Lupus Science & Medicine最新文献

{"title":"Efficacy and safety of telitacicept combined with standard of care in patients with proliferative lupus nephritis: a real-world retrospective study.","authors":"Ting Tian, Aifei Zhang, Xiaoxia Liu, Pengjia Wu, Na Li, Leyan Zeng, Jun Liu, Longyan Qin, Jiashun Zeng","doi":"10.1136/lupus-2026-002191","DOIUrl":"10.1136/lupus-2026-002191","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to assess the efficacy and safety of telitacicept combined with standard of care (SoC) as induction therapy for proliferative lupus nephritis (LN) patients in a real-world setting.</p><p><strong>Methods: </strong>A total of 112 patients with biopsy-proven International Society of Nephrology/Renal Pathology Society Class III/IV (with or without Class V) LN were retrospectively enrolled. Subjects were divided into two groups: the telitacicept group (n=56) receiving telitacicept plus SoC and the control group (n=56) receiving SoC alone. The primary endpoints were the rates of complete renal response (CR) and partial renal response (PR) at week 24, while the secondary endpoints included CR and PR rates, serological profiles, disease activity markers, glucocorticoid tapering and safety assessments at weeks 12 and 52. To minimise selection bias and confounding factors, propensity score-based inverse probability of treatment weighting (IPTW) was employed. The adequacy of the covariates after IPTW was evaluated via standardised mean differences.</p><p><strong>Results: </strong>Baseline characteristics were comparable between the two groups. The telitacicept group demonstrated significant improvements in disease activity markers, including serum albumin, 24-hour urinary protein and urinary leucocyte and erythrocyte counts. At week 24, the telitacicept group achieved significantly higher CR rate (67.9% vs 39.3%, p=0.004) and PR rate (83.9% vs 67.9%, p=0.023) compared with the control group. Glucocorticoid dosages, systemic lupus erythematosus disease activity index 2000, British Isles Lupus Assessment Group and Physician's Global Assessment scores were significantly reduced in the telitacicept group. Furthermore, multivariate Cox regression analysis identified telitacicept treatment as an independent predictor for achieving CR (HR=4.43; 95% CI, 2.25 to 8.73; p<0.001).</p><p><strong>Conclusions: </strong>This real-world study demonstrates that telitacicept combined with SoC significantly improves renal response rates during the induction phase of proliferative LN while reducing glucocorticoid exposure. Consequently, telitacicept may represent a promising and effective therapeutic option for the management of proliferative LN.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13583796/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148956149","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Discordance between disease activity and long-term outcomes in DNASE1L3 deficiency: a multicentre longitudinal cohort study.","authors":"Reem Abdwani, Safiya Al Abrawi, Najla Aljaberi, Kothar Alsakiti, Shama Al Shimli, Mahadev Mal, Ruqaiya Al Jashmi, Eman Al Masroori, Ibrahim Al-Zakwani","doi":"10.1136/lupus-2026-002175","DOIUrl":"10.1136/lupus-2026-002175","url":null,"abstract":"<p><strong>Objectives: </strong>DNASE1L3 deficiency is a rare monogenic cause of early-onset SLE and hypocomplementaemic urticarial vasculitis syndrome (HUVS). While early disease manifestations are increasingly recognised, longitudinal data extending into adulthood remain scarce. In this study, we described the long-term disease trajectory, damage accrual and mortality across the largest DNASE1L3 deficiency cohort reported to date.</p><p><strong>Methods: </strong>We conducted a multicentre retrospective longitudinal cohort study of patients with genetically confirmed DNASE1L3 deficiency followed at paediatric rheumatology tertiary centres in Oman and the UAE. Primary outcomes included damage accrual, flare rates, hospitalisation frequency, serious infections and mortality. Longitudinal comparisons were made between the last paediatric assessment and most recent adult follow-up.