LUPUS

Racial differences in medication beliefs and adherence barriers among patients with systemic lupus erythematosus
Rangaswamy S, Eudy AM, Clowse MEB, Rogers J, Sadun RE, Criscione-Schreiber LG, Doss J, Maheswaranathan M, Harris N, Burshell DR and Sun K
IntroductionBlack patients with systemic lupus erythematosus (SLE) have lower medication adherence than White patients, contributing to worse health outcomes. However, racial differences in reasons for nonadherence and beliefs about medications are not well understood.MethodsWe conducted a cross-sectional analysis of Black and White patients with SLE who completed the Beliefs about Medicines Questionnaire and the SLE-specific Domains of Subjective Extent of Nonadherence survey. We compared scores by race and by adherence level within each racial group.ResultsAmong 123 patients (52% Black, 48% White), adherence was lower in Black patients (44% vs 64%, = .02). Black patients reported greater concerns about SLE medications and medication overuse and harm than White patients. Nonadherent Black patients reported weaker beliefs in SLE medication necessity and greater concerns about medication overuse and harm than adherent Black patients. Reasons for nonadherence reported by Black patients but not White patients included feeling well (45%), concerns about future fertility (14%), and doubts about their doctors and medicines (8%).ConclusionNonadherence among Black patients was uniquely associated with stronger concerns about medication overuse and harm and weaker beliefs that SLE medicines were necessary, potentially reflecting medical mistrust that may drive skipping doses when feeling well or when concerns arise. These insights can help clinicians more astutely probe and address each patient's needs to enhance medication adherence and SLE management.
A randomized controlled trial of a lifestyle intervention on wellbeing in patients with systemic lupus erythematosus: Results from "Living well with lupus"
Caruso Mazzolani B, Infante Smaira F, Mendes Sieczkowska S, Romero M, Gofinet Pasoto S, de Sá Pinto AL, Rodrigues Lima F, Braga Benatti F, Roschel H and Gualano B
BackgroundPatients with systemic lupus erythematosus (SLE) experience impaired wellbeing and elevated cardiovascular risk. Lifestyle interventions targeting exercise and nutrition may improve overall health outcomes, but evidence in this population remains limited.PurposeThis study aimed to investigate the effects of a newly developed healthy lifestyle intervention, the (LWWL) program, on overall wellbeing in patients with SLE and high cardiovascular risk. Research DesignA 6-month, parallel-group, randomized controlled trial was conducted in São Paulo, Brazil, between August 2020 and March 2023. Participants were randomly assigned to either the LWWL intervention group or a standard care control group. The LWWL program was a goal-setting, behavioral intervention including a home-based exercise program and nutritional counseling. Study SampleEighty adults with SLE and high cardiovascular risk were included. Twelve participants in the LWWL group dropped out due to personal reasons, and six in the control group did not respond to post-intervention assessments.Data Collection and/or AnalysisSecondary outcomes related to wellbeing were assessed, including quality of life (SF-36), fatigue (FACIT), functional capacity, anxiety and depression symptoms, and sleep quality. Between-group comparisons were performed at post-intervention, and complete-case sensitivity analyses were conducted. ResultsSignificant between-group differences were observed for the domain of the SF-36 (EMD [95% CI]: -1.0 [-1.6; -0.4]; = .01; ES [95% CI]: -1.03 [-1.6; -0.4]) and the FACIT fatigue score (EMD [95% CI]: 7.1 [1.6; 12.6]; = .01; ES [95% CI]: 1.24 [0.5; 2.0]) in favor of the LWWL group. No significant between-group differences were detected for other variables ( > .05). Sensitivity analyses corroborated these findings and suggested improvements in mood and strength following the program. ConclusionsThe LWWL intervention improved fatigue and the role physical domain of quality of life in SLE patients with high cardiovascular risk. This behavioral lifestyle approach represents a potentially clinically relevant strategy to enhance selected aspects of wellbeing in SLE.
