sle

sle
  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种影响身体几乎所有系统的多系统疾病。在这种情况下,肾脏的参与被称为狼疮性肾炎(LN)。LN是SLE的重要疾病表现之一,在发病率和死亡率方面对患者预后有很大影响。一名33岁的女性因腹痛而来到OPD,4个月以来很少出现稀便。病人也有关节痛,主要是小关节,自2个月以来。患者入院并进行所有常规检查。患者接受了食管胃十二指肠镜检查(OGD)和结肠镜检查,因为她的腹痛和稀便对常规药物无反应。食管和结肠中存在明显的水肿,在显微镜下显示嗜酸性粒细胞浸润。患者的尿常规显示蛋白质1+和24小时尿蛋白定量为1427mg/24小时。在进一步评估中,发现患者具有阳性ANA印迹(dsDNA,AMAM2,Ro52和Sm)。鉴于蛋白尿和ANA印迹阳性,计划对患者进行肾活检。患者在USG指导下接受了肾活检,发现患有狼疮性肾炎3级(ISNRPS分期)。SLE是一种累及多器官的疾病,如果最早不诊断,可能会出现严重的并发症,并导致终末期器官衰竭甚至死亡。非典型表现通常会造成诊断困境,并可能延迟诊断和治疗。早期诊断和治疗可使SLE患者获得长寿和正常的生活。诊断指南有助于诊断这种非典型表现。
    Systemic lupus erythematosus (SLE) is a multi-systemic disorder affecting almost all systems of the body. Involvement of the kidney in this condition is known as lupus nephritis (LN). LN is one of the important disease manifestations of SLE with considerable influence on patient outcomes in terms of morbidity and mortality. A 33-year-old female came to the OPD with complaints of abdominal pain, infrequent loose stools since 4 months. The patient also had joint pain, predominantly small joints, since 2 months. Patient was admitted and all routine investigations were done. Patient underwent an oesophagogastroduodenoscopy (OGD) and colonoscopy for her abdominal pain and loose stools which did not respond to routine medication. Grossly there was edema present in the oesophagus and colon which on microscopy showed eosinophilic infiltration. Urine routine of the patient showed protein 1+and 24-hour urine protein quantification of 1427 mg/24 h. On further evaluation patient was found to have a positive ANA blot (dsDNA, AMAM2, Ro52 and Sm). The patient was planned for a renal biopsy in view of the proteinuria and positive ANA blot. The patient underwent a renal biopsy under USG guidance and was found to have Lupus nephritis Class 3 (ISN RPS staging). SLE is a multi-organ involving disease which if not diagnosed at the earliest can have serious complications and lead to end stage organ failure and even death. Atypical presentations often pose a diagnostic dilemma and may delay diagnosis and treatment. Early diagnosis and treatment can give patients of SLE a long and normal life. Diagnostic guidelines have helped in the diagnosis of such atypical presentations.
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  • 文章类型: Case Reports
    病毒感染是系统性狼疮肾炎(SLE)发作的最常见诱因之一。COVID-19肺炎在SLE患者中可能很严重,这是狼疮性肾炎发作的危险因素。我们报告一例28岁女性,有狼疮性肾炎(LN)病史,COVID-19肺炎消退后复发,患有严重肾病-肾病综合征。此外,我们进行了文献综述,分析了所有描述的LN病例,接种疫苗和未接种疫苗,在COVID-19中显示,在有肾脏受累的SLE患者中,COVID-19的病程更严重,尤其是那些没有接种疫苗的人。疫苗接种是SLE等风湿性疾病患者预防COVID-19的最重要措施。我们提供的病例和数据表明,即使在感染解决后,LN复发也可能发生,并说明了疫苗接种的益处。COVID-19期间免疫抑制调节的作用以及SARS-CoV-2感染期间疾病复发的特定风险。
    Viral infections are one of the most common triggers of Systemic Lupus Nephritis (SLE) flare-ups. COVID-19 pneumonia can be severe in patients affected by SLE representing a risk factor for lupus nephritis flare. We report the case of a 28-year-old woman with a history of lupus nephritis (LN), who relapsed with severe nephritic-nephritic syndrome after the resolution of COVID-19 pneumonia. In addition, we conducted a literature review to analyze all described cases of LN, vaccinated and unvaccinated, in COVID-19 showing that the course of COVID-19 is more severe in SLE patients with renal involvement, especially in those who have not been vaccinated. Vaccination is the most important measure for preventing COVID-19 in people with rheumatic diseases such as SLE. The case and data we present suggests that LN relapses can occur even after the infection has resolved and illustrates the benefit of vaccination, the role of modulation of immunosuppression during COVID-19 and the specific risk of disease relapse during SARS-CoV-2 infection.
