Lupus Science & Medicine最新文献

Objective: Antiphospholipid syndrome (APS) is a rare autoimmune condition characterised by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies. Despite the publication of updated American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria in 2023, persistent diagnostic and therapeutic challenges remain. This qualitative study, conducted within the European Reference Network for Connective Tissue and Musculoskeletal Diseases, ReCONNET, aimed to map stakeholder-identified gaps in APS care across Europe to inform subsequent quantitative prioritisation.

Methods: Between February and April 2025, we conducted 16 semistructured interviews with purposively sampled stakeholders (clinicians across different specialties-rheumatologists, haematologists, obstetricians, neurologists, general practitioners, paediatricians, researchers and translational scientists, health systems and policy professionals, laboratory medicine experts, pharmaceutical trialists, patient and research nurses, medical students and trainees). Transcripts were thematically analysed and categorised into three domains: clinical, systemic and educational/research-related unmet needs.

Results: Over 85% of stakeholders cited delayed diagnosis as a major barrier. Eleven participants highlighted limitations of the current classification criteria in capturing all the clinical settings of APS. 75% (12 out of 16) identified lack of access to multidisciplinary teams as a barrier to optimal care. Nine respondents reported that warfarin remains the mainstay of therapy despite challenges in adherence and monitoring. Twelve stakeholders emphasised the need for integration across national or European APS registries and insufficient integration in rare disease policy frameworks. Gaps in undergraduate and postgraduate education were universally reported, with 100% of educational stakeholders noting minimal curricular exposure to APS.

Conclusion: APS remains underdiagnosed, inconsistently managed and inadequately represented in both policy and education. This study identifies quantifiable stakeholder-perceived gaps that will inform the design of Europe-wide standardised, multidisciplinary strategies to improve APS care and research infrastructure within the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases and similar networks.

Background: SLE is a chronic autoimmune disease with heterogeneous manifestations and variable outcomes. Geographic factors such as rural residence may influence disease severity, access to care and treatment adherence, yet evidence remains fragmented. This systematic review aimed to evaluate the impact of rurality on clinical outcomes in adults with SLE.

Methods: We systematically searched PubMed, Embase and Web of Science for observational studies published up to May 2025 that assessed the association between rural residence and clinical outcomes in SLE. Eligible studies included adult patients with SLE and reported at least one relevant outcome stratified by rurality. Using the Newcastle-Ottawa Scale, data were extracted on study characteristics, definitions of rurality, outcome domains and risk of bias. Due to heterogeneity in study design and outcomes, a narrative synthesis was conducted.

Results: Eight studies, including over 34 000 participants from the USA, Greece, China, Egypt, Puerto Rico and Latin America (Grupo Latino Americano de Estudio del Lupus cohort), met inclusion criteria. Definitions of rurality varied widely, encompassing administrative classifications, demographic thresholds and self-reported residence. Rural residence was often associated with delayed diagnosis, higher disease activity, lower physical quality of life, increased hospital readmissions and poorer medication adherence. Survival findings were mixed, and one study found no rural disadvantage where specialised care was available. Methodological quality was generally moderate to high.

Conclusion: Across diverse settings, rural SLE populations frequently experience worse outcomes, although this is not universal and appears to be strongly influenced by structural disadvantages rather than geography alone. Standardised definitions of rurality and multidimensional measurement approaches are needed to improve comparability and guide effective interventions. Targeted strategies-such as telemedicine, outreach programmes and policies addressing healthcare access-may help reduce inequities in SLE care.

Objectives: There is increasing recognition of the need for patient-centred lupus self-management (SM) programmes to improve outcomes. The purpose of this manuscript is to describe the underlying methodology and initial pilot results of Strategies to Embrace Living with Lupus Fearlessly (SELF): an evidence-based digital SM intervention designed for individuals with lupus based on the Transtheoretical Model of Behaviour Change.

