systemic lupus erythematous

系统性红斑狼疮
  • 文章类型: Journal Article
    背景:系统性红斑狼疮(SLE)和重症肌无力(MG)是自身免疫性疾病。以前的病例报告和病例系列表明,这些疾病之间可能存在关联,以及MG胸腺切除术后SLE的风险增加。我们进行了这项研究,以确定SLE和MG在大型队列中是否相关。
    方法:我们搜索了IBMWatsonHealthExporys平台和退伍军人事务部百万退伍军人计划(MVP)数据库,以诊断SLE和MG。此外,我们检查了纳入狼疮家庭登记处(LFRR)的受试者以及诊断MG的对照.
    结果:在探索中捕获的59,780,210个人中,MG患者为25,750,SLE患者为65,370。370名受试者都有。患有MG的人患SLE的可能性是没有MG的人的10倍以上。患有这两种疾病的人更有可能是女性,非洲裔美国人,年龄比没有SLE的MG受试者年轻。此外,与未接受胸腺切除术的MG患者相比,接受胸腺切除术的MG患者发生SLE的风险增加(OR3.11,95%CI:2.12~4.55).自身免疫性疾病如恶性贫血和其他合并症如慢性肾病在发展为SLE的MG患者中明显更常见。在MVP中,SLE和MG也显著相关。在具有严格SLE分类的大型SLE队列中,SLE和MG的关联证实了SLE与MG的关联处于相似水平。
    结论:虽然患有MG和SLE的患者数量很少,SLE和MG在大型数据库和大型SLE队列中紧密相关。
    Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are autoimmune diseases. Previous case reports and case series suggest an association may exist between these diseases, as well as an increased risk of SLE after thymectomy for MG. We undertook this study to determine whether SLE and MG were associated in large cohorts.
    We searched the IBM Watson Health Explorys platform and the Department of Veterans Affairs Million Veteran Program (MVP) database for diagnoses of SLE and MG. In addition, we examined subjects enrolled in the Lupus Family Registry and Repository (LFRR) as well as controls for a diagnosis of MG.
    Among 59,780,210 individuals captured in Explorys, there were 25,750 with MG and 65,370 with SLE. 370 subjects had both. Those with MG were >10 times more likely to have SLE than those without MG. Those with both diseases were more likely to be women, African American, and at a younger age than MG subjects without SLE. In addition, the MG patients who underwent thymectomy had an increased risk of SLE compared to MG patients who had not undergone thymectomy (OR 3.11, 95% CI: 2.12 to 4.55). Autoimmune diseases such as pernicious anemia and miscellaneous comorbidities such as chronic kidney disease were significantly more common in MG patients who developed SLE. In the MVP, SLE and MG were also significantly associated. Association of SLE and MG in a large SLE cohort with rigorous SLE classification confirmed the association of SLE with MG at a similar level.
    While the number of patients with both MG and SLE is small, SLE and MG are strongly associated together in very large databases and a large SLE cohort.
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  • 文章类型: Journal Article
    背景分析皮肤红斑狼疮(CLE)住院的研究有限。在这项研究中,我们旨在分析系统性红斑狼疮(SLE)和CLE患者的基线人口统计学,找出最常见的住院原因,并找出住院结果。材料和方法我们在2016年至2019年之间使用国家(全国)住院样本(NIS)数据库进行了分析。对于CLE队列,我们提取了使用国际疾病分类-第10次修订(ICD-10)代码诊断CLE的18岁及以上成人的数据.为了比较,SLE队列由年龄在18岁及以上且使用ICD-10编码诊断为SLE的原发性或继发性患者确定.卡方检验用于比较基线人口统计学特征。多变量逻辑和线性回归用于计算感兴趣的结果。结果与SLE队列相比,CLE队列不仅年龄较大,女性比例较低,但停留时间也较短,减去医院的总费用,大多数人将医疗保险作为主要保险。SLE队列主要包括非裔美国患者,而CLE队列主要是白种人患者。心血管风险在CLE队列中更为普遍,最常见于败血症,心血管疾病,和精神健康障碍。结论我们的研究强调了门诊随访对CLE患者密切监测心血管危险因素的重要性,早期识别感染,和常规精神健康检查,以减少住院和资源利用。
    Background There are limited studies analyzing cutaneous lupus erythematosus (CLE) hospitalizations. In this study, we aimed to analyze baseline demographics of systemic lupus erythematosus (SLE) and CLE patients, identify the most common reasons for hospitalizations, and find out the hospitalization outcomes.  