autoimmune disorders

自身免疫性疾病
  • 文章类型: Journal Article
    目标:了解暴露于COVID-19疫苗后发生的可能免疫起源的神经系统表现的频率和特征。此外,预先研究西班牙药物警戒系统和淋巴细胞转化试验在建立因果关系中的有用性。方法:回顾性病例研究,包括2021年1月至2022年5月神经内科收治的可能患有神经免疫疾病的患者。人口统计,从病历中收集临床和COVID-19疫苗接种前因数据.结果:共有108例患者,30例由于随访后的病因诊断不同而被排除。36名患者(46.2%)在过去3个月内接种了COVID-19疫苗(上个月为21.8%)。BioNTech-Pfizer疫苗是该组中最常见的疫苗(63.9%)。69/108为女性,平均年龄51.2岁(SD22.59),与最近未接种疫苗没有显着差异(U-MannWhitney,p=0.256)。发现的神经综合征是(接种疫苗/全部):多发性神经根神经病(8/16),脑炎(5/11),多发性硬化症复发(5/16),视神经炎(1/4),脊髓炎(3/6),颅神经病变(6/10),无菌性脑膜炎(1/3)和其他(7/11)。61.1%的病例接受了急性免疫抑制治疗,47.2%的病例临床症状完全好转,与未接种疫苗的患者没有显着差异(卡方,p=0.570)。在药物警戒办公室对11名接种疫苗的患者进行了可能的药物不良反应研究。根据西班牙药物警戒系统(SPVS)算法,在11例体外研究(淋巴细胞转化试验)对聚乙二醇-2000和聚山梨酯-80阳性的病例中,因果关系与COVID-19疫苗(评分≥4)“相关”4例。结论:服用COVID-19疫苗后出现的神经免疫疾病似乎没有重要的临床和/或进化特征。对疫苗辅料的迟发性超敏反应可能是其病理生理机制之一,而淋巴细胞转化试验是鉴定它的有用工具。
    Objectives: To know the frequency and characteristics of neurological manifestations of probable immune origin occurring after exposure to COVID-19 vaccination. In addition, to pre-study the usefulness of the Spanish pharmacovigilance system and lymphocyte transformation test in establishing causality. Methods: Retrospective case study, including patients admitted to the Neurology department from January 2021 to May 2022 with a probable neuroimmune disorder. Demographic, clinical and COVID-19 vaccination antecedent data were collected from medical records. Results: From a total of 108 patients, 30 were excluded due to a different etiological diagnosis after follow-up. Thirty-six patients (46.2%) had received the COVID-19 vaccine in the previous 3 months (21.8% during the previous month). BioNTech-Pfizer vaccine was the most frequent in this group (63.9%). 69/108 were female and mean age 51.2 years (SD 22.59), with no significant difference with not recently-vaccinated (U-Mann Whitney, p = 0.256). The neurological syndromes found were (vaccinated/total): polyradiculoneuropathy (8/16), encephalitis (5/11), multiple sclerosis relapse (5/16), optic neuritis (1/4), myelitis (3/6), cranial neuropathy (6/10), aseptic meningitis (1/3) and others (7/11). Acute immunosuppressive treatment was administered in 61.1% of cases and 47.2% presented complete clinical improvement, without significant differences with non-vaccinated patients (chi-square, p = 0.570). Eleven vaccinated patients were studied in the pharmacovigilance office for possible adverse drug reaction. Causality according to the Spanish pharmacovigilance system (SPVS) algorithm was \"Related\" to COVID-19 vaccine (score ≥ 4) in 11 cases with positive in vitro study (lymphocyte transformation test) to polyethylene glycol-2000 and polysorbate-80 in 4 cases. Conclusion: Neuroimmune disorders appearing after administration of COVID-19 vaccine do not seem to present important differentiating clinical and/or evolutive features. Delayed hypersensitivity to vaccine excipients could be one of the pathophysiological mechanisms, and lymphocyte transformation test is a useful tool to identify it.
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  • 文章类型: Case Reports
    骨髓增生异常综合征(MDS)代表了一系列与自身免疫性疾病相关的骨髓性疾病。这里,我们介绍了一例60岁男性患者,该患者表现出MDS与热自身抗体自身免疫性溶血性贫血(wAIHA)的异常共存.诊断评估,包括阳性的直接抗球蛋白测试,证实了他的贫血的自身免疫病因,尽管他的MDS低风险分类。泼尼松治疗的迅速开始导致显著的血液学和临床改善,允许不需要输血的保守管理方法。这个案例强调了确定WAIHA和MDS之间关系的重要性,特别是在低风险的情况下。此外,这些研究结果表明,在合并wAIHA和MDS的情况下,皮质类固醇治疗自身免疫性贫血的有效性.
