lupus

狼疮
  • 文章类型: Journal Article
    慢性移植物抗宿主病(GvHD)是异基因造血干细胞移植受者晚期死亡的主要原因,其中肾脏是一个潜在的目标。在这篇文章中,我们报道了一例极为罕见的慢性GvHD病例,以免疫复合物介导的弥漫性增生性肾小球肾炎和血清中检测到的各种自身抗体为特征;这是迄今为止报道的首例狼疮样慢性GvHD。患者对强化免疫抑制治疗反应良好,达到完全缓解。在这种情况下,霉酚酸酯比他克莫司更有效,提示慢性GvHD相关肾脏疾病的治疗应基于发病机制和病理模式。
    Chronic graft-versus-host disease (GvHD) is the leading cause of late death in allogenic hematopoietic stem cell transplantation recipients, of which the kidney is a potential target. In this article, we report an extremely rare case of chronic GvHD, characterized by immune complex-mediated diffuse proliferative glomerulonephritis and various autoantibodies detected in the serum; it is the first case of lupus-like chronic GvHD reported to date. The patient responded well to intensive immunosuppressive therapy and reached complete remission. Mycophenolate mofetil was more effective than tacrolimus in this case, suggesting that treatment of kidney diseases associated with chronic GvHD should be based on pathogenesis and pathological patterns.
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  • 文章类型: Case Reports
    硫胺素是一种必需的水溶性维生素,必须通过饮食获得。这种维生素对各种生化反应至关重要,对有氧代谢至关重要。当个体缺乏硫胺素时,这可能是由于新陈代谢过度(例如在炎症中,缺血,或者营养不良,除其他原因外),厌氧代谢可用于维持能量需求。这样的化学过程产生乳酸。过量的乳酸可引起各种临床体征和症状,尽管乳酸脱氢酶(LDH)通常可以分解该化合物。以下病例是一个非常不寻常的例子,一名51岁的白人妇女提出了持续和严重腹痛的主要投诉。经过广泛的检查,排除了许多诊断和八天的住院时间,据信她可能患有硫胺素缺乏症继发的高乳酸血症,服用这种维生素后,她的病情明显改善。人们认为这可能是由于她以前的系统性红斑狼疮(SLE)诊断,血管炎,慢性炎症,和高代谢状态,除了并发LDH故障。
    Thiamine is an essential water-soluble vitamin that must be obtained through diet. This vitamin is crucial for various biochemical reactions and is vital for aerobic metabolism. When individuals are deficient in thiamine, which can be due to hypermetabolism (such as in inflammation, ischemia, or malnutrition, among other reasons), anaerobic metabolism may be utilized to maintain energy needs. Such chemical processes produce lactic acid. Excess lactic acid can cause various clinical signs and symptoms, though lactate dehydrogenase (LDH) can typically break down this compound. The following case presents a very unusual instance where a 51-year-old Caucasian woman presented with the chief complaint of ongoing and severe abdominal pain. After an extensive work-up ruling out numerous diagnoses and an eight-day hospital stay, it was believed that she may be suffering from hyperlactatemia secondary to thiamine deficiency, as she improved significantly after administration of this vitamin. It was thought that this was likely due to her previous systemic lupus erythematosus (SLE) diagnosis, vasculitis, chronic inflammation, and a hypermetabolic state, in addition to concurrent LDH malfunction.