</p><p><strong>Results: </strong>57 patients from 22 families were enrolled, most carrying the homozygous DNASE1L3 c.643delT loss of function variant. Median age at diagnosis was 5 (1.75-13) years and median disease duration was 9 (1-22) years. Initial phenotypes comprised of SLE in 36 patients (63%) and HUVS in 21 (37%). Despite a significant reduction in disease activity (SLE Disease Activity Index scores) over the follow-up period (9 vs 4; p<0.001), this was not accompanied by clear improvement in long-term outcomes. Mean Systemic Lupus Interntational Collaborating Clinics (SLICC)/American College of Rheumatology Damage Index increased numerically from 0.37±0.85 to 0.72±1.17; p=0.295. The proportion with lupus nephritis increased from 34% to 46% (p=0.07) and pulmonary involvement from 3.7% versus 11% at follow-up (p=0.289), osteoporosis increased from 23% to 41% (p=0.065) and mortality was numerically higher following transition, increasing from 8.8% to 13.5% (p=0.125).</p><p><strong>Conclusions: </strong>In the largest DNASE1L3 deficiency cohort, reduction in clinical disease activity does not prevent progressive organ damage. These findings suggest that apparent disease quiescence may mask ongoing subclinical injury, underscoring the need for sustained long-term surveillance and optimised transitional care strategies. However, the findings should be interpreted with caution in light of the small sample size in this ultra-rare disease.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560980/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148919515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development and temporal validation of a clinical prediction model to identify bacterial infection in hospitalised patients with systemic lupus erythematosus.","authors":"Rong-Qia Wu, Ze-Xin Zheng, Qiong-Bing Zheng, Hong-Jin Liang, Yao Gong, Dan-Min Wang, Zhi-Duo Hou, Ling Lin","doi":"10.1136/lupus-2026-002229","DOIUrl":"10.1136/lupus-2026-002229","url":null,"abstract":"<p><strong>Objective: </strong>To develop and temporally validate a clinical prediction model for identifying bacterial infection (BI) among hospitalised patients with SLE.</p><p><strong>Methods: </strong>We retrospectively included hospitalised patients with SLE admitted for BI or active disease between January 2023 and January 2026. Admissions from January 2023 to January 2025 formed the training cohort, and admissions from February 2025 to January 2026 formed the temporal validation cohort. BI included infection with or without concomitant active SLE; non-infected active (NA) SLE was the comparator. Candidate predictors were prespecified according to clinical relevance, prior literature, routine availability and missingness. LASSO (Least Absolute Shrinkage and Selection Operator) regression was applied across imputed datasets, and final coefficients were estimated using multivariable logistic regression and pooled using Rubin's rules. Performance was assessed by discrimination, calibration, full-workflow bootstrap internal validation, same-centre temporal validation and decision curve analysis.</p><p><strong>Results: </strong>The training cohort included 395 admissions (170 BI, 225 NA), and the validation cohort included 200 admissions (75 BI, 125 NA). The final model included age, disease duration, previous hospitalisation for BI, fever, white cell count, C reactive protein (CRP), procalcitonin, C4 hypocomplementemia and ordinal SLE-DAS (Systemic Lupus Erythematosus Disease Activity Score) category. Training area under the curve (AUC) was 0.869 (95% CI 0.833 to 0.905), exceeding CRP (0.771), procalcitonin (0.682) and the erythrocyte sedimentation rate/C reactive protein (ESR/CRP) ratio (0.736). Full-workflow bootstrap validation yielded an optimism-corrected AUC of 0.837 and corrected Brier score of 0.162. Same-centre temporal validation showed stable discrimination (AUC 0.852, 95% CI 0.794 to 0.910), acceptable calibration and preserved net benefit.</p><p><strong>Conclusions: </strong>A routinely available, interpretable model identified BI among hospitalised patients with SLE using NA SLE as the comparator. C4 hypocomplementemia and higher SLE-DAS categories should be interpreted as markers of disease activity predominance rather than protective factors against infection. Multicentre external validation and clinical impact studies are required.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13548269/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887839","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Seasonal 25-hydroxyvitamin D levels in new childhood-onset SLE: associations with disease activity and peripheral lymphocyte subsets.","authors":"Chenxi Wei, Shufeng Zhi, Jiayin Li, Lijun Jiang, Xue Zhao, Qingxiao Su, Xingjie Qi, Hui Zhao, Zanhua Rong","doi":"10.1136/lupus-2026-002044","DOIUrl":"10.1136/lupus-2026-002044","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to compare 25-hydroxyvitamin D (25-(OH)D) levels and peripheral lymphocyte subsets between children with new childhood-onset SLE and healthy controls across different seasons, and to explore the relationships of seasonal 25-(OH)D concentrations with the disease activity and peripheral lymphocyte subsets in paediatric patients with SLE.