Comparative efficacy of tacrolimus-based induction therapy with and without mycophenolate mofetil in lupus nephritis: A target trial emulation study
Kim YK, Lee H, Park JW, Lee EB, Ha YJ and Park JK
ObjectivesTo compare the efficacy of tacrolimus (TAC) plus glucocorticoids (GCs) with that of TAC plus mycophenolate mofetil (MMF) and GCs for the induction of remission in proliferative lupus nephritis (LN).MethodsThis multicentre cohort study, designed as a target trial emulation, included patients with biopsy-proven proliferative LN who received TAC-based induction therapy. Patients were classified into the TAC + GC and TAC + MMF + GC groups. The primary outcome was total renal response at 12 months, which was defined as a complete or partial renal response. To address baseline imbalances between the groups, inverse probability of treatment weighting (IPTW) was applied. Binary logistic regression was used to estimate the odds ratio (OR) for the renal response.ResultsIn total, 115 patients (48 with TAC + GC and 67 with TAC + MMF + GC) were included in the study. A 12-month total renal response was achieved in 16 (33.3%) patients in the TAC + GC group and 40 (59.7%) patients in the TAC + MMF + GC group (p = .009). After IPTW adjustment, the TAC + MMF + GC group showed significantly higher 12-month total renal response (IPTW-adjusted OR 2.84 [1.31-6.35]). Adverse drug reactions occurred in 7 patients in the TAC + GC group and 11 patients in the TAC + MMF + GC group.ConclusionsIn patients with proliferative LN, TAC + MMF + GC therapy was associated with a significantly higher 12-month renal response than TAC + GC. These findings support TAC + MMF + GC as the preferred TAC-based induction regimen for proliferative LN.
Thrombocytopenia in systemic lupus erythematosus real-world insights from the nationwide RISE database
Pamuk ON, Fitzpatrick J, Li J, Schmajuk G and Magrey MN
ObjectivesSystemic lupus erythematosus (SLE) is an autoimmune rheumatic disease with a highly heterogeneous presentation. Thrombocytopenia (TP) is an uncommon manifestation in SLE patients and is often associated with major organ involvement and poor prognosis. In this study, we aimed to evaluate the prevalence of TP in SLE patients and analyze its association with demographic and clinical factors.MethodsWe utilized data from the ACR's Rheumatology Informatics System for Effectiveness (RISE) registry, a large, electronic health record-enabled database that collects data as part of routine clinical care. The study included patients who were ≥18 years old, had ≥2 SLE diagnostic codes recorded ≥30 days apart between 2016 and 2022, and had a valid complete blood count (CBC) recorded within 1 year of the second SLE code. Thrombocytopenia (TP) was classified based on the lowest platelet count within 1 year of the second SLE code: mild: 100,000-150,000/µL, moderate: 50,000-99,000/µL, and severe: <50,000/µL. We reported the frequency of TP by demographic and clinical characteristics. To identify factors associated with moderate-to-severe TP, we employed multi-level logistic regression models, controlling for covariates and accounting for clustering by practices.ResultsThe study included 30,062 patients (91.2% female; mean age: 56 years, SD: 16). The frequencies of TP and severe TP were 9.3% and 0.5%, respectively. Patients with moderate-to-severe TP had significantly higher frequencies of male sex, African-American and Hispanic ethnicity, lupus nephritis, leukopenia, anemia, hypocomplementemia, anti-dsDNA positivity, end-stage renal disease, thrombosis, hemolytic anemia, antiphospholipid syndrome, and multiple comorbidities compared to other SLE patients. Multivariable logistic regression analysis demonstrated persistent independent associations of sex, race/ethnicity, multiple comorbidities, positive dsDNA, and hypocomplementemia with an increased risk of moderate-to-severe TP in SLE.ConclusionsThe RISE registry revealed that severe TP was less common in SLE patients from community practice compared to previously reported data from selected tertiary centers. Serologically active SLE, multiple comorbidities, male sex, and non-white race were identified as independent factors associated with TP in this database. Further studies are needed to elucidate the exact mechanisms and clinical significance of TP in SLE.