Methods: We describe pilot testing and initial feasibility and acceptability results of SELF among individuals with lupus. Participants for the 90-day pilot test were recruited through three Centers for Disease Control & Prevention-funded lupus registry sites across the USA and the Lupus Foundation of America's constituency, including a diverse sociodemographic lupus population, using varied sources. Feasibility, acceptability and preliminary impact were assessed using data on enrolment, retention, ease of utilisation and satisfaction with SELF's features, changes in readiness for SM behaviours, impact on patient-reported outcomes (eg, fatigue interference) and user feedback.

Results: Nearly 80% of participants had moderate or high skill gaps at programme intake (ie, were in an early stage of change for a key SM skill), underscoring the importance of digital SM programmes like SELF. Among those who completed the programme, almost 60% of participants closed a skill gap by mastering one or more SM skills. Significant effects on patient-provider interactions and fatigue interference were also noted, highlighting a need for future investigation. Qualitative data were mostly positive in terms of feasibility and acceptability, with specific recommendations for future improvement.

Conclusions: SELF utilisation and pilot data indicate that SELF is generally feasible and acceptable, particularly among those needing to build lupus SM skills. Future work will explore ways to improve the digital SM intervention and reduce barriers to initial and ongoing engagement.

Objective: No studies have investigated the incidence and correlation of scarring and dyspigmentation across all cutaneous lupus erythematosus (CLE) categories and subtypes. As better characterisation of such symptoms may impact how clinicians document disease damage as well as manage patients' expectations on their disease course, we set out to investigate the relationship between dyspigmentation and scarring in CLE further.

Methods: We conducted a cross-sectional review of our IRB-approved CLE database at the University of Pennsylvania. Patients were divided by category and chronic CLE (CCLE) subtype. Dyspigmentation and scarring were quantified using the CLE Disease Area and Severity Index damage score. Data on dyspigmentation and scarring were captured from the patient visit where dyspigmentation was highest.

Results: Dyspigmentation and scarring significantly differed between CLE categories and CCLE subtypes. Across categories, CCLE had the highest medians for dyspigmentation and scarring. Across CCLE subtypes, discoid lupus erythematosus (DLE) had the highest medians for dyspigmentation and scarring. Positive correlations for dyspigmentation and scarring were seen in CCLE, DLE and LE panniculitis.

Conclusions: Dyspigmentation and scarring correlation differences highlight the importance of accurate assessment and documentation of both elements of damage as well as classification of CLE category and subtype. Our results reflect important differences in the CLE categories and CCLE subtypes which may be helpful for clinicians and patients as they navigate the course of the disease.

Objective: Lupus nephritis (LN) is associated with a poorer prognosis in Latin American populations. However, the contributing risk factors contributing to this remain to be fully elucidated. This study aimed to develop a prognostic model for poor renal outcomes in patients of mestizo descent.

Methods: We conducted a multicentre, retrospective analysis including 290 adult mestizo patients with incident, biopsy-proven pure proliferative LN (International Society of Nephrology/Renal Pathology Society (ISN/RPS) class III or IV) from nine Chilean hospitals. Clinical, biological and histological variables were assessed. The primary outcome was a composite of stage IV/V chronic kidney disease, dialysis or death. Predictive variables were selected using multivariable Cox regression, and prognostic scores were derived accordingly. Internal validation was performed via bootstrapping. External validation included 93 Mexican patients, with model performance assessed using Harrel's concordance index.

Results: Two baseline factors were independently associated with poor renal outcome: estimated glomerular filtration rate <30 mL/min/m² (HR 3.82, 95% CI 2.15 to 6.78; p<0.001) and histological chronicity index >2 (HR 2.01, 95% CI 1.18 to 3.43; p=0.01). Patients were stratified into three risk categories according to the presence of none (low risk), one (intermediate risk) or both (high risk) of these factors. The likelihood of the primary outcome increased progressively across these groups: high versus intermediate risk (HR 3.22, 95% CI 1.64 to 6.34; p=0.001), and intermediate versus low risk group (HR 2.41, 95% CI 1.35 to 4.30; p=0.003). The three-tier model was replicated in the validation cohort with a concordance index of 79% (95% CI 71% to 87%; p<0.001) between predicted and observed results.