Materials and methods We performed the analysis using the National (Nationwide) Inpatient Sample (NIS) database between 2016 and 2019. For the CLE cohort, data for adults aged 18 years and older with the primary or secondary diagnosis of CLE using International Classification of Disease - 10th revision (ICD-10) codes were extracted. For comparison, the SLE cohort was identified by patients aged 18 years and older with primary or secondary diagnoses of SLE using ICD-10 codes. Chi-squared test was used to compare baseline demographic characteristics. Multivariable logistic and linear regression was used to calculate outcomes of interest. Results In comparison to the SLE cohort, the CLE cohort was not only older in age and lower percentage female, but also had shorter length of stay, less total hospital charge, and the majority had Medicare as primary insurance. The SLE cohort included predominantly African American patients while the CLE cohort was majority Caucasian patients. The cardiovascular risks were more prevalent in the CLE cohort and most commonly admitted for sepsis, cardiovascular disease, and mental health disorders. Conclusion Our study highlights the importance of outpatient follow-up in CLE patients to closely monitor cardiovascular risk factors, early identification of infections, and routine mental health screenings to reduce hospitalizations and resource utilization.
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  • 文章类型: Journal Article
    弱势群体,包括系统性红斑狼疮(SLE)患者,已被优先接受抗SARS-CoV-2疫苗。关于这些疫苗在SLE中的安全性的数据很少。我们的研究目的是研究抗SARS-CoV-2疫苗在SLE中的安全性。我们纳入了452例SLE患者,指七个三级中心,接种疫苗的人。共有119例(26%)在第一次和/或第二次注射后报告了副作用(SE)(最常见的SE是发烧,局部反应,疲劳,和关节痛)。有全身症状的患者和接受免疫抑制方案的患者(尤其是贝利木单抗)表现出更多的SE。此外,19(4%)在免疫接种后出现了耀斑(按器官受累分类的耀斑:6种肌肉骨骼有体质症状,四个肾,三个心肺,三个血液学,两个粘膜皮肤)。没有患者需要住院治疗,也没有死亡。此外,15个需要皮质类固醇的短暂增加,四个用类固醇脉冲治疗。一名患者需要额外的利妥昔单抗疗程。抗dsDNA,疫苗接种前中度/高度DAS,在疾病发作患者中发现贝利木单抗更为常见.抗SARS-CoV-2疫苗在SLE患者中是安全的,应该在这些患者中推荐它们,因为潜在的好处远远超过了SE的风险。可能会考虑进行治疗调整,以最大程度地减少SE风险和耀斑。
    Vulnerable subjects, including systemic lupus erythematosus (SLE) patients, have been prioritised to receive anti-SARS-CoV-2 vaccines. Few data about the safety of these vaccines in SLE are available. The aim of our study is to investigate the safety of anti-SARS-CoV-2 vaccines in SLE. We included 452 SLE patients, referring to seven tertiary centres, who were immunised. A total of 119 (26%) reported side effects (SE) after the first and/or the second shot (the most frequent SE were fever, local reaction, fatigue, and arthralgia). Patients with constitutional symptoms and those on an immunosuppressive regimen (especially belimumab) showed more SE. In addition, 19 (4%) had a flare after the immunisation (flares classified by organ involvement: six musculoskeletal with constitutional symptoms, four renal, three cardio-respiratory, three haematological, two mucocutaneous). None of the patients needed hospitalisation and none died. Moreover, 15 required a transient increase in corticosteroids and four were treated with steroid pulses. One patient required an additional rituximab course. Anti-dsDNA, moderate/high DAS before vaccine, and belimumab were found more frequently in patients with disease flare. Anti-SARS-CoV-2 vaccines are safe in SLE patients, and they should be recommended in these patients, as the potential benefits widely outweigh the risk of SE. Treatment adjustment might be considered with the aim of minimising SE risk and flare.
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  • 文章类型: Journal Article
    BACKGROUND: Disease-specific factors that predispose patients to diverse cardiac diseases in systemic lupus erythematosus (SLE) have been established. The aim of this study was to identify risk factors for cardiac involvement in patients with SLE drawn from the Korean Lupus Network (KORNET) registry.