Risk factors associated with tuberculosis in systemic lupus erythematosus: A case-control study in Indonesia
Wirawan C, Ghassani NG, Susandi E, Alisjahbana B, Sahiratmadja E, Rahmadi AR and Hamijoyo L
ObjectivesTuberculosis (TB) may increase morbidity and mortality of systemic lupus erythematosus (SLE) patients. The aim of the study was to determine the risk factors associated with TB in SLE patients.MethodsThis case-control study included SLE patients from the Hasan Sadikin Lupus Registry (HSLR) cohort between 2008 and 2024. Lupus patients with TB (cases) were matched by age with those without TB (controls). TB risk was estimated as adjusted OR (aOR) with 95% CI using univariate and multivariable logistic regression analyses.ResultsIn total, 90 SLE patients with TB and 270 without TB were included, predominantly female ( = 342; 95%). The median SLE duration to TB diagnosis was 30 (range 8-95) months, of which 56.7% ( = 51) had pulmonary TB, and 21.1% ( = 19) had extrapulmonary TB with meningitis and lymphadenitis being the most prevalent. Logistic regression analysis showed that SLE duration <9 months (aOR 4.04; 95% CI 1.78-9.18), history of TB contact (aOR 3.67; 95% CI 1.31-10.30), history of methylprednisolone ≥24 mg/day for ≥4 weeks (aOR 1.96; 95% CI 1.06-3.63), using <2 disease-modifying antirheumatic drugs (DMARDs) (aOR 3.34; 95% CI 1.89-5.90), and lymphopenia (aOR 2.84; CI 95% 1.65-4.91) were associated with TB ( < .05).ConclusionsAmong SLE patients living in TB endemic country, TB contact, high dose corticosteroid, and lymphopenia increase risk of TB. Of note, lower disease duration and using <2 DMARDs are also associated with TB. Further research is necessary to evaluate the need of TB prophylaxis in SLE patients with these risk factors.
Frailty in systemic lupus erythematosus:A scoping review
Qiao C, Song W, Wang Y, Xue F and Miao H
ObjectiveThis scoping review aimed to explore studies concerning frailty in patients with SLE, focusing on clarifying the prevalence of frailty, the assessment tools used, the influencing factors, and the adverse effects of frailty on the health outcomes of patients with SLE, and to suggest future research directions.MethodsA systematic search was conducted in PubMed, Web of Science, Embase, Cochrane Library, China National Knowledge Infrastructure (CNKI), Wanfang, WeiPu (VIP), and China Biomedical Literature Service System for studies related to frailty in SLE patients. The search timeframe extended from the inception of the databases to July 26, 2025. Information from the included literature was extracted and summarized.ResultsA total of 22 studies were included. Qualitative age stratification reveals higher frailty prevalence in elderly SLE patients (43.7%-83.5%) versus stable rates in non-elderly adults (16.0%-28.8%); unstratified cohorts show notably wider variation (6.2%-80.9%). Seven evaluation tools were identified, with SLICC-FI and FP being the most frequently used. Influencing factors were categorized as sociodemographic, disease-related, medication-related, and other factors. The adverse effects of frailty on the health outcomes of SLE patients included increased emergency department utilization, hospitalization rate, readmission rate, mortality, and risk of complications. Declines in physical function, activity ability, quality of life, and potential cumulative injuries, pain, fatigue, and disability were also observed.ConclusionA difference in frailty prevalence exists between elderly and elderly-excluded adult SLE patients, indicating the need for age-stratified management strategies. The SPPB is currently not advised for frailty assessment in SLE patients. Regarding the remaining six tools, their diverse characteristics necessitate multifactorial considerations in clinical adoption. Current evidence regarding the influencing factors of frailty in SLE remains insufficient, necessitating focused investigations of modifiable factors. While frailty substantially compromises the health status of patients with SLE, no intervention studies have been identified in the extant literature. Prioritizing intervention research is a critical pathway for delaying frailty progression and enhancing the quality of life in this vulnerable cohort.
Exploring prefrontal alpha modulation as a mechanistic treatment target in lupus-associated depression: A transcranial alternating current stimulation study
Camenzind M, Schwippel T, Saxena-Beem S, Lee YZ, Trujillo A, Huamani R, Riddle J, Zuberer A, Frohlich F and Sheikh SZ
ObjectiveThis study explored whether modulating prefrontal alpha oscillations using transcranial alternating current stimulation (tACS) could alleviate depressive symptoms in individuals with systemic lupus erythematosus (SLE).MethodsOver the course of 5 days, three individuals underwent daily 40-min sessions of 2 mA bifrontal individual alpha frequency tACS (IAF-tACS) while watching a relaxing video. Resting-state 128-channel electroencephalography (EEG) was recorded on Day 1 and Day 5 before each tACS session. Self-reported assessments of depression, anxiety, insomnia, and fatigue were administered on Day 1, Day 5, and at the 2- and 4-week follow-ups.ResultsTwo out of three participants showed a reduction in left prefrontal alpha power after 5 days of IAF-tACS. Only those with this reduction reported short-term improvements in depression, insomnia, anxiety, and well-being.ConclusionThese preliminary results suggest that modulating prefrontal alpha oscillations through tACS may offer a potential approach for alleviating depressive symptoms in individuals with SLE.