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  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种自身免疫性疾病,以II型和III型超敏反应为特征,影响多个器官,包括关节,心,肺,大脑,皮肤,还有肾脏.SLE患者会出现一系列症状,从发烧和关节痛到独特的蝴蝶面部皮疹。严重的并发症可能包括弥漫性肺泡出血(DAH),肺动脉高压,和狼疮性肾炎,在其他人中。其中,DAH,严重的SLE肺部并发症,涉及由于免疫复合物损伤引起的间质毛细血管和肺泡出血。此病例报告描述了最初被误诊但后来被证实患有SLE的患者。患者出现持续症状,包括咳嗽,呼吸困难,发烧,超过两周,随后在过去两天内出现血尿和咯血。症状的进展导致急性加重,导致她进入急诊科。随后的评估证实了狼疮性肾炎和DAH的诊断。此病例强调了在不明原因的全身症状的鉴别诊断中考虑SLE的重要性,并强调了迫切需要对DAH进行医疗干预以大大降低死亡率。
    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by type II and type III hypersensitivity reactions that affect multiple organs, including the joints, heart, lungs, brain, skin, and kidneys. Patients with SLE can experience a range of symptoms, ranging from fever and joint pain to a distinctive butterfly facial rash. Severe complications may encompass conditions such as diffuse alveolar hemorrhage (DAH), pulmonary hypertension, and lupus nephritis, among others. Among them, DAH, a critical pulmonary complication in SLE, involves bleeding from interstitial capillaries and alveoli due to immune complex damage. This case report describes a patient who was initially misdiagnosed but later confirmed to have SLE. The patient presented with persistent symptoms, including cough, dyspnea, and fever, over two weeks and subsequently developed hematuria and hemoptysis within the last two days. The progression of symptoms led to an acute exacerbation, resulting in her admission to the emergency department. Subsequent evaluations confirmed the diagnosis of lupus nephritis and DAH. This case highlights the importance of considering SLE in the differential diagnosis of unexplained systemic symptoms and underscores the urgent need for medical intervention in DAH to substantially reduce mortality.
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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: 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
    灾难性抗磷脂抗体综合征是一种罕见且严重的抗磷脂综合征亚型,由于血栓栓塞事件导致多系统器官衰竭,导致高死亡率。在文献中很少报道灾难性抗磷脂抗体综合征与自身免疫性甲状腺疾病之间的关联。我们报告了一例35岁以前健康的女性患有Graves\'甲状腺风暴,狼疮抗体阳性,和可能的灾难性抗磷脂抗体综合征。她的住院过程因广泛的静脉血栓栓塞而复杂化,上腔静脉综合征,血栓栓塞性中风,和Takotsubo心肌病。最终,尽管紧急治疗,但这导致8天后因严重震惊而不幸死亡。我们的病例报告讨论了自身免疫性甲状腺疾病与灾难性抗磷脂抗体综合征之间的联系。我们强调在重症患者中诊断灾难性抗磷脂抗体综合征的困难,并强调将其视为多器官衰竭和高凝状态的甲状腺毒症患者的可能原因的重要性。早期识别和及时管理对于改善这些患者的预后至关重要。
    Catastrophic antiphospholipid antibody syndrome is a rare and severe subtype of antiphospholipid syndrome with multisystemic organ failure due to thromboembolic events, resulting in high mortality rates. The association between catastrophic antiphospholipid antibody syndrome and autoimmune thyroid diseases is rarely reported in the literature. We report a case of a 35-year-old previously healthy female with Graves\' thyroid storm, positive lupus antibodies, and probable catastrophic antiphospholipid antibody syndrome. Her hospital course was complicated by extensive venous thromboembolism, superior vena cava syndrome, thromboembolic strokes, and Takotsubo cardiomyopathy. Eventually, this led to an unfortunate death secondary to profound shock after 8 days despite emergent treatment. Our case report discusses the link between autoimmune thyroid disorders and catastrophic antiphospholipid antibody syndrome. We emphasize the difficulty in diagnosing catastrophic antiphospholipid antibody syndrome in extremely ill patients and stress the significance of considering it as a possible cause in thyrotoxicosis patients with multiple organ failure and hypercoagulability. Early recognition and prompt management are crucial in improving outcomes in these patients.