    METHODS: A total of 437 patients with SLE recruited from the KORNET registry were included in the analysis. The Cox proportional hazard model was used to identify risk factors for the development of cardiac involvement during the follow-up period. The hazard ratios for risk factors of cardiac involvement were assessed using Kaplan-Meier curves and log-rank test.
    RESULTS: Of 437 patients with SLE, 12 patients (2.7%) developed new cardiac involvement during a median follow-up period of 47.6 months. Frequencies in men and in patients with anti-Sm antibody, anti-Ro antibody, and at least one Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI) score in patients with cardiac involvement were higher, compared to those without cardiac involvement (P < 0.001, P = 0.026, P = 0.015, and P < 0.001, respectively). Men gender, older age, anti-Sm antibody, SDI, and corticosteroid dosage were potent predictors for cardiac involvement in patients with SLE in the determination of risk factors for cardiac involvement. Men, anti-Sm antibody positivity, and SDI ≥ 1 increased incidence rates of cardiac involvement for (P < 0.001, P = 0.036, and P < 0.001, respectively).
    CONCLUSIONS: The results of this study reveal that SLE-related factors such as anti-Sm antibody, SDI, and corticosteroid dosage at baseline are risk factors for cardiac involvement in SLE.
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  • 文章类型: Journal Article
    OBJECTIVE: Pregnancy in women with systemic lupus erythematosus (SLE) is one of the challenges of recent studies. Women should prevent the onset of relapses with medications before and after pregnancy, and on the other hand, the effect of these medicines considers the health and development of the fetus. In this retrospective study, the effects of anti-phospholipid syndrome and the use of common drugs such as methotrexate, cyclosporine, and azathioprine and their side effects on maternal health and ultimately the development of the fetus have been investigated.
    METHODS: This study is a descriptive and retrospective epidemiologic study that was conducted in 2016 to investigate maternal and fetal complications in SLE patients. We prepared forms of data recording, including age, occupation, and other important information and then analyzed them in SPSS version 22.
    RESULTS: The results showed that the presence of anti-phospholipid syndrome in pregnant women can lead to abnormalities such as preterm, IUGR, abortion, and fetal death (P value 0.0001). It also leads to complications such as nephritis, arthritis, and preeclampsia in the mother (P value 0.003). This study suggests that methotrexate and cyclosporine medications could cause fetal developmental disorders. The P value of cyclosporine was 0.0001 and the P value of methotrexate was 0.001.
    CONCLUSIONS: Anti-phospholipid syndrome in women with SLE who intend to become pregnant can disrupt the development of the embryo. The consumption of methotrexate and cyclosporine medications before and during the pregnancy can have irreparable effects on fetal growth. Key Points • Anti-phospholipid syndrome can disrupt the development of the embryo in women with SLE who intend to become pregnant. • Methotrexate and cyclosporine consumption before and during pregnancy can affect fetal growth. • 7 to 33% of patients whose disease had been suppressed and controlled 6 months before pregnancy seams to relapse during the pregnancy. • Taking medications to control the disease during pregnancy plays an important role in the progression of pregnancy and fetus health.
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  • 文章类型: Evaluation Study
    To investigate efficacy, safety and survival of belimumab and to identify predictors of drug response and drug discontinuation in patients with active SLE in clinical practice.
    Data of SLE patients, treated with belimumab, from 11 Italian prospective cohorts were analyzed. SLEDAI-2K, anti-dsDNA, C3, C4, prednisone daily dose, DAS-28, 24-h proteinuria, CLASIa (Cutaneous LE Disease Area and Severity Index Activity) were recorded at baseline and every 6 months. SLE Responder Index-4 (SRI-4) was calculated at 12 and 24 months. Demographic and clinical features and comorbidities were included in the univariate and multivariate analysis. Adverse events were recorded at each visit. Statistics was performed using the SPSS software.