Targeting systemic lupus erythematosus (SLE) using derived compounds: A network and molecular dynamics study
Barik D, Sarangi AK, Mandal K, Panigrahi J, Mishra R and Panda AK
BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune disease with a scarcity of effective treatment options and considerable side effects linked to current therapies. , is rich in phytochemicals that have demonstrated immunomodulatory and anti-inflammatory effects, suggesting its promise as a natural therapeutic candidate for SLE.MethodsAn methodology explored the therapeutic potential of phytocompounds for SLE. Phytochemicals were obtained from Indian Medicinal Plants, Phytochemistry, and Therapeutics (IMPPAT) and KNApSAcK databases, followed by virtual screening using SwissADME, MOLSOFT, and ProTox 3.0 to identify drug-like and non-toxic candidates. Target genes were predicted using SwissTargetPrediction and STITCH, while SLE-associated genes were compiled from GeneCards and Online Mendelian Inheritance in Man (OMIM). The intersection of these genes was analyzed to construct a protein-protein interaction network, with hub genes identified through Cytoscape. Molecular docking and 100 ns Molecular Dynamic simulations, with Molecular Mechanics, General Born Surface Area (MM-GBSA) free energy calculations, were conducted for lead compounds against top hub proteins.ResultsThe study identified three phytocompounds-vanillic acid, (+)-catechin, and withanolide K-that show favorable pharmacokinetic and toxicity characteristics. Network analysis identified 161 common target genes, with Caspase 3 (CASP3), HIF1A (Hypoxia-inducible factor 1-alpha subunit), Interleukin 1 beta (IL1B), and Interleukin 6 (IL6) as significant hub proteins. Docking studies revealed (+)-catechin and withanolide K have strong binding affinities with IL6 and CASP3. Molecular dynamics simulations confirmed complex stability, and MM-GBSA calculations showed favorable binding free energies, especially in (+)-catechin-protein interactions.Conclusions(+)-Catechin and withanolide K are promising biomolecules for SLE, demonstrating a strong binding affinity with key proteins linked to the disease. These results offer a computational basis for experimental validation and the potential development of safer, plant-based therapies for SLE.
A high level of sTREM-1 predicts occurrence of thrombosis, obstetrical event or death in a prospective cohort of patients with antiphospholipid antibodies and/or systemic lupus. Results of the APLART study
Dufrost V, Foret T, Moulinet T, Lagrange J, Jacquot A, Baumann C, Clerc-Urmès I, Eschwège V, Lakomy C, Devreese KMJ, Lacolley P, Regnault V, Gibot S, Zuily S and Wahl D
ObjectivesThe main challenge in the care of patients with primary antiphospholipid syndrome (APS) or associated to systemic lupus erythematosus (SLE) is to determine whether patients will experience new events that may impair their clinical outcome. Triggering receptor expressed on myeloid cells-1 (TREM-1) is an amplifier of the Toll like receptor (TLR4) pathway, which is involved in APS. Plasma soluble TREM-1 (sTREM-1) levels indicate increased receptor activation and were significantly greater in thrombotic primary APS patients compared to controls. This prospective cohort study investigated the predictive value of plasma sTREM-1 levels at inclusion for the occurrence of thrombotic events or death in patients with APS, antiphospholipid antibodies (aPLs) and/or SLE.MethodsSerum sTREM-1 levels were measured at inclusion in 108 patients with APS, isolated aPL or SLE followed during 46 months. The primary outcomes included thrombosis, death and obstetrical morbidity. The occurrence of the first event of interest and predictors were modeled in a multivariable Cox model.ResultsDuring follow-up, 15 of the 108 patients presented with thromboses (14%), 5 patients died (5%), and 3 women experienced obstetrical morbidities (3%). Elevated serum sTREM-1 levels were an independent predictor for the occurrence of the composite outcome (HR 7.54 [95% CI; 2.44-23.31] < .001). In addition, sTREM-1 levels were greater in patients with APS than patients with isolated aPL ( < .01).ConclusionHigh levels of sTREM-1 at inclusion predicted the occurrence of a thrombotic and obstetric event or death in patients with aPL and/or SLE. Therefore, sTREM-1 represents a potential new prognostic biomarker in these patients.