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  • 文章类型: Case Reports
    后巩膜炎是一种罕见的炎症性眼部疾病,影响巩膜的后段,在女性中更为普遍。其临床表现,通常是非特异性的,包括眼痛,头痛,和视力丧失。由于缺乏对视力构成潜在威胁的特定症状,误诊很常见。病因通常与风湿病有关,如类风湿性关节炎(RA),系统性红斑狼疮(SLE),和抗中性粒细胞胞浆抗体(ANCA)相关血管炎。后巩膜炎提出了诊断挑战,模仿许多其他眼部疾病,因此有必要进行全面的临床眼科检查。实验室研究,包括炎症标志物和风湿性疾病标志物,可以识别潜在的全身性疾病。成像,包括B超和磁共振成像(MRI),有助于准确诊断。治疗包括非甾体抗炎药(NSAID),以及用于轻度疾病的局部皮质类固醇和用于严重疾病的全身性皮质类固醇。对于难治性病例,生物治疗变得越来越重要。涉及眼科和风湿病的多学科方法对于这种潜在的视力威胁疾病的管理至关重要。该病例报告重点介绍了一名46岁的女性,有RA相关的后巩膜炎病史。
    Posterior scleritis is a rare inflammatory eye condition affecting the posterior segments of the sclera and is more prevalent in females. Its clinical presentation, often nonspecific, includes ocular pain, headache, and vision loss. Misdiagnosis is common due to a lack of specific symptoms posing a potential threat to vision. The etiology is often tied to rheumatic diseases, such as rheumatoid arthritis (RA), systemic erythematous lupus (SLE), and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Posterior scleritis poses diagnostic challenges, mimicking many other ocular conditions, hence necessitating a thorough clinical eye exam. Laboratory studies, including inflammatory markers and markers of rheumatic diseases, may identify underlying systemic diseases. Imaging, including B-scan ultrasound and magnetic resonance imaging (MRI), aids in accurate diagnosis. Treatment involves non-steroidal anti-inflammatory drugs (NSAID), as well as topical corticosteroids for mild disease and systemic corticosteroids for severe disease. Biologic therapy has become increasingly significant for refractory cases. A multidisciplinary approach involving ophthalmology and rheumatology is crucial in the management of this potential sight-threatening disease. This case report highlights a 46-year-old woman with a history of RA-associated posterior scleritis.
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  • 文章类型: Journal Article
    系统性红斑狼疮(SLE)影响多器官系统,最近有越来越多的证据表明癌症风险显著上升。尽管有越来越多的证据,SLE与多发性骨髓瘤(MM)之间的关系仍未得到重视。这篇综述综合了2012年至2023年发表的病例报告的结果,以探讨这种关系。我们使用PubMed进行了全面搜索,Embase,和谷歌学者用关键词“SLE”和“多发性骨髓瘤”描述了SLE患者MM的临床特点。回顾了7例病例报告。五个病例报告包括女性参与者,两个人同时诊断为SLE和MM,而在其他方面,MM随访SLE7个月至30年。2例报告MM有所改善。四例报告因并发症死亡,其中包括震惊,心肌梗塞,和肺炎。2例狼疮性肾炎合并MM和SLE综合征。较大,专注于临床表现的成熟研究,诊断策略,治疗,和结果需要更好地了解SLE和MM之间的关联。医护人员应该意识到SLE中恶性肿瘤的风险增加,并相应地定制筛查。
    Systemic lupus erythematosus (SLE) affects multiple organ systems, and there has recently been increasing evidence that suggests a considerable rise in cancer risk. Despite growing evidence, the relationship between SLE and multiple myeloma (MM) remains underlooked. This review synthesizes findings from case reports published between 2012 and 2023 to explore this relationship. We conducted a comprehensive search using PubMed, Embase, and Google Scholar with the keywords \'SLE\' and \'multiple myeloma\' and described the clinical profile of MM in patients with SLE. Seven case reports were reviewed. Five case reports included female participants, two had a simultaneous diagnosis of SLE and MM, and in others, MM followed SLE varying from 7 months to 30 years. Two cases reported an improvement in MM. Four cases reported death due to complications, which included shock, myocardial infarction, and pneumonia. Lupus nephritis was seen to complicate MM and SLE complex in 2 cases. Larger, well-developed studies focusing on clinical presentation, diagnostic strategy, treatment, and outcomes are needed to better understand the association between SLE and MM. Healthcare workers should be aware of the increased risk of malignancy in SLE and customize screening accordingly.