    We studied 188 SLE patients, mean follow-up 17.5 ± 10.6 months. The most frequent manifestations, which required the use of belimumab, were polyarthritis (45.2%) and skin rashes (25.5%). SRI-4 was achieved by 77.0% and 68.7% of patients at 12 and 24-months. Independent predictors of 12-month response were SLEDAI-2K ≥ 10 (OR 40.46, p = 0.001) and polyarthritis (OR 12.64, p = 0.001) and of 24-month response were SLEDAI-2K ≥ 10 (OR 15.97, p = 0.008), polyarthritis (OR 32.36, p = 0.006), and prednisone ≥7.5 mg/day (OR 9.94, p = 0.026). We observed a low rate of severe adverse events. Fifty-eight patients (30.8%) discontinued belimumab after a mean follow-up of 10.4 ± 7.5 months. The drug survival was 86.9%, 76.9%, 69.4%, 67.1%, and 61.9% at 6, 12, 18, 24, and 30 months, respectively. No factors associated with drug discontinuation were found.
    Belimumab is effective and safe when used in clinical practice setting.
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  • 文章类型: Comparative Study
    目的:为了确定抗磷脂综合征(APS)的患病率是否存在种族差异,系统性红斑狼疮(SLE)患者罗姆和白种人的临床表现和自身抗体谱.
    方法:进行了一项横断面研究,包括连续在西班牙六家医院就诊的罗姆人和白种人SLE患者的数据。社会人口特征,APS的患病率,在不同种族之间比较了SLE和APS的临床和分析特征.
    结果:纳入52例罗姆人和98名白种人SLE患者的数据。RomaSLE患者的APS风险较高(比值比2.56,95%CI1.02-6.39)和患病率较高(28.8%vs.13.3%,P=0.027)。此外,RomaSLE患者的流产患病率在统计学上显着较高(23.5%vs.10.2%,P=0.049)。关于其他APS诊断标准,罗马SLE患者的胎儿死亡发生率无统计学意义(14.3%vs.5.1%,P=0.106)和血栓事件(21.1%vs.12.2%,P=0.160)。关于SLE临床特征,罗马患者的关节炎患病率明显较高(75%vs.57.1%,P=0.034)和非显著较高的浆膜炎患病率(44.2%vs.29.6%,P=0.104),盘状病变(11.5%vs.5.1%,P=0.191),口腔溃疡(46.1%vs.34.7%,P=0.218)和网状livedo(21.1%vs.15.3%,P=0.374)。在系统性狼疮国际合作诊所损害指数或自身免疫血清学特征中没有发现统计学上的显着差异。
    结论:在RomaSLE患者中,APS的患病率和风险显著增高。此外,罗姆患者的流产患病率明显较高,而胎儿死亡和血栓事件的患病率则不明显。
    OBJECTIVE: To determine if there are ethnic differences in the prevalence of antiphospholipid syndrome (APS), clinical presentation and autoantibody profile between Roma and Caucasian patients with systemic lupus erythematosus (SLE).
    METHODS: A cross-sectional study was conducted including data from Roma and Caucasian SLE patients consecutively attending six hospitals in Spain. Socio-demographic characteristics, prevalence of APS, clinical and analytical features of SLE and APS were compared between ethnic groups.
    RESULTS: Data from 52 Roma and 98 Caucasian SLE patients were included. Roma SLE patients had a higher risk (odds ratio 2.56, 95% CI 1.02-6.39) and prevalence of APS (28.8% vs. 13.3%, P = 0.027). Furthermore, Roma SLE patients had a statistically significant higher prevalence of abortions (23.5% vs. 10.2%, P = 0.049). In relation to other APS diagnostic criteria, Roma SLE patients had a non-statistically significant higher prevalence of fetal deaths (14.3% vs. 5.1%, P = 0.106) and thrombotic events (21.1% vs. 12.2%, P = 0.160). In relation to SLE clinical features, Roma patients had a significantly higher prevalence of arthritis (75% vs. 57.1%, P = 0.034) and non-significant higher prevalence of serositis (44.2% vs. 29.6%, P = 0.104), discoid lesions (11.5% vs. 5.1%, P = 0.191), oral ulcers (46.1% vs. 34.7%, P = 0.218) and livedo reticularis (21.1% vs. 15.3%, P = 0.374). No statistically significant differences were found in the Systemic Lupus International Collaborating Clinics Damage Index or the autoimmune serological profile.
    CONCLUSIONS: Prevalence and risk of APS were significantly higher in Roma SLE patients. Furthermore, Roma patients had a significantly higher prevalence of abortions and a non-significant higher prevalence of fetal deaths and thrombotic events.
