Lupus最新文献

BackgroundSleep disturbances are common among patients with systemic lupus erythematosus (SLE) and significantly impair quality of life. Despite increasing awareness, determinants of poor sleep in this population remain underexplored.ObjectivesThis study aimed to assess sleep quality in Tunisian SLE patients and identify associated clinical and psychological factors.MethodsA cross-sectional study was conducted at Rabta Hospital in Tunisia from February to May 2023, including 100 SLE patients fulfilling the 2019 EULAR/ACR classification criteria. Sleep quality was measured using the Pittsburgh Sleep Quality Index (PSQI). Depression, anxiety, fatigue, pain, and quality of life were assessed using validated Arabic versions of the Hospital Anxiety and Depression Scale, Fatigue Severity Scale, Numeric Pain Rating Scale, and Lupus QoL.ResultsPoor sleep quality (PSQI ≥6) was observed in 58% of participants. Univariate analysis revealed associations between poor sleep and older age, family history of SLE, depression, anxiety, fatigue, moderate-to-severe pain, and disease activity. However, multivariate analysis identified emotional health domain as the sole independent predictor of poor sleep (OR = 0.955; p = .03).ConclusionEmotional health emerged as a key determinant of sleep quality among SLE patients in Tunisia. These findings highlight the need for integrated psychosocial interventions to improve both sleep and overall quality of life. Future longitudinal studies are needed to confirm these associations and assess causal relationships.

Background: 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.Methods: 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.Results: 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.Conclusion: 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.

BackgroundAntiphospholipid syndrome (APS) is the most common acquired hypercoagulable disorder characterized mainly by thrombotic events and obstetric morbidity. Quantifying chronic damage caused by APS is crucial for patient management. The original Damage Index for Thrombotic APS (DIAPS) was developed to address this need, but required updates to improve its comprehensiveness and specificity.ObjectiveTo report the initial content validation process of a new version of the damage index: DIAPSv2.MethodsA modified Delphi panel was conducted in two stages. Stage 1 involved a systematic scoping review of DIAPS studies, with data extracted from questionnaires and confirmed by the leading committee. These questionnaires identified items from the original instrument that needed modification, elimination, or replacement with new candidate items. Stage 2 employed the modified Delphi method over three rounds, considering expert panelists' opinions to select concepts for inclusion in DIAPSv2.ResultsIn Stage 1, evidence supported the validity of the original DIAPS but highlighted missing conditions reflecting chronic damage, such as alveolar hemorrhage and significant bleeding due to anticoagulation therapy. In Stage 2, participants from different working groups reached a consensus to evaluate 41 of the 67 items that the leading committee proposed for accurately measuring chronic damage in APS. A final consensus included 32 items. Definitions of all items were updated according to specialists' input and international recommendations for each domain.ConclusionsA collaborative and multidisciplinary consensus-based approach developed a new version of the damage index, comprising 11 domains and 32 items (an update from 10 domains and 38 items in the original version). DIAPSv2 offers a more specific tool for evaluating irreversible damage in patients with thrombotic APS.

ObjectiveCervical cancer remains a leading cause of death among women of childbearing age despite the proven efficacy of screening in reducing mortality rates. Women with systemic lupus erythematosus (SLE) are at a higher risk for cervical cancer but tend to have lower screening rates. This study aimed to assess cervical cancer screening (CCS) rates and identify factors influencing screening uptake among Korean women of childbearing age with SLE.MethodsWomen aged 20-49 with SLE and age matched controls, randomly selected at a 1:5 ratio, were identified from the 2016-2017 Korean National Health Insurance Service-National Health Information Database (NHIS-NHID). Data from 10,981 women with SLE and 54,905 controls eligible for National Health Screening Program (NHSP) in 2018-2019 were analyzed. The CCS rate was determined based on participation in Papanicolaou test among eligible individuals for NHSP. Logistic regression was used to estimate odds ratios (ORs) for factors associated with CCS uptake.ResultsThe CCS rate was significantly lower in women with SLE compared to controls (49.6% vs 52.1%, P < .0001). Logistic regression revealed that younger age, lower income, self-employment or medical aid insurance, and rural residence were associated with reduced CCS uptake in both groups. The highest CCS uptake occurred in the 40-44 age group for both women with SLE (OR 5.09, 95% CI 4.17-6.22) and controls (OR 4.65, 95% CI 4.26-5.07). Comorbidities increased CCS uptake among controls (OR 1.18, 95% CI 1.13-1.23), but were associated with mild non-significant decrease in uptake among women with SLE (OR 0.96, 95% CI 0.87-1.04).ConclusionNational CCS program is often underutilized by Korean women of childbearing age with SLE, particularly among those with lower income and those of rural residency. Targeted interventions are needed to improve screening rates and address the unique challenges faced by this high-risk population.