Conclusions: Based on two readily available features at the time of diagnosis, the proposed model effectively stratifies Latin American mestizo patients with pure proliferative LN (ISN/RPS class III or IV) into three risk categories for poor renal outcome. This tool may support improved risk-based management in this high-risk population.

Objective: To identify different clusters of SLE patients in remission based on patient-reported outcomes (PROs) and clinical factors that predict cluster membership.

Methods: A cross-sectional monocentric study of adult consecutive SLE outpatients in stable clinical remission. Clinical and laboratory data were collected. At enrolment, each patient completed PROs. A hierarchical clustering analysis based on PROs exploring fatigue (Functional Assessment of Chronic Illness Therapy), disease impact (Lupus Impact Tracker), mental and physical health (mental component summary and physical component summary of the Short Form 36) was performed. Logistic regression assessed predictors of cluster membership, adjusting for demographic and clinical variables.

Results: 195 SLE patients were enrolled. Two distinct clusters emerged: a 'low symptom burden (LSB)' cluster, including 81/195 (41.5%) patients, and a 'high symptom burden (HSB)' cluster, including 114/195 (58.5%) patients. The HSB was characterised by worse scores in all PROs compared with the LSB. The HSB cluster exhibited older age at diagnosis (33.23±11.97 vs 27.75±11.57; p=0.002), less frequent previous renal involvement (33.3% vs 65.4%; p<0.001), more frequent previous neuropsychiatric lupus (17.2% vs 1.3%; p=0.001), more fibromyalgia (26.3% vs 5.3%; p=0.001) and ongoing glucocorticoid use (58.8% vs 37.0%; p=0.004). Fibromyalgia (OR 7.27, 95% CI 2.27 to 29.15, p=0.002) and glucocorticoid treatment (OR 3.05, 95% CI 1.43 to 6.77, p=0.005) were independently associated with higher likelihood of HSB membership; renal involvement was associated with LSB membership (OR 0.23, 95% CI 0.10 to 0.51, p≤0.001).

Conclusions: Among SLE patients in remission, two distinct health-related quality of life phenotypes can be identified: an HSB and an LSB. Fibromyalgia and glucocorticoid treatment emerged as independent predictors of HSB. These findings underline the need to optimise disease management and align treatment goals with patient priorities.

Introduction: SLE is a chronic autoimmune disease characterised by multisystem involvement and fluctuating clinical course, often leading to permanent organ damage. Childhood-onset SLE (cSLE) tends to be more aggressive with increased organ involvement compared with adult-onset SLE. Despite advances in treatment, there is a rising incidence of morbidity and chronic damage in cSLE patients. This study aims to evaluate the patterns of damage and identify risk factors associated with damage accrual in a cohort of cSLE patients.

Materials and methods: We conducted a retrospective cohort study of 120 patients meeting the Systemic Lupus International Collaborating Clinics 2012 criteria for cSLE, followed by the paediatric rheumatology clinic from 2004 to March 2023. After excluding 18 patients for monogenic lupus or inadequate follow-up, 102 patients were analysed. Damage accrual was assessed using the Paediatric Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (PedSDI) and associations between clinical, laboratory and demographic variables with damage accrual were evaluated using univariate and multivariate analyses.

Results: The mean age at diagnosis was 12.9 years, with a mean disease duration of 6.3 years. At the end of the study, 33.3% of patients had accrued damage (PedSDI≥1). The most frequently involved domains were growth failure (24%), renal (11.8%), neuropsychiatric (8.8%) and mucocutaneous (5.9%). Multivariate analysis revealed that higher median SLE Disease Activity Index-2000 scores, haemolytic anaemia and renal involvement were independent risk factors for damage accrual.