Protein Kinase Cδ deficiency in Arab children: A link to fatal monogenic lupus and BCGitis susceptibility
Al-Mayouf SM, Assiri M, Al Abrawi S, Al Ghadani A, Alsonbul A and Alsaleem A
BackgroundMonogenic lupus is a rare form caused by single pathogenic gene variants, leading to diverse clinical symptoms from multi-organ involvement. Variants in () are known contributors, though remain underreported.ObjectiveTo describe the phenotypic, genetic, and outcome profiles of Arab children with monogenic lupus and deficiency.MethodsWe retrospectively reviewed medical records of children with deficiency lupus at two institutions in Saudi Arabia and Oman. The cohort included genetically confirmed and clinically suspected cases (due to unavailable testing in deceased siblings). Demographic, clinical, genetic, and follow-up outcome data were collected and analyzed.ResultsSeven children (four females) from three unrelated consanguineous Arab families were identified, with four having confirmed variants. All presented before the age of 2 years with fever and multi-organ involvement. Recurrent infections were common, with three patients developing BCGitis. All had high ANA, ds-DNA, and APL, with variable positivity for other autoantibodies. Complement studies revealed low C/C, and reduced C1q and CH in four patients. Treatment included corticosteroids and sequential immunosuppressive therapy, with five patients receiving biologic agents. While four achieved low disease activity on intensive treatment, three died due to severe disease and serious infections.ConclusionOur findings demonstrate that deficiency is associated with autosomal recessive monogenic lupus, characterized by severe and potentially fatal outcomes. They also confirm the link with BCGitis susceptibility. The observed heterogeneity in disease course and treatment response highlights the need for precision medicine and warrants further investigation.
Exploring endothelial dysfunction in SLE: cGAS-STING-IRF3 pathway activation by dsDNA
Yan Z, Li L, Shun Z, Xiaoyu Z, Wenfei M, Hong Y and Ruixian Z
: Systemic lupus erythematosus (SLE) is an autoimmune disease often associated with anti-double stranded DNA (anti-dsDNA) antibodies and endothelial dysfunction, yet the mechanisms linking extracellular DNA to vascular injury remain unclear.: We collected clinical samples from SLE patients and healthy controls, cultured human umbilical vein endothelial cells (HUVECs), and applied cell viability assays, ELISA, RT-qPCR, and Western blot to assess endothelial injury and activation pathways. We measured levels of von Willebrand factor (vWF), soluble thrombomodulin (sTM), and E-selectin in patient sera and cell culture supernatants.: SLE patients, particularly those positive for anti-dsDNA antibodies, showed significantly higher serum vWF, sTM, and E-selectin compared to controls (P < 0.05). In vitro, exposure of HUVECs to dsDNA induced a dose- and time-dependent reduction in cell viability, with an IC50 of 2.874 μg/ml, and significantly upregulated vWF, sTM, and E-selectin secretion. Mechanistically, dsDNA treatment activated the cGAS-STING-IRF3 signaling pathway, evidenced by increased mRNA and protein expression of cGAS, STING, and IRF3 (P < 0.05), as well as enhanced cytoplasmic cGAS fluorescence intensity in immunofluorescence analysis. These results demonstrate that elevated extracellular dsDNA can directly damage endothelial cells through cGAS-STING-IRF3 pathway activation, mirroring the endothelial injury observed in SLE patients.: Our findings suggest that cell-free dsDNA is a potent inducer of endothelial dysfunction, and that targeting the cGAS-STING-IRF3 axis may offer new therapeutic opportunities for SLE and other diseases marked by elevated extracellular DNA.