</p><p><strong>Methods: </strong>A retrospective study was conducted, enrolling 285 patients with new childhood-onset SLE and 175 healthy children. All participants were divided into a winter-spring group and a summer-autumn group according to sampling seasons. Serum 25-(OH)D levels and peripheral lymphocyte subsets were detected and quantified.</p><p><strong>Results: </strong>Compared with healthy controls, patients with SLE presented with significantly higher proportions of CD8⁺ T cells (p<0.05), alongside lower total lymphocyte counts, lower percentages of CD4⁺ T cells and Natural Killer(NK) cells, reduced CD4⁺/CD8⁺ ratio and decreased serum 25(OH)-D levels (all p<0.05). Within the SLE cohort, patients in the summer-autumn SLE group had markedly higher serum level of 25-(OH)D levels than those in the winter-spring group (18.1 vs 22.8 ng/mL; p<0.001). 25-(OH) D deficiency was correlated with a higher prevalence of lupus nephritis, higher Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores and lower serum calcium levels. In both seasonal subgroups, serum calcium and serum albumin showed weak positive correlations with serum 25-(OH)D levels. A moderate negative correlation was observed between SLEDAI-2K scores and 25-(OH)D levels in the winter-spring group, while only a weak negative correlation was detected in the summer-autumn group.</p><p><strong>Conclusions: </strong>Serum 25-(OH)D levels are affected by seasonal changes and closely correlated with disease activity in children with SLE. Patients with new childhood-onset SLE manifest abnormal peripheral lymphocyte subsets and insufficient 25-(OH)D.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536044/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148874562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic and epigenetic determinants of relapse risk in lupus nephritis.","authors":"Elisabeth Skoglund, Oliver Skoglund, Holme Vestin, Nina Oparina, Ahmed Sayadi, Francesca Faustini, Elisabet Svenungsson, Lars Ronnblom, Maria K Svensson, Juliana Imgenberg-Kreuz, Iva Gunnarsson, Christopher Sjöwall, Dag Leonard","doi":"10.1136/lupus-2026-002043","DOIUrl":"10.1136/lupus-2026-002043","url":null,"abstract":"<p><strong>Objective: </strong>Lupus nephritis (LN) is a potentially severe manifestation of SLE, often resulting in permanent kidney damage. We investigated whether polygenic and/or epigenetic risk is associated with time to LN relapse and whether this association differs by initial therapy.</p><p><strong>Methods: </strong>Caucasian patients with biopsy-verified LN between 1974 and 2022 (n=149) were genotyped using the Illumina Global Screening Array, and DNA methylation was assessed with the Illumina HumanMethylation450k BeadChip. A weighted Polygenic Risk Score (PRS) based on 55 non-Human leukocyte antigen (HLA) SLE risk gene variants and a Methylation Risk Score (MRS) based on the 17 most differentially methylated cytosine-phosphate-guanine sites identified in SLE-control comparison were calculated. Time to LN relapse was analysed using Cox proportional hazards models, adjusted for centre, sex and year of the first LN.</p><p><strong>Results: </strong>Neither PRS nor MRS was associated with time to LN relapse in the overall cohort, irrespective of treatment (PRS: HR=1.00 (0.77-1.30); p=0.99; MRS: HR=1.01 (1.00-1.02); p=0.21). No associations were observed among cyclophosphamide (CYC)-treated patients (PRS: HR=0.81 (0.55-1.20); p=0.29; MRS: HR=1.00 (0.99-1.02); p=0.89). In non-CYC-treated patients, PRS showed a non-significant trend towards shorter time to LN relapse (HR=1.26 (0.88-1.81); p=0.21), with a similar trend for MRS (HR=1.02 (1.00-1.03); p=0.066). Adding MRS to the PRS model improved performance in this subgroup (likelihood ratio test, p=0.028), with significant associations for MRS (PRS: HR=1.56 (0.97-2.50); p=0.066; MRS: HR=1.02 (1.00-1.04); p=0.037).</p><p><strong>Conclusions: </strong>Genetic and epigenetic susceptibility appears to influence LN relapse risk in a treatment-dependent manner. The absence of an association among patients receiving CYC suggests that initial treatment with CYC may attenuate the impact of genetic and epigenetic predisposition on relapse. These findings support the potential of genetic and epigenetic profiling at LN diagnosis to improve relapse risk stratification and individualise selection of treatment.