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  • 文章类型: Case Reports
    系统性红斑狼疮是一种自身免疫性疾病,主要影响育龄妇女。在怀孕期间,它可能导致母体和胎儿并发症。然而,妊娠诊断具有挑战性,因为该疾病模拟了与其他疾病相关的许多特征以及与妊娠相关的一些并发症.在这里,我们报告了一名妊娠26周的24岁妇女,她出现了不明原因的发烧。她出现了心动过速,恶心,疲劳,严格的,和全血细胞减少症.一旦排除了脓毒症和其他慢性疾病,咨询了风湿病学。诊断为系统性红斑狼疮后,羟氯喹的组合,硫唑嘌呤,开始使用皮质类固醇,患者表现出快速改善。她在足月分娩并不复杂。此病例报告强调了妊娠新发系统性红斑狼疮的独特表现。诊断延迟可能导致母体和胎儿并发症;然而,及时诊断和治疗可以改善症状并导致良好的妊娠结局。
    Systemic lupus erythematosus is an autoimmune disease that primarily affects women of reproductive age. In pregnancy, it can lead to maternal and fetal complications. However, diagnosis in pregnancy is challenging since the disease mimics many features associated with other disorders and some complications related to pregnancy. Here we report a 24-year-old woman at 26 weeks gestation who presented with a fever of unknown origin. She developed tachycardia, nausea, fatigue, rigors, and pancytopenia. Once sepsis and other chronic conditions were ruled out, rheumatology was consulted. Following the diagnosis of systemic lupus erythematosus, a combination of hydroxychloroquine, azathioprine, and corticosteroids was started, and the patient showed rapid improvement. She had an uncomplicated delivery at term. This case report highlights a unique presentation of new-onset systemic lupus erythematous in pregnancy. Delay in diagnosis can lead to maternal and fetal complications; however, prompt diagnosis and treatment can improve symptoms and lead to a favorable pregnancy outcome.
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  • 文章类型: Case Reports
    母亲自身免疫性疾病是先天性心脏传导阻滞(CHB)的最常见原因,一种罕见的疾病,其特征是由于母体自身抗体抗SSA/Ro和抗SSB/La引起的胎儿房室(AV)结纤维化和钙化。我们报告了一名房室结高度房室传导阻滞和钙化的女性新生儿的完整尸检和临床信息,房室结的心房入路,以及左右束分支,生于一名27岁女性,患有亚临床自身免疫性疾病。
    Maternal autoimmune disease is the most common cause of congenital heart block (CHB), a rare illness characterized by fibrosis and calcification of the fetal atrioventricular (AV) node due to maternal autoantibodies anti-SSA/Ro and anti-SSB/La. We report the full autopsy and clinical information on a female neonate with high degree AV block and calcification in the AV node, atrial approaches to the AV node, and both right and left bundle branches, born to a 27-year-old female with subclinical autoimmune disease.
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  • 文章类型: Journal Article
    狼疮患者的肾脏受累会大大增加发病率和死亡率。近年来,一些报道强调了临床和组织学发现之间的分离,并强调了肾活检作为狼疮性肾炎诊断和随访工具的作用.最初诊断时的肾活检可以早期诊断,活动和慢性评估,并检测非免疫性复合性肾炎。治疗后数月重复肾活检有助于检测持续性组织学炎症,这与未来肾脏复发的发生有关。复发时的肾活检检测到组织学变化,包括慢性瘢痕形成。最后,对接受维持免疫抑制的临床反应患者进行肾活检可能有助于治疗逐渐减少和/或暂停。支持在整个狼疮性肾炎的不同情况下使用肾活检的证据是异质的,大多数报告评估首次或复发性耀斑的诊断价值。相比之下,较少证据表明,来自重复治疗后活检和活检的额外治疗改变信息,以评估治疗逐渐减少或暂停.在这项基于临床病例的审查中,我们研究了肾活检作为改善狼疮性肾炎患者临床结局的工具的作用.