A baby girl, born to a 35-year-old mother, was admitted at 14.5 hours of life due to apnoea and seizures. Diffusion-weighted magnetic resonance imaging revealed multiple ischemic lesions. Laboratory screening for thrombophilia showed elevated levels of anti-cardiolipin IgG antibodies (aCL-IgG). The mother also tested positive for aCL-IgG, raising the possibility of transplacental antibody transfer. The neonate's aCL-IgG levels gradually declined and normalized by 4 months of age. Although causality cannot be established, this case suggests a potential association between transient maternal antiphospholipid antibodies and neonatal ischemic stroke.

IntroductionJuvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage.ObjectivesTo evaluate risk factors associated with damage occurrence in patients with jSLE.MethodsThis multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01-0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics.ResultsA total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (p = .006).ConclusionPersistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.

ObjectiveTo estimate the prevalence of Systemic Lupus Erythematosus (SLE) in Peru in 2017 and its association with altitude, environmental temperature, and physician density.MethodsThis ecological study was performed using population data from the 2017 Peruvian census. The number of SLE cases for each department was obtained from the National Health Registries using the ICD-10 code M32. Altitude, environmental temperature and physician density were obtained for each department from the National Institute of Statistics and Informatic (Instituto Nacional de Estadística e Informática) registries. The prevalence for each department was calculated adjusting for age and sex. Then a negative binomial regression was performed to estimate the prevalence ratio (PR) and evaluate factors associated with the prevalence of SLE.ResultsThe national prevalence of SLE was 40.2 per 100,000 people. Two age groups had the highest prevalence: 12-17 years and 30-59 years. Females exhibited a higher prevalence than males, particularly in the 30-59 age group (113.9 vs 16.1 per 100,000, respectively). An inverse relationship was observed between the age- and sex-adjusted prevalence in each department and altitude (PR 0.97; 95% CI: 0.94-0.99). On the other hand, there was a direct relationship with physician density (PR: 1.04; 95% CI: 1.01-1.07). No association was found between the adjusted prevalence and environmental temperature or latitude.ConclusionThe prevalence of SLE in Peru aligns with global estimates. The inverse relationship with altitude and the direct association with physician density suggest that environmental and healthcare access factors may influence disease distribution. Further research is needed to explore the underlying mechanisms driving these associations.

BackgroundLupus nephritis (LN) is a key manifestation of systemic lupus erythematosus (SLE).Aim of the workTo assess urinary transferrin level, renal iron accumulation and transferrin receptor (TfR) gene expression in SLE.Patients and methodsA case-control study was conducted on 80 SLE patients (40 with LN and 40 without LN), and 90 age and sex matched healthy control. Iron markers (Serum iron, ferritin, TfR gene, and urinary transferrin) were assessed in all participants. Iron accumulation and TfR gene expression were evaluated in renal biopsy for LN cases. Disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index-2K(SLEDAI2k).ResultsOf 80 patients, 85% were female. Mean SLEDAI was 15.0525 ± 1.1592, the disease duration was of mean 47.6 ± 26.56 months, and mean age was 31.32 ± 8.85. Serum and urinary iron biomarkers were significantly higher in SLE patients compared to controls except for iron which was lower in lupus patients with similar significant difference between patients subgroups. TfR gene expression in renal tissue didn't significantly differ across LN classes. Tubular iron deposition was observed histopathologicaly. Urinary transferrin showed significant correlation with activity score and proteinuria (r = 0.94, r = 0.43 and p < .0001, p = .03) while serum TfR significantly correlated with disease activity and ESR (r = 0.61, r = 0.4 and p < .0001, p = .03, respectively). TfR gene expression on renal tissue did not correlate with urinary transferrin, disease activity or other laboratory parameters.ConclusionsElevated urinary transferrin and serum TfR correlated with disease activity in lupus population and may serve as a potential non-invasive biomarker for lupus nephritis.

ObjectiveTo systematically compare the clinical effectiveness and safety of Belimumab, Rituximab and Voclosporin in the treatment of lupus nephritis based on network meta-analysis method.MethodsSystematic search of registered clinical trials in four major databases (Pubmed, Embase, Web of Science, The Cochrane Register of Clinical Trials) and ClinicalTrials.gov. According to the inclusion and exclusion criteria of the protocol, Screening of registered randomized controlled clinical trials of Belimumab, Rituximab and Voclosporin in the treatment of lupus nephritis.ResultsThis study included a total of five registered randomized controlled clinical trials involving 1212 subjects. In terms of complete renal remission, Voclosporin -1 year, High-Voclosporin-1 year, Voclosporin-2 years and Belimumab-2 years were all significantly better than placebo; At the same time, the effect of Voclosporin-1 year on complete renal remission rate was significantly better than that of Rituximab-1 year [OR = 3.2, 95% CI (1.41,7.24)]. In terms of safety, placebo was significantly better than High-Voclosporin-1 year [OR = 0.23, 95% CI (0.07,0.82)], and the difference was statistically significant; There was no significant difference in the incidence of serious adverse events.ConclusionVoclosporin and Belimumab showed significant clinical efficacy in the treatment of lupus nephritis, and the safety was not statistically different from placebo. Voclosporin had significantly better clinical efficacy than Rituximab during 1-year treatment period. Increasing the dose of Voclosporin did not significantly improve the efficacy, but it will increase the safety risk of adverse events.