    Myelodysplastic syndrome (MDS) represents a spectrum of myeloid disorders occasionally linked to autoimmune diseases. Here, we present a case of a 60-year-old man demonstrating an unusual coexistence of MDS with warm-autoantibody autoimmune hemolytic anemia (wAIHA). Diagnostic evaluation, including positive direct antiglobulin testing, confirmed the autoimmune etiology of his anemia despite his low-risk MDS classification. Prompt initiation of prednisone therapy resulted in significant hematological and clinical improvement, allowing for a conservative management approach without transfusion requirements. This case underscores the importance of identifying the relationship between wAIHA and MDS, particularly in low-risk scenarios. Moreover, these findings suggest the efficacy of corticosteroids in managing autoimmune anemia in the context of concomitant wAIHA and MDS.
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  • 文章类型: Case Reports
    恶性贫血,源于维生素B12缺乏和影响内在因子产生的自身免疫过程,由于模糊的初始症状,在早期诊断中提出了挑战。该病例报告介绍了并发HLA-B27关节病的患者中恶性贫血诱发的周围神经病变的独特发生。强调自身免疫机制的复杂相互作用。虽然HLA-B27通常与恶性贫血无关,该病例强调了探索特定HLA单倍型对于了解自身免疫性疾病的细微差别表现的重要性.在有恶性贫血迹象的个体中,全面筛查抗内在因子和抗壁细胞抗体至关重要,尤其是那些有HLA-B27关节病病史的人,指导量身定制的管理策略。本报告有助于不断探索恶性贫血中复杂的自身免疫景观。
    Pernicious anemia, stemming from Vitamin B12 deficiency and autoimmune processes affecting intrinsic factor production, presents challenges in early diagnosis due to vague initial symptoms. This case report introduces a unique occurrence of pernicious anemia-induced peripheral neuropathy in a patient with concurrent HLA-B27 arthropathy, highlighting the complex interplay of autoimmune mechanisms. While HLA-B27 is not typically associated with pernicious anemia, the case underscores the importance of exploring specific HLA haplotypes in understanding the nuanced manifestation of autoimmune disorders. Comprehensive screening for anti-intrinsic factor and anti-parietal cell antibodies is crucial in individuals with signs of pernicious anemia, especially those with a history of HLA-B27 arthropathy, guiding tailored management strategies. This report contributes to the ongoing exploration of the intricate autoimmune landscape in pernicious anemia.
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  • 文章类型: Case Reports
    本文介绍了一名22岁妇女的病例,该妇女多次住院,并伴有高血糖。她的精神病以幻听为特征,视觉幻觉,杂乱无章的言论和行为。患者既往有1型糖尿病(T1DM)和Graves病病史,且不坚持糖尿病饮食和药物治疗。病人是一名索马里难民,一年前移居美国。我们探讨了患者高血糖引起的精神病的相对独特的观察,特别是在自身免疫性疾病的背景下。我们还讨论了与难民社区心理健康和糖尿病管理的文化方面相关的一些复杂性及其在临床实践中的意义。
    This paper presents the case of a 22-year-old woman who was hospitalized multiple times with episodes of psychosis co-occurring with hyperglycemia. Her psychosis was characterized by auditory hallucinations, visual hallucinations, and disorganized speech and behavior. The patient has a prior medical history of type 1 diabetes mellitus (T1DM) and Graves\' disease and was non-adherent to diabetic diet and medications. The patient is a Somalian refugee who moved to the United States (US) a year ago. We explore the relatively unique observation of hyperglycemia-induced psychosis in the patient, specifically in the context of autoimmune disorders. We also discuss some of the complexities associated with the cultural aspects of mental health and diabetes management in refugee communities and their implications in clinical practice.
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  • 文章类型: Case Reports
    背景:BrentuximabVedotin(BV)彻底改变了霍奇金淋巴瘤的治疗前景,然而,它对先前存在的自身免疫性疾病的影响仍然难以捉摸。方法:这里,我们介绍了4例并发自身免疫性疾病的患者-克罗恩病,白癜风,I型糖尿病,和微小变化疾病-接受BV治疗霍奇金淋巴瘤。由于具有高IPI评分的晚期疾病,患者用A-AVD代替ABVD治疗。结果:我们的发现揭示了BV暴露与自身免疫表现之间令人惊讶和复杂的相互作用,强调在患者管理中需要多学科合作。值得注意的是,在T细胞介导的自身免疫占优势的前3例病例中观察到自身免疫症状加重.此外,BV暴露导致白癜风患者自身免疫性血小板减少症,强调了免疫调节的深刻破坏。相反,在微小变化的疾病案例中,一种以B细胞和T细胞介导的免疫混合为特征的疾病,结果是有利的。结论:本文强调了在并发自身免疫性疾病的患者中,对BV引起的自身免疫性发作保持警惕的重要性。为量身定制的患者护理提供见解。
    Background: Brentuximab Vedotin (BV) has revolutionized the treatment landscape for Hodgkin\'s lymphoma, yet its effects on pre-existing autoimmune disorders remain elusive. Methods: Here, we present four cases of patients with concurrent autoimmune conditions-Crohn\'s disease, vitiligo, type I diabetes, and minimal change disease-undergoing BV therapy for Hodgkin\'s lymphoma. The patients were treated with A-AVD instead of ABVD due to advanced-stage disease with high IPI scores. Results: Our findings reveal the surprising and complex interplay between BV exposure and autoimmune manifestations, highlighting the need for multidisciplinary collaboration in patient management. Notably, the exacerbation of autoimmune symptoms was observed in the first three cases where T-cell-mediated autoimmunity predominated. Additionally, BV exposure precipitated autoimmune thrombocytopenia in the vitiligo patient, underscoring the profound disruptions in immune regulation. Conversely, in the minimal change disease case, a disease characterized by a blend of B- and T-cell-mediated immunity, the outcome was favorable. Conclusions: This paper underscores the critical importance of vigilance toward autoimmune flare-ups induced by BV in patients with concurrent autoimmune conditions, offering insights for tailored patient care.