Corrigendum to: "Which patients with systemic lupus erythematosus in remission can withdraw low dose steroids? Results from a single inception cohort study"
Differential glomerular expression patterns of Th17-related cytokines in Mexican patients with lupus nephritis
Aguilar-Vazquez A, Pazarín-Villaseñor HL, Quirarte-Tovar EF, Chavarria-Avila E, Juarez-Gomez C, Gomez-Rios CA, Pizano-Martinez O, Rubio-Arellano ED, Esparza-Michel JA, Arteaga-Gallegos IY, Magaña-García L, Durán-Barragán S, Perez-Vazquez F, Llamas-Garcia A, Martin-Marquez BT, Martinez-Garcia EA, Cabrera-Lopez M, Soto-Vargas J, Andrade-Ortega L, Villanueva-Pérez A and Vazquez-Del Mercado M
We aimed to investigate expression of interleukin IL-17A, IL-21, IL-22, and IL-35 in lupus nephritis (LN). A cross-sectional study using immunohistochemistry (IHC) in renal biopsy to detect expression of interleukin IL-17 family and IL-35 from 20 Mexican patients with moderate to severe LN. We found predominant expression of IL-17A and IL-21 in the glomerular region. IL-22 was detected in the tubulointerstitium and inflammatory aggregates, notwithstanding IL-21 and IL-35 in fibrotic and atrophic tubules. A positive correlation was found between IL-17A expression in the glomerulus and tubulointerstitium with creatinine, urea, and blood urea nitrogen levels, whereas IL-35 expression decreases in the presence of proteinuria. IL-21 was high in disease activity and severity versus IL-35 that decreased in systemic lupus erythematosus (SLE) disease activity. These findings provide evidence for the compartment-specific expression in LN of Th17-related cytokines and IL-35 in Mexican patients.
Delayed diagnosis contributes to increased thrombotic burden and independently predicts severe organ damage in thrombotic antiphospholipid syndrome
Santos D, Abrantes AM, Costa FM, Parreira I, Lopes AR, Costa-Silva R, Chícharo AT, Martins JR, Marques JP, Cruz-Machado AR and Gaspar P
ObjectivesTo investigate the impact of time to diagnosis on damage accrual in thrombotic antiphospholipid syndrome (APS).MethodsRetrospective cohort study including 252 patients with thrombotic APS (Sydney criteria). Time to diagnosis was defined as the period from the first thrombosis to diagnosis (diagnostic delay ≥12 months). Recurrent events occurred during antithrombotic therapy. Damage was assessed using the damage index for APS (DIAPS). Risk factors for diagnosis and treatment delay (≥12 months) were assessed. Cox regression analysis was used to determine predictors of damage (DIAPS ≥1) and severe damage (DIAPS ≥ 3). Significance was set as α < 0.05.ResultsMost patients were female (76.7%) with primary APS (71.8%) and a median age at onset of 40.5 (28-51) years. The median time to diagnosis was 12 months (≥12 months, 53.6%). Damage and severe damage affected 75.4% and 21.0% of patients, respectively. Diagnosis delay was associated with higher cumulative thrombotic events, recurrent thrombosis and anticoagulation delay (all < .001). Secondary APS (HR 2.65, 95% CI 1.52-4.62) and recurrent thrombosis (HR 2.66, 95% CI 1.52-4.66) predicted severe damage but not damage. Diagnosis delay did not predict damage but predicted severe damage (HR 2.99, 95% CI 1.25-7.16), even after multivariate analysis (aHR 3.18, 95% CI 1.24-8.12).ConclusionIn thrombotic APS, diagnosis delay is associated with increased number of thrombotic events and recurrent thrombosis, and is an independent predictor of severe organ damage.
Assessment of subclinical cardiovascular risk in systemic lupus erythematosus utilizing carotid ultrasound and clinical evaluation
Mendonça JA, de Oliveira A, Lima Santos L, Vieira D, Barbosa Carbinatto R and Victor Martinez J
ObjectiveTo compare and correlate cardiovascular risk (CVR) factors through ultrasound findings, clinical, and laboratory variables.Methods37 patients underwent ultrasound assessment, with CVR clinical scores applied: Framingham and QRISK-3 (Risk for Increased Cardiovascular Disease in SLE). The SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) assessed systemic lupus erythematosus (SLE) activity.ResultsStatistically significant correlations were observed between the Framingham score, QRISK-3, and carotid artery diameter, with QIMT RF and expected values. Correlation coefficients ranged from 0.45 to 0.84, and p-values from 0.01 to <0.01. In linear regression analysis, variables influencing expected QIMT included QRISK-3, age, statin use, and diabetes mellitus, with p-values from 0.034 to <0.001. For QIMT RF, age and the Framingham score showed influence, with p-values from 0.028 to 0.004. SLEDAI did not significantly impact ultrasonographic parameters.ConclusionCarotid ultrasound was associated with in the subclinical detection of CVR in this cohort, providing complementary data to clinical evaluations.
