Lupus最新文献

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.

ObjectiveAccurate diagnosis and continuous monitoring are crucial for effective management of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Conventional biomarkers exhibit limitations regarding their sensitivity and specificity. Recent research highlights the importance of DNA methylation, particularly in the PARP9 gene, in relation to these diseases. This study examines PARP9 promoter methylation in peripheral blood mononuclear cells (PBMCs) obtained from SLE and RA patients to evaluate its diagnostic potential.MethodsIn this study, we assessed the quantitative methylation levels of the PARP9 promoter in PBMCs from 75 SLE patients, 75 RA patients, and an equal number of healthy controls using methylation-quantification of endonuclease-resistant DNA (MethyQESD) method.ResultsThe study revealed significant hypomethylation of the PARP9 promoter in both SLE and RA patients compared to control group (p < .001). The optimal cutoff values identified were 24.31% for SLE, demonstrating a sensitivity of 81.33%, and a specificity of 77.33%, and 27.73% for RA patients with a sensitivity of 77.33%, and a specificity of 70.66%). ROC curve analysis showed an AUC of 0.758 for SLE and 0.717 for RA, reflecting a moderate diagnostic accuracy (p < .001). Additionally, hypomethylation of PARP9 was negatively correlated with anti-dsDNA levels in SLE patients and with ESR and CRP levels in RA patients, while showed a positive correlation with C3 and C4 levels in SLE group (p < .001).ConclusionPARP9 promoter hypomethylation shows potential as a diagnostic biomarker for SLE and RA. The significant association between hypomethylation of PAPR9 promoter and disease activity factors in SLE and RA patients, is suggesting that PARP9 hypomethylation could be used as an alternative biomarker for monitoring of disease activity.

ObjectiveTo detect the expression levels of TWEAK and CD163 in monocytes from the peripheral blood of patients with systemic lupus erythematosus (SLE) complicated with renal involvement (SLE+RI) and to explore the application value of TWEAK and CD163 in the diagnosis of SLE and SLE+RI.MethodsThe expression levels of TWEAK and CD163 in the monocytes of 70 SLE patients and the control group were determined by real-time fluorescence quantitative polymerase chain reaction (RT‒qPCR). To analyse the relationship between TWEAK/CD163 expression levels and laboratory examination and clinical manifestations in monocytes of SLE+RI patients. The sensitivity and specificity of TWEAK and CD163 for the diagnosis and differential diagnosis of SLE+RI were analysed by receiver operating characteristic (ROC) curves. Western blot experiments were used to evaluate the protein expression of TWEAK and CD163 in monocytes.ResultsThe expression levels of TWEAK and CD163 in monocytes were significantly greater in the SLE group than in the healthy control (HC) and rheumatoid arthritis (RA) groups. The expression levels of TWEAK and CD163 in monocytes from anti-double-stranded DNA antibody (anti-dsDNA)-positive patients and patients with proteinuria were respectively greater than those from anti-dsDNA-negative patients and patients without proteinuria. The expression levels of both genes were significantly lower after treatment than before treatment in the SLE+RI group (p < 0.05). The expression levels of TWEAK and CD163 in monocytes were positively correlated with the SLE activity score (SLEDAI) in the SLE+RI group. ROC curve analysis revealed that the area under the curve (AUC) of TWEAK expression was 0.869 in the SLE+RI group. The AUC of CD163 in the SLE+RI group was 0.792, the combined expression of TWEAK and CD163 was 0.842 in the SLE+RI group. TWEAK and CD163 protein expression in monocytes from patients with SLE+RI was significantly increased compared with that in controls.ConclusionThe expression levels of TWEAK and CD163 are increased in SLE patients, and the expression levels in SLE+RI patients are greater than those in SLE-RI patients. These findings are closely related to disease activity, autoantibody production and clinical symptoms and can be used as biomarkers for the diagnosis and activity of SLE+RI.

PurposeSystemic lupus erythematosus (SLE) is a chronic autoimmune disease that associated with great mortality. However, studies on survival & predictors mortality in SLE are lacking in developing countries in Asia region. To calculate survival rates and to determine the cause and suggestive risk factors of mortality in SLE patients.Patients and methodsThis study included all SLE patients in Hasan Sadikin Lupus Registry (HSLR) cohort in Hasan Sadikin Hospital Bandung between September 2001 to December 2020. Cox-regression model was used to determine the risk factors of mortality, whereas Kaplan-Meier method used to estimate survival probabilities since diagnosis.ResultsThere were 1263 patients included in this study and 125 of them were deceased. Infection (40.8%) was the most common cause of death. The 1, 5 and 10-year survival rate among our patients were 95.6%, 89.7% and 82.1%, respectively. Male sex (HR 1.89), active SLE (HR 6.06), hemolytic anemia (HR 1.62) and serositis (HR 1.74) involvement throughout the disease, and use of methylprednisolone pulse (HR 1.75) due to high disease activity significantly affect mortality. On the other hand, the use of azathioprine (HR 0.61), mycophenolic mofetil/ mycophenolic acid (MMF/MPA, HR 0.43), and anti-malaria (chloroquine/hydroxychloroquine (CQ/HCQ)) (HR 0.5) had protective effect towards mortality.ConclusionSurvival rates of SLE patients was still low in Indonesia. Hemolytic anemia and serositis involvement throughout the disease, male sex, and active SLE, along with the use of methylprednisolone pulse were significantly affect mortality of SLE in this study. Meanwhile the use of azathioprine, MMF/MPA, and anti-malaria therapy had protective effect on SLE mortality.