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  • 文章类型: Journal Article
    The aim of this study was to investigate the association of three polymorphisms of CD154 with risk of SLE in Chinese population. The study population comprised 770 Chinese individuals, including 350 SLE patients and 420 healthy controls. The gene polymorphism was measured using Snapshot SNP genotyping assays and confirmed by sequencing. Serum CD154 (sCD154) level was measured by ELISA. Compared with control group, sCD154 levels were significantly increased in case group (P < 0.001). The minor C allele of rs1126535 was associated with a significantly increased risk of SLE as compared to the major T allele (P < 0.001). Furthermore, an increased frequency of C-G-A haplotype was also detected in case group which associated with an increased risk of SLE (P = 0.009). Notably, patients carrying rs1126535CT/CC genotypes had a higher sCD154 level compared with that carrying rs1126535TT genotype (P < 0.05). Unfortunately, analyses on the association between rs1126535 and several clinical manifestations of SLE failed to find any significant results. In conclusion, these results indicated that CD154 gene polymorphisms may associate with the risk of SLE and may play regulation role in the expression of sCD154 in SLE patients.
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  • 文章类型: Journal Article
    The objective of this paper is to investigate drivers of cost and health-related quality of life (HRQoL) related to disease activity and fatigue among patients with systemic lupus erythematous (SLE). A questionnaire was sent to members of a patient organization with a self-reported diagnosis of SLE, requesting information on demographics and disease characteristics, medications, resource utilization, informal care, loss of productivity, fatigue and HRQoL in relation to SLE. Mean annual costs per patient were estimated from a societal perspective. HRQoL was measured through EQ-5D and fatigue was measured through a 10 cm VAS scale. Patient-reported disease activity was measured through the Systemic Lupus Activity Questionnaire (SLAQ) and corticosteroid dose. Drivers of costs and HRQoL were analyzed through regression analysis. A total of 339 patients out of 737 returned the questionnaire. Mean age was 55; 94% were female. The mean HRQoL measured through the five-item EQ-5D instrument was 0.64 and total costs were estimated at €22,594 (direct costs €7818; indirect costs €14,776). Disease activity, fatigue and corticosteroid doses had a statistically significant impact on costs and HRQoL. This study demonstrates that Swedish patients with SLE have low HRQoL and incur high societal costs and that are both associated with and most likely driven by disease activity, fatigue and corticosteroid use.
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  • 文章类型: Journal Article
    OBJECTIVE: To compare pregnancy outcomes in cutaneous lupus erythematosus (CLE) with systemic lupus erythematosus (SLE) and healthy pregnant women.
    METHODS: Cohort comparative study.
    METHODS: Two university maternity centers in Saudi Arabia and Egypt.
    METHODS: Pregnant women with CLE and SLE and healthy pregnant women.
    METHODS: Over a three-year period, 201 participants were allocated to three groups: group 1 (n = 67) contained women with CLE, group 2 (n = 67) women with SLE, and group 3 healthy controls (n = 67). Diagnosis of lupus erythematosus was based on American College of Rheumatology criteria. All participants were followed until delivery. Lupus exacerbation was evaluated by Lupus Activity Index score. ANOVA and chi-squared tests were used to compare obstetrical and neonatal outcomes, and regression analysis was used to define independent factors of adverse pregnancy outcomes.
    METHODS: Pregnancy losses, preterm labor, intrauterine growth restriction, preeclampsia, neonatal intensive care unit admissions, cesarean sections and lupus exacerbations.
    RESULTS: There was no significant difference between groups 1 and 3 in rates of pregnancy loss, preterm labor, preeclampsia, intrauterine growth restriction and neonatal intensive care admission. Group 1 had lower pregnancy loss (p = 0.005), growth restriction (p = 0.001), preeclampsia (p = 0.05), neonatal intensive care admissions (p = 0.001), cesarean section (p = 0.03), lupus exacerbations (p = 0.05) and anti-phospholipid antibodies (p = 0.02) compared with group 2. In groups 1 and 2, lupus exacerbation and anti-phospholipid antibodies were significant independent factors for adverse outcomes.
    CONCLUSIONS: Cutaneous lupus erythematosus means comparable pregnancy outcomes to those of the healthy population. Lower rates of disease exacerbation and anti-phospholipid antibodies are potential factors for better pregnancy outcome in CLE compared with SLE.
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