Efficacy of adding belimumab to hydroxychloroquine for patients with serologically active clinically quiescent in systemic lupus erythematosus: A retrospective study
Yanagimoto Y, Minowa K, Yoshida E, Nakanishi E, Makiyama A, Amano H, Yamaji K and Tamura N
ObjectivesIn systemic lupus erythematosus (SLE), a serologically active clinically quiescent (SACQ) state is defined as one in which high anti-DNA antibody levels or low complement levels persist, however, disease activity remains stable. Although treatment intensification is not recommended for the SACQ state under the Treat to Target strategy, SACQ has been reported to be a risk factor for flares. The present study aimed to evaluate the efficacy of adding belimumab (BLM) to hydroxychloroquine (HCQ), the standard treatment for SLE, for patients in the SACQ state.MethodsPatients treated with BLM were defined as the exposure group and those who did not were defined as the control group. Propensity score analysis with inverse probability of treatment weighting was used to analyze outcomes. The primary outcome was analyzed using the Kaplan-Meier method to determine time-to-event achievement to reduce prednisolone (PSL) dose to 7.5 mg/day or 5 mg/day. Secondary outcomes were as follows: (1) time to flares, analyzed using the Kaplan-Meier method, and (2) difference in median PSL dose at the last observation, analyzed using the Mann-Whitney U-test.ResultsOf the 146 patients in the SACQ state who received HCQ, 27 were included in the exposure group and 107 in the control group. The primary outcome, time-to-event achievement to reduce PSL dose to 7.5 mg/day and 5 mg/day had an HR value of 1.21 (95% confidence interval (CI) 0.78-1.89, = 0.396) and 1.19 (95% CI 0.74-1.89, = 0.471) in the two groups, respectively, with no significant difference between the two groups. No significant differences were observed in other outcomes.ConclusionsAdding BLM to HCQ in patients in the SACQ state did not demonstrate effects in preventing flares or reducing glucocorticoids (GC) dose.
Eculizumab as an adjunctive rescue treatment for life-threatening childhood onset of systemic lupus erythematosus
Tiezzi AC, Zollo L, Marrani E, Pagnini I, Mastrolia MV, Maccora I, Giani T and Simonini G
Childhood-onset systemic lupus erythematosus (cSLE) often presents with aggressive manifestations that may not respond to standard immunosuppressive therapy. We describe two paediatric cSLE cases with severe life-threatening complications-autoimmune hemolytic anemia in one patient and macrophage activation syndrome with acute respiratory distress syndrome in the other-successfully managed with Eculizumab as adjunctive treatment. Both patients demonstrated rapid stabilization after Eculizumab initiation and showed favorable outcomes at their latest follow-up. These cases highlight the potential role of complement inhibition in select cSLE complications and suggest that Eculizumab may serve as a rescue therapy beyond its established use in thrombotic microangiopathy. Further studies are needed to better define its optimal use and target patient population.
The polish validation of a disease-specific patient-reported outcome measure of systemic lupus erythematosus
Pękala A, Jolly M, Fronczyk K, Czuszyńska Z, Sierakowska M, Szmyrka-Kaczmarek M and Olesińska M
BackgroundLupusPRO is a disease-targeted, patient-reported outcome measure developed for assessing the quality of life in patients with systemic lupus erythematosus. Initially, the questionnaire was validated among U.S. patients of varied ethnic backgrounds and genders. This study aims to carry out a cross-cultural adaptation and validation of the Polish-translated version of LupusPRO.MethodWe administered the Polish version of LupusPRO along with the 36-Item Short Form Health Survey (SF-36) and EQ-5D-5L questionnaire, and the Polish modification of the Hospital Anxiety and Depression Scale (HADS-M). At the same time, we collected demographics and clinical characteristics. Disease activity, damage, and exacerbation were assessed using SELENA-SLEDAI, SLICC/ACR DI and LFA. Furthermore, we tested internal consistency reliability (measured with Cronbach's alpha), test-retest reliability (using r-Pearson correlation coefficient), convergent validity (against corresponding domains of SF-36) and criterion validity (against disease activity, damage and EQ-VAS), and known group validity.ResultsA total of 199 (91% females) patients with SLE with a mean age of 42.6 ± 12.62 years participated in the study. We observed that the mean SELENA-SLEDAI reached 5.3 (±5.9) points, while SLICC/ACR DI was at 1.3 (±2.0) points. The internal consistency reliability of LupusPRO domains ranged between 0.737 and 0.925 (except for Lupus Symptoms, Social Support, Coping, and Satisfaction with care). For all domains except Social Support, test-retest reliability exceeded 0.7. Convergent validity with corresponding domains of the SF-36 was good (r > 0.5). All health-related quality of life domains performed well against disease activity and damage measures, establishing its criterion validity. Confirmatory factor analysis showed a satisfactory fit. (± expression of range).ConclusionThe Polish version of LupusPRO has proved to have fair psychometric properties among Polish patients with SLE.