    Kidney involvement in patients with lupus highly increases morbidity and mortality. In recent years, several reports have emphasized the dissociation between clinical and histological findings and highlighted the role of kidney biopsy as an instrument for diagnosis and follow-up of lupus nephritis. The kidney biopsy at initial diagnosis allows an early diagnosis, assessment of activity and chronicity, and detection of nonimmune complex nephritis. A kidney biopsy repeated months after treatment aids in the detection of persistent histological inflammation, which has been linked to the occurrence of future kidney relapses. A kidney biopsy at a relapse detects histological changes including chronic scarring. Finally, a kidney biopsy in patients with a clinical response undergoing maintenance immunosuppression may aid therapy tapering and/or suspension. The evidence supporting the use of a kidney biopsy in different scenarios across the course of lupus nephritis is heterogeneous, with most reports assessing the value for the diagnosis of a first or relapsing flare. In contrast, less evidence suggests additional therapeutic-modifying information derived from repeat posttreatment biopsies and biopsies to evaluate treatment tapering or suspension. In this clinical case-based review, we examine the role of kidney biopsy as a tool to improve clinical outcomes of patients with lupus nephritis.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    目的:研究SARS-CoV2感染对系统性红斑狼疮(SLE)耀斑的影响。
    方法:在2022年2月至11月间符合ACR/SLICCSLE标准并有COVID-19记录的患者从我们医院的COVID-19登记中进行回顾性鉴定。没有SARS-CoV2感染的SLE对照组随机匹配年龄,性别和感染时间与感染者的比例为2:1。感兴趣的主要结果是COVID-1990天内SLE的临床发作。比较了SARS-CoV2感染的SLE患者和对照组之间的SLE发作率(轻度/中度或重度)。
    结果:研究了91名患有COVID-19的SLE患者(年龄48.6±14.0岁;95.6%为女性)和182名SLE对照(年龄48.7±13.8岁;95.6%为女性)。91例(12.1%)SARS-CoV2感染患者中有11例表现严重。1例(1.1%)患者死亡,7例(7.7%)出现严重并发症。SARS-CoV2感染90天内,14例(15.4%)患者出现轻度/中度临床SLE耀斑,2例(2.2%)患者出现重度SLE耀斑。SARS-CoV2感染患者的SLE耀斑发生率明显高于未感染患者(17.6%vs5.5%;比值比3.67[1.59-8.46];p=0.001)。抗dsDNA和补体水平的变化,然而,两组之间无显著差异。在SARS-CoV2感染的SLE患者中,有临床SLE耀斑的患者在感染前的C3值(p=0.004)显著低于无SLE耀斑的患者.
    结论:在感染SARS-CoV2的SLE患者中,90天内的临床耀斑明显多于匹配的未感染SLE对照组。
    OBJECTIVE: To study the effect of SARS-CoV2 infection on flares of systemic lupus erythematosus (SLE).
    METHODS: Patients who fulfilled the ACR/SLICC criteria for SLE and had documented COVID-19 between February and November 2022 were identified retrospectively from our hospital COVID-19 registry. SLE controls who did not have SARS-CoV2 infection were randomly matched for age, sex and the time of infection in a 2:1 ratio with those infected. The primary outcome of interest was clinical flare of SLE within 90 days of COVID-19. The rate of SLE flares (mild/moderate or severe) was compared between SARS-CoV2-infected SLE patients and controls.
    RESULTS: 91 SLE patients with COVID-19 (age 48.6 ± 14.0 years; 95.6% women) and 182 SLE controls (age 48.7 ± 13.8 years; 95.6% women) were studied. Eleven of 91 (12.1%) SARS-CoV2-infected patients had serious manifestations. One (1.1%) patient died and 7(7.7%) developed severe complications. Within 90 days of SARS-CoV2 infection, 14(15.4%) patients developed mild/moderate clinical SLE flares and 2(2.2%) patients had severe SLE flares. The incidence of SLE flares in SARS-CoV2-infected patients was significantly higher than those without the infection (17.6% vs 5.5%; odds ratio 3.67[1.59-8.46]; p = 0.001). The changes in anti-dsDNA and complement levels, however, were not significantly different between the two groups. Among SARS-CoV2-infected SLE patients, those with clinical SLE flares had significantly lower C3 values (p = 0.004) before the infection than those without.