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536080/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148874591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Crotonylome profiling identifies MLKL crotonylation in lupus nephritis associated with RAB1A-mTOR signalling and autophagy changes in tubular epithelial cells.","authors":"Rui-Ling Lu, Shu-Hui Meng, Quan-Zhou Peng, Yanran Chen, Xufa Yang, Hong Yang Liu, Feng-Ping Zheng, Dongzhou Liu, Xiaoping Hong","doi":"10.1136/lupus-2026-002116","DOIUrl":"10.1136/lupus-2026-002116","url":null,"abstract":"<p><strong>Objective: </strong>To investigate whether MLKL crotonylation is associated with tubular autophagy-lysosome pathway homeostasis in lupus nephritis (LN) and to explore its relationship with RAB1A-mechanistic target of rapamycin (mTOR) signalling.</p><p><strong>Methods: </strong>Crotonylome proteomics was performed in peripheral blood mononuclear cells from patients with LN, patients with systemic lupus erythematosus without nephritis and healthy controls. Renal biopsy tissues were evaluated for tubulointerstitial fibrosis and autophagy-lysosome pathway-related markers. Mechanistic studies were conducted in lipopolysaccharide-stimulated HK-2 cells. Autophagic flux was assessed using bafilomycin A1. The dependency of mTOR/autophagy changes on RAB1A was tested by siRNA-mediated knockdown.</p><p><strong>Results: </strong>MLKL was identified as a differentially crotonylated protein in LN, with increased crotonylation at K95 and K219. Kidney tissues from patients with LN showed increased fibronectin and collagen III deposition compared with controls, whereas no significant difference was observed between class IV and class V LN. LC3 signal did not differ significantly between groups, whereas LAMP1 expression and LC3-LAMP1 co-localisation were reduced in LN. In HK-2 cells, crotonylation-deficient MLKL mutants were associated with increased LC3-II and reduced p62, whereas K219Q showed the opposite pattern. Autophagic flux assays using bafilomycin A1 showed that K219R-expressing cells had higher LC3-II levels than WT cells both before and after lysosomal inhibition, with comparable BafA1-induced LC3-II accumulation, consistent with increased autophagosome formation rather than impaired lysosomal degradation. HDAC1 knockdown increased MLKL crotonylation and was accompanied by mTOR activation. MLKL crotonylation enhanced RAB1A guanriphosphat osphate (GTP) binding without altering total RAB1A abundance. RAB1A knockdown in MLKL WT-expressing cells attenuated mTOR phosphorylation and partly reversed the autophagy-suppressive marker profile. Sodium crotonate induced an autophagy-suppressive marker profile that was partly reversed by rapamycin.</p><p><strong>Conclusion: </strong>MLKL crotonylation is associated with activation of the RAB1A-mTOR axis and altered tubular autophagy-lysosome pathway homeostasis in LN. These findings suggest that tubular injury-related changes in LN may not be fully reflected by glomerulus-based classification alone.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536117/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"DIA proteomics of FFPE renal biopsies reveals two molecular subtypes of lupus nephritis and identifies APOL1 as candidate biomarker for stratification.","authors":"Lei Wang, Wuqian Wang, Yang Hao, Luan Chen, Chaojun Qi, Dongyu Liang, Li Guo, Jiangfeng Zhao, Xiaofang Sun, Shengying Qin, Liou Cao","doi":"10.1136/lupus-2026-002123","DOIUrl":"10.1136/lupus-2026-002123","url":null,"abstract":"<p><strong>Introduction: </strong>Lupus nephritis (LN) exhibits substantial clinical and pathological heterogeneity. We aimed to define proteomics-based molecular subtypes of LN and identify candidate biomarkers for subtype discrimination.</p><p><strong>Methods: </strong>We analysed formalin-fixed paraffin-embedded (FFPE) renal biopsy specimens from 292 patients with biopsy-proven LN from four tertiary hospitals using data-independent acquisition (DIA)-liquid chromatography-tandem mass spectrometry (LC-MS/MS) proteomics. Molecular subtypes were identified by non-negative matrix factorisation. Differential proteins, functional enrichment, immune pathway activity, protein-protein interaction networks and subtype-associated clinical/pathological features were evaluated. Extreme Gradient Boosting (XGBoost) with SHapley Additive exPlanations (SHAP) and Least Absolute Shrinkage and Selection Operator (LASSO) logistic regression were used to identify key subtype-related features and derive a protein panel distinguishing proliferative (class III/IV) from membranous (class V) LN.