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  • 文章类型: Journal Article
    暂无摘要。
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  • 文章类型: Case Reports
    自身免疫性疾病可导致影响关节以外的各种器官系统的额外症状和并发症。这些会影响眼睛,皮肤,呼吸,心脏,和肾脏系统。认识和理解这些不同的表现形式,例如在类风湿性关节炎(RA)中看到的严重眼部问题和可能危及生命的Felty综合征,对于临床医生及时识别和有效治疗这些疾病至关重要。在这种情况下,我们报道了一名因双侧巩膜炎入院的患者,被发现继发于3型多重自身免疫综合征。在病人住院期间,由于观察到的RA组合,偶然诊断出Felty综合征,脾肿大,和绝对中性粒细胞减少症。对这种情况的迅速认识使患者能够接受适当的护理,包括口服类固醇,羟氯喹,和甲氨蝶呤,降低严重并发症的风险。
    Autoimmune diseases can result in additional symptoms and complications impacting various organ systems beyond the joints. These can affect the eyes, skin, respiratory, cardiac, and renal systems. Recognizing and understanding these diverse manifestations, such as the severe eye issues seen in rheumatoid arthritis (RA) and the potentially life-threatening Felty syndrome, is crucial for clinicians to promptly identify and treat these conditions effectively. In this case presentation, we report on a patient admitted for bilateral scleritis, which was found to be secondary to multiple autoimmune syndrome type 3. During the patient\'s hospital stay, Felty syndrome was incidentally diagnosed due to the observed combination of RA, splenomegaly, and absolute neutropenia. Prompt recognition of this condition allowed the patient to receive appropriate care, including oral steroids, hydroxychloroquine, and methotrexate, decreasing the risk of severe complications.
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  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种系统性免疫疾病,典型地发生在年轻到中年妇女身上,并可能表现为皮肤,肾,血液学,神经学,和/或诊断时的其他症状。晚发性SLE或老年人SLE是一种亚型,在年龄组方面与经典SLE不同,临床症状,器官受累和严重程度。
    一名63岁的女性患者患有全血细胞减少症。患者在获得临床表现和血清学标志物后被诊断为狼疮,以及高滴度的抗核抗体和/或抗双链DNA抗体。患者接受糖皮质激素和霉酚酸酯治疗,这导致了快速反应。
    迟发性SLE占SLE患者的2-12%,发病年龄在50岁以上,导致诊断严重延误。晚发性SLE在性别和种族患病率方面与早发性SLE不同,临床症状和体征,器官损伤的发展,疾病活动和严重程度,和预后。一些研究还表明,晚期SLE患者的RF和抗Ro/抗La抗体阳性率较高,较低的补体滴度,肌酐升高和肌酐清除率降低的发生率较高。全血细胞减少症的一线治疗是糖皮质激素。在难治性病例中,可以使用利妥昔单抗和免疫抑制剂。
    当临床怀疑仍然很高时,评估SLE的任何异常表现并进行进一步的实验室和影像学检查非常重要。
    UNASSIGNED: Systemic lupus erythematosus (SLE) is a systemic immune disease that classically occurs in young to middle-aged women and may present with cutaneous, renal, haematologic, neurological, and/or other symptoms at the time of diagnosis. Late-onset SLE or SLE in the elderly is a subtype that differs from classic SLE in terms of age group, clinical symptoms, organ involvement and severity.
    UNASSIGNED: A 63-year-old female noted to have pancytopenia. The patient was diagnosed with lupus upon obtaining clinical presentations and serological marker, along with high titres of the antinuclear antibody and/or anti-double-stranded DNA antibody. The patient was managed with glucocorticoids and mycophenolate mofetil therapy, which led to a rapid response.
    UNASSIGNED: Late-onset SLE accounts for 2-12% of SLE patients with a minimum age of onset of 50 years and older, leading to significant delays in diagnosis. Late-onset SLE differs from early-onset SLE in terms of sex and ethnicity prevalence, clinical symptoms and signs, development of organ damage, disease activity and severity, and prognosis. Some studies have also shown that late-stage SLE patients have higher rates of RF and anti-Ro/anti-La antibody positivity, lower complement titre, and higher incidence of elevated creatinine and decreased creatinine clearance. First-line treatment of pancytopenia is glucocorticoid. In refractory cases, rituximab and immunosuppressants can be used.
    UNASSIGNED: It is important to assess any unusual presentation of SLEs when clinical suspicion remains high and conducting further laboratory and imaging investigation.
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  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种复杂的异质性疾病,具有多种临床表现。最近,两种药物,anifroumab和voclosorporin,已被批准用于治疗成人SLE和狼疮性肾炎(LN),分别。我们介绍了一名患有LN和难治性盘状红斑狼疮(DLE)的老年妇女的病例,他成功地接受了voclosporin和anifroummab的联合治疗,没有严重的感染。
    Systemic lupus erythematosus (SLE) is a complex heterogeneous disease with multiple clinical manifestations. Recently, two medications, anifrolumab and voclosporin, have been approved for the treatment of adults with SLE and lupus nephritis (LN), respectively. We present the case of an elderly woman with LN and refractory discoid lupus erythematosus (DLE), who was treated successfully with a combination of voclosporin and anifrolumab without major infections.
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