratio (OR) = 13.714, 95% confidence interval (CI) = 3.804-49.442, p < 0.001). There was no significant association between cardiac abnormalities and SLE duration. No significant correlation between cardiac abnormalities and modified SLEDAI-2K score was found. Conclusion:Cardiac abnormalities, especially left ventricular diastolic dysfunction (LVDD),valvular involvement, and pulmonary hypertension, are common in SLE patients.For SLE patients, especially those with active diseases, echocardiographicassessment should be considered in the management of SLE patients to enable early detection of cardiac abnormalities, early treatment, and thus a decrease in morbidity and mortality associated with cardiovascular involvement in SLE patients.

Impact of telemedicine on disease activity assessment: A case-crossover study nested within a cohort of patients with systemic lupus erythematosus.

The utilisation of telemedicine has been rapidly growing among patients with rheumatic diseases, especially following the corona virus disease 2019 pandemic. Ease and convenience appear to dominate the reasons for this growth. However, the effects of this approach in patients with systemic lupus erythematosus (SLE) are yet to be revealed. In this study, we examined the effect of telemedicine on disease activity assessment and damage scores in patients with SLE. This case-crossover study was nested within a national prospective cohort of patients with SLE in Saudi Arabia. Patients with SLE were included if they fulfilled the Systemic Lupus International Collaborating Clinics classification criteria between March 2020 and March 2021 and were assessed at three time points with 3 months between assessments, according to the standardised protocol of this cohort. Telemedicine was conducted for the first evaluation, while in-person assessments were used at the second and third visits. The primary outcome was the difference in the SLE disease activity index 2000 (SLEDAI-2K) score. The primary analysis was conducted using the repeated measure model and adjusted for potential confounders, including demographics, medications, and changes in steroid doses. Several sensitivity analyses were conducted to mitigate selection and time-varying confounders. A total of 92 participants were included in this study. Most patients were females (88%), with a mean (±standard deviation [SD]) age of 36 (±13) years. The mean (±SD) disease activity scores at baseline were as follows: SLEDAI-2K, 5 (±5); SLE responder index, 3.8 (±3.5); Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index, 1 (±1). The mean difference in SLEDAI-2K score was -1.641 (95% confidence interval -2.773 to -0.510, p = 0.005*) between telemedicine and follow-up visits. The results were consistent in all sensitivity analyses. We found that telemedicine assessment was associated with a much higher disease activity score than subsequent assessments, which may suggest an overestimation of disease activity and later assessment accuracy. Cautious adoption has been suggested for SLE patients with active disease.