</p><p><strong>Results: </strong>Two stable molecular subtypes were identified, with 1002 differential proteins between them. Subtype_2 was enriched for interferon-related innate immunity, complement activation, phagocytosis-endocytosis-lysosome pathways and ribosome biogenesis/RNA metabolism, whereas Subtype_1 was characterised by keratinisation and epithelial structural remodelling. Subtype_2 was associated with higher serum creatinine, lower estimated glomerular filtration rate and higher chronicity index. APOL1 showed discriminatory value between subtypes, and serum ELISA demonstrated a consistent pattern with FFPE proteomic findings. A five-protein LASSO panel achieved an area under the curve of approximately 0.76 for distinguishing class III/IV from class V LN.</p><p><strong>Conclusion: </strong>DIA-based proteomic profiling of FFPE renal biopsies identifies biologically and clinically relevant LN molecular subtypes and may support tissue-informed classification and risk stratification.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13504870/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148795170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating the gap: the challenge of applying treat-to-target in routine lupus care-a national perspective.","authors":"Isabel Castrejón, María Galindo-Izquierdo, Susana Gómez, Sara Manrique-Arija, Susana Romero-Yuste, Paloma Vela, Estíbaliz Loza, Teresa Otón, Carlos Marras","doi":"10.1136/lupus-2026-001979","DOIUrl":"10.1136/lupus-2026-001979","url":null,"abstract":"<p><strong>Objective: </strong>(1) To explore practice patterns and variability in the management of systemic lupus erythematosus (SLE) in Spain, including the adoption of the treat-to-target (T2T) strategy; (2) to identify key multilevel contextual factors, barriers and facilitators; and (3) to propose strategies to overcome the identified barriers.</p><p><strong>Methods: </strong>A qualitative analysis was conducted using a contextual analysis (CA) framework based on the components of the Basel Approach for coNtextual ANAlysis. This involved collecting contextual data through a literature review and other relevant sources, as well as collecting primary data to address gaps via structured focus groups with rheumatologists.</p><p><strong>Results: </strong>Considerable variability in SLE management was described by rheumatologists across regions and healthcare centres. Major barriers included disease complexity, delayed diagnosis, workforce shortages, limited training and awareness among healthcare professionals, insufficient multidisciplinary collaboration, underutilisation and lack of standardisation of clinical indices, inadequate patient education and poor integration of patient-reported outcomes into electronic medical records. Implementing T2T and clinical guidelines was rare due to perceived complexity, time constraints, a lack of digital support and limited training. Rheumatologists proposed several strategies, including improved training, structured referral pathways, digital solutions, shared decision-making tools, practical checklists and the development of multidisciplinary care networks.</p><p><strong>Conclusions: </strong>This CA highlights significant challenges in implementing evidence-based SLE management in Spain, particularly regarding the adoption of the T2T strategy. Addressing contextual factors at the healthcare provider, organisational and patient levels is crucial for improving the quality of care and patient outcomes. These findings lay the groundwork for designing tailored national-level implementation initiatives.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13504930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148794537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and incidence of interstitial lung diseases in systemic lupus erythematosus: systematic review and meta-analyses.","authors":"Joy Kiss Semmler, Jesper Rømhild Davidsen, Josefine B H Adelhelm, Sille Fløjborg, Anne Voss, Henrik Zachar Langkilde","doi":"10.1136/lupus-2026-002101","DOIUrl":"10.1136/lupus-2026-002101","url":null,"abstract":"<p><strong>Objective: </strong>Our objective is to estimate the prevalence and incidence of interstitial lung diseases (ILDs) among adults with systemic lupus erythematosus (SLE) and to explore possible differences in reported prevalence and incidence of SLE-ILDs between clinical observational and register-based studies.