    CONCLUSIONS: Clinical flares within 90 days were significantly more common in SLE patients infected with SARS-CoV2 than matched non-infected SLE controls.
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  • 文章类型: Journal Article
    背景:远程医疗在风湿性疾病患者中的应用迅速增长,特别是在2019年冠状病毒病大流行之后。轻松和便利似乎是这种增长的主要原因。然而,这种方法对系统性红斑狼疮(SLE)患者的疗效尚待揭示.在这项研究中,我们研究了远程医疗对SLE患者疾病活动性评估和损伤评分的影响.
    方法:本病例交叉研究是在沙特阿拉伯的SLE患者的国家前瞻性队列中进行的。如果SLE患者在2020年3月至2021年3月之间符合系统性狼疮国际合作诊所分类标准,并在三个时间点进行评估,两次评估之间间隔3个月,根据该队列的标准化方案。远程医疗进行了第一次评估,在第二次和第三次访问中使用了亲自评估。主要结果是SLE疾病活动指数2000(SLEDAI-2K)评分的差异。主要分析使用重复测量模型进行,并针对潜在的混杂因素进行调整,包括人口统计,药物,和类固醇剂量的变化。进行了几项敏感性分析,以减轻选择和时变混杂因素。
    结果:共有92名参与者被纳入本研究。大多数患者是女性(88%)。平均(±标准差[SD])年龄为36(±13)岁。基线时的平均(±SD)疾病活动评分如下:SLEDAI-2K,5(±5);SLE响应者指数,3.8(±3.5);系统性狼疮国际合作诊所/美国风湿病学会损伤指数,1(±1)。远程医疗和随访之间SLEDAI-2K评分的平均差异为-1.641(95%置信区间-2.773至-0.510,p=0.005*)。所有敏感性分析结果一致。
    结论:我们发现远程医疗评估的疾病活动性评分比后续评估高得多,这可能表明对疾病活动的高估和后来评估的准确性。对于患有活动性疾病的SLE患者,建议谨慎采用。
    BACKGROUND: The utilisation of telemedicine has been rapidly growing among patients with rheumatic diseases, especially following the corona virus disease 2019 pandemic. Ease and convenience appear to dominate the reasons for this growth. However, the effects of this approach in patients with systemic lupus erythematosus (SLE) are yet to be revealed. In this study, we examined the effect of telemedicine on disease activity assessment and damage scores in patients with SLE.
    METHODS: This case-crossover study was nested within a national prospective cohort of patients with SLE in Saudi Arabia. Patients with SLE were included if they fulfilled the Systemic Lupus International Collaborating Clinics classification criteria between March 2020 and March 2021 and were assessed at three time points with 3 months between assessments, according to the standardised protocol of this cohort. Telemedicine was conducted for the first evaluation, while in-person assessments were used at the second and third visits. The primary outcome was the difference in the SLE disease activity index 2000 (SLEDAI-2K) score. The primary analysis was conducted using the repeated measure model and adjusted for potential confounders, including demographics, medications, and changes in steroid doses. Several sensitivity analyses were conducted to mitigate selection and time-varying confounders.
    RESULTS: A total of 92 participants were included in this study. Most patients were females (88%), with a mean (±standard deviation [SD]) age of 36 (±13) years. The mean (±SD) disease activity scores at baseline were as follows: SLEDAI-2K, 5 (±5); SLE responder index, 3.8 (±3.5); Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index, 1 (±1). The mean difference in SLEDAI-2K score was -1.641 (95% confidence interval -2.773 to -0.510, p = 0.005*) between telemedicine and follow-up visits. The results were consistent in all sensitivity analyses.
    CONCLUSIONS: We found that telemedicine assessment was associated with a much higher disease activity score than subsequent assessments, which may suggest an overestimation of disease activity and later assessment accuracy. Cautious adoption has been suggested for SLE patients with active disease.
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