Conclusions: Our study confirms that higher median disease activity over time, haemolytic anaemia and renal disease are significant predictors of long-term damage in cSLE. Additionally, growth failure was the most frequently involved domain in PedSDI, followed by renal and neuropsychiatric domains. These findings underscore the importance of early and effective disease management and regular monitoring for these risk factors.

Objective: SLE affects multiple organs and is associated with increased mortality and organ damage. While treatment recommendations for non-renal SLE stratify by disease activity, no standard approach to measuring severity exists. This study aimed to estimate the prevalence and incidence of SLE in Sweden, quantify patients with moderate-to-severe disease using register-based algorithms and examine associations between disease severity and survival.

Methods: We identified adult SLE patients in the Swedish National Patient Register with at least two SLE-coded visits between July 2005 and December 2019. Annual incidence and point prevalence were calculated. SLE patients were matched 5:1 with non-SLE comparators. SLE disease severity was classified annually postdiagnosis using an adapted algorithm based on prescriptions and comorbidities. Survival between SLE patients by each severity level in the year following diagnosis was compared with matched comparators using Cox regression models.

Results: In total, 10 186 SLE patients were identified, including 5076 incident cases. The average incidence of adult SLE in Sweden between 2015 and 2019 was 4.1 cases per 100 000, with a 2019 prevalence of 93.8 per 100 000. In the first year postdiagnosis, 61% of SLE patients had moderate-to-severe disease, stabilising at approximately 45% after 3-4 years among patients still in follow-up. Across all severity levels, SLE patients had poorer survival than comparators. High severity in the year following diagnosis was independently associated with increased mortality risk compared with mild disease, regardless of age, sex or prescribed treatments.

Conclusions: Using national register data, the estimated prevalence of SLE in Sweden is higher than published figures from previous years. This is the first Swedish register-based study characterising SLE severity and its survival impact. Mortality risk is particularly increased in patients with moderate or severe SLE at diagnosis, underscoring the need for targeted strategies to improve outcomes for this group.

Objective: Negative self-perceptions of ageing are associated with decreased health-related quality of life (HRQoL) in older adults. We sought to characterise the association of self-perceptions of ageing, both positive and negative, with pain, depression and self-reported disability and frailty status in middle-aged and older adults with SLE.

Methods: We enrolled adults ≥50 years with validated SLE in a single-centre cross-sectional study. Sociodemographic characteristics and disease features were self-reported. Self-perceptions of ageing were assessed using awareness of age-related change (AARC). We assessed patient-reported outcomes, disability and frailty status using the Patient-Reported Outcomes Measurement Information System 29-Item Profile (PROMIS-29), Valued Life Activities and Fatigue, Resistance, Ambulation, Illness, Loss of Weight Scale. Associations between AARC gains (ie, positive self-perception of ageing) and losses (ie, negative self-perception of ageing) and pain interference, depression, disability and frailty status (frail ≥3/5 criteria) were assessed using linear or logistic regression and adjusted for age, race, ethnicity, and SLE disease activity and organ damage.

Results: Participants (n=80) were mostly female (95.0%) with mean age and SLE duration of 63.2 (SD=8.5) years and 23.1 (SD=14.5) years, respectively. Mean PROMIS-29 T-scores for pain interference and depression were 54.6 (SD=10.1) and 49.8 (SD=8.5), respectively; 29.9% were frail. After covariate adjustment, AARC losses, but not gains were significantly associated with pain interference (ß coefficient 0.94, 95% CI 0.33 to 1.54, p<0.01), depression (ß coefficient 0.67, 95% CI 0.04 to 1.30, p=0.04) and frailty (OR 2.09, 95% CI 1.30 to 3.37, p<0.01). After covariate adjustment, both AARC gains (ß coefficient -0.07, 95% CI -0.09 to -0.02, p<0.01) and losses (ß coefficient 0.08, 95% CI 0.03 to 0.12, p<0.01) were statistically significantly associated with disability.

Conclusions: Negative self-perception of ageing was independently associated with decreased HRQoL among middle-aged and older adults with SLE. Addressing negative self-perceptions of ageing may improve HRQoL in this population.