Cotrimoxazole-associated atrioventricular block in lupus nephritis: Is there a new elephant in the room?
Velasco Á, Jiménez López-Guarch C, Marco Del Castillo Á, Galindo M and Morales E
BackgroundCotrimoxazole (trimethoprim-sulfamethoxazole) is widely used for infection prophylaxis in immunocompromised patients, including those with lupus nephritis. Although generally well tolerated, rare cardiovascular adverse effects may occur and are not well recognized.Case presentationWe report the case of a 43-year-old man with newly diagnosed class IV lupus nephritis who developed a high-grade atrioventricular block (AVB) shortly after initiation of cotrimoxazole prophylaxis. The patient had no previous cardiac disease, normal renal function, and normal serum potassium levels at presentation. Cotrimoxazole was discontinued immediately, leading to complete recovery of atrioventricular conduction within 7 days. No recurrence occurred during follow-up.DiscussionWhile AVB has been associated with cotrimoxazole in the context of acute kidney injury or hyperkalemia, this case demonstrates a potential direct drug-induced nodal toxicity, as no metabolic or structural abnormalities were identified. The temporal relationship, absence of alternative explanations, and reversibility after drug withdrawal support a probable causal association between cotrimoxazole and AVB.ConclusionThis case highlights the importance of considering cotrimoxazole as a potential, reversible cause of atrioventricular conduction disturbances, even in patients with normal renal and electrolyte profiles. Clinicians should be aware of this possible complication and monitor electrocardiographic changes when prescribing cotrimoxazole, particularly in patients receiving immunosuppressive therapy.
Distinct autoantibody patterns and clinical phenotypes by sex in Chinese systemic lupus erythematosus: A cross-sectional study
Xu S, Tu Z, Zhang Y, Xie S, Cai S, Xu X, Xie Z, Lin Z, Zhang K, Jiang Y and Ni W
BackgroundSystemic Lupus Erythematosus (SLE) exhibits marked sex-based difference. Understanding these sex-specific variations in clinical manifestations and autoantibody profiles, particularly within diverse ethnic cohorts like Chinese populations, is crucial for advancing personalized management strategies.MethodsWe retrospectively analyzed data from 1029 SLE patients (121 males, 908 females). Comprehensive immunological profiles, including complement C3, C4, CH50, antinuclear antibodies (ANA) titers and patterns, and antiphospholipid (aPL) antibodies, were assessed. Statistical analyses were performed to identify sex-specific prevalence and correlation patterns between autoantibodies and clinical indicators.ResultsDistinct autoantibody patterns emerged by sex. The speckled ANA pattern was more prevalent in males (84.1% vs 53.5%) and anti-Smith (Sm) antibodies were significantly higher in females (34.4% vs 19.8% in males; = .001). No sex differences were found in ANA titers or aPL antibodies. Females had significantly lower complement levels and higher mean SLEDAI-2K scores ( < .001), while males showed more cardiovascular ( = .039) and pulmonary involvement ( = .026). Sex-specific correlations were evident: males displayed positive autoantibody-autoantibody correlations (e.g., anti-Sm vs anti-RNP), whereas females showed broader correlations, particularly between complement components and disease activity, serum creatinine, and anti-double-stranded DNA (dsDNA) antibodies, reflecting established markers of disease activity and renal involvement. Anti-Sm positivity linked to lower complement in both sexes. Notably, females with anti-Sm had increased anti-dsDNA positivity and elevated SLEDAI-2K, while males with anti-Sm showed higher 24-h urinary protein ( = .02), directly linking anti-Sm to renal involvement in males, a key aspect of male SLE severity.ConclusionsThis study highlights significant sex-based variations in autoantibody patterns and their clinical associations in a Chinese SLE cohort. The distinct prevalence of ANA patterns and anti-Sm antibodies, coupled with sex-specific immunological correlation networks, underscores the complex interplay of immune factors.
Calcineurin inhibitors for initial induction treatment of lupus nephritis in children
Martínez de la Hoya F, Herrera-van Oostdam DA and Abud-Mendoza C