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  • 文章类型: Case Reports
    非狼疮全屋肾病是一种罕见的实体,仍然知之甚少。它可以使移植后的肾脏复杂化并导致从头过程。治疗是困难的,但可以通过优化免疫抑制来实现。
    非狼疮性全屋肾病是一种罕见的实体,发病率不清楚。它描述了IgG阳性沉积物的肾活检结果,IgA,IgM,C3和C1q在免疫荧光上缺乏系统性狼疮性肾炎的经典诊断特征。这种疾病实体越来越被认可,但仍需要进一步的研究来评估发病率。病因,和管理这种情况。移植肾小球病是肾移植物丢失的主要原因。它可以表现出各种各样的表现;它可以引起AKI,CKD,或通过免疫复合物或自身免疫介导的损伤引起的肾小球炎症。
    UNASSIGNED: Non-lupus full house nephropathy is a rare entity that is still poorly understood. It can complicate post-transplant kidneys and result in a de novo process. Treatment is difficult but can be possibly achieved with optimization of immune suppression.
    UNASSIGNED: Non-lupus full house nephropathy is a rare entity with an unclear incidence. It describes the kidney biopsy findings of positive deposits for IgG, IgA, IgM, C3, and C1q on immunofluorescence in the absence of the classical diagnostic features of systemic lupus nephritis. This disease entity is becoming more recognized but further studies are still needed to evaluate the incidence, etiologies, and management of this condition. Transplant glomerulopathy is a major cause for renal graft loss. It can present with a wide variety of manifestations; it can cause AKI, CKD, or glomerular inflammations through an immune complex or autoimmune-mediated damage.
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  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)主要影响年轻女性,并与血栓形成风险增加有关。抗磷脂抗体综合征(APS)可能会使临床表现复杂化,常导致动静脉血栓复发。此病例报告强调了两个独特方面的重要性:罕见的心房血栓发生和抗核抗体(ANA)阴性SLE的存在。
    一名32岁女性,有对称性多关节疼痛史,口腔溃疡,显著的体重减轻,无缘无故的pop血栓形成和两次原因不明的流产史。入院前一周,她经历了严重的头痛和血压升高。临床评估显示几个异常,包括收缩期杂音,网状livedo,经胸超声心动图显示严重的二尖瓣反流和心房血栓。经食道超声心动图证实右心房存在带蒂病变,具有挑战性的鉴别诊断。
    ANA阴性SLE,虽然罕见,在这个病人身上观察到,突出诊断的复杂性。APS加重了临床表现,强调识别特异性自身抗体和复发性血栓事件的重要性。在心房血栓的情况下,与其他心脏病的区别,如粘液瘤或植被,是一把钥匙。
    此案例强调了识别和管理心房血栓的重要性,系统性红斑狼疮和抗磷脂综合征患者的一种罕见但危及生命的并发症。此外,ANA阴性SLE的诊断挑战值得在具有疾病特征性特征的患者中仔细考虑.
    UNASSIGNED: Systemic lupus erythematosus (SLE) predominantly affects young women and is associated with an increased risk of thrombosis. Antiphospholipid antibody syndrome (APS) may complicate the clinical picture, often leading to recurrent arteriovenous thrombosis. This case report underscores the significance of two unique aspects: the rare occurrence of an atrial thrombus and the presence of antinuclear antibody (ANA)-negative SLE.
    UNASSIGNED: A 32-year-old woman presented with a history of symmetric polyarticular joint pain, oral ulcers, significant weight loss, and a history of unprovoked popliteal thrombosis and two unexplained abortions. One week prior to admission, she experienced severe headaches and elevated blood pressure. Clinical evaluation revealed several abnormalities, including a systolic murmur