</p><p><strong>Method: </strong>EMBASE, MEDLINE, Cochrane Library and Scopus were searched for eligible studies investigating SLE-ILDs prevalence and/or incidence. Risk of bias was assessed using the Joanna Briggs Institute Prevalence Critical Appraisal Tool. Data were extracted in a predefined Excel spreadsheet by two reviewers independent and blinded to each other. Pooled weighted prevalences and incidences were calculated with corresponding 95% CIs, visualised in forest plots. Heterogeneity was assessed with I² test.</p><p><strong>Results: </strong>Ninety-eight studies comprising 155 964 participants were included in the systematic review and meta-analyses. The pooled weighted SLE-ILDs prevalence was 11.0% (9.0% to 13.0%), with substantial heterogeneity (I²=99.9%), and 18% when identifying SLE-ILDs with chest high-resolution CT (HRCT) scans. Clinical observational studies reported a higher prevalence of 25.0% (19.0% to 31.0%) compared with register-based studies 7.0% (5.0% to 8.0%). The incidence analysis was based on three registry-based studies and estimated an incidence of 4.9 per 1000 person-years (-0.5 to 10.3) with substantial heterogeneity (I² = 99.8%).</p><p><strong>Conclusion: </strong>ILDs appear to be a prevalent manifestation in SLE with a pooled prevalence estimate of 11.0%, although this estimate should be interpreted with caution due to substantial heterogeneity across studies. HRCT-based studies reported a higher prevalence (18.0%), suggesting that diagnostic modality influences prevalence estimates. The incidence estimate (4.9 per 1000 person-years) should be interpreted as exploratory because it was based on only three registry-based studies. Overall, the findings suggest that ILD may be more common in SLE than previously assumed, highlighting the importance of awareness of pulmonary manifestations. We found the prevalence to be higher in clinical observational studies than register-based studies.</p><p><strong>Prospero registration number: </strong>CRD420250642237.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13504845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148794590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subclinical ocular microvascular involvement in primary antiphospholipid syndrome and antiphospholipid antibody carriers.","authors":"Maria Iacovantuono, Carolina Nesi, Mattia Iuliano, Sara Ferrigno, Elisabetta Greco, Barbara Kroegler, Raffaele Mancino, Alessio Martucci, Carlo Nucci, Maria Sole Chimenti, Ricard Cervera, Massimo Cesareo, Paola Conigliaro","doi":"10.1136/lupus-2026-002075","DOIUrl":"10.1136/lupus-2026-002075","url":null,"abstract":"<p><strong>Objectives: </strong>Investigate the role of antiphospholipid antibodies (aPL) in determining subclinical microvascular alterations in patients with primary antiphospholipid syndrome (PAPS) and antiphospholipid carriers, using posterior segment optical coherence tomography angiography (OCTA) and spectral-domain OCT (SD-OCT).</p><p><strong>Methods: </strong>In this monocentric, cross-sectional study, PAPS and aPL-carrier patients and age-matched and sex-matched healthy controls were enrolled. OCTA and OCT were performed to assess vessel density (VD) in the superficial and deep retinal capillary plexi and to obtain high-resolution sectional retinal images.</p><p><strong>Results: </strong>38 patients from the aPL-cohort (30 PAPS and 8 aPL-carriers) and 32 matched healthy controls were enrolled. OCTA revealed reduced superficial and deep whole en face VD, particularly at the parafoveal level. SD-OCT demonstrated thinning of the central and outer regions of peripapillary retinal nerve fibre layer and of the macular, inner and outer plexiform, nuclear and nerve fibre layers. Increased thickness was observed in the inner inferior and superior regions across all retinal layers. No significant associations were found between OCTA parameters and disease duration, thrombotic risk profile, lupus anticoagulant positivity, cumulative drug exposure or treatments with low-dose aspirin or vitamin K antagonists. However, the VD in the superficial whole en face and parafoveal regions remained significantly reduced in the aPL-cohort after adjustment for age and cardiovascular risk factors.</p><p><strong>Conclusion: </strong>Subclinical microvascular and structural damage occurs in aPL positive patients without ocular symptoms. This study suggests a possible independent effect of aPL, making the eye a unique, non-invasive window into aPL-related pathogenic mechanisms for early detection and longitudinal monitoring.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"13 2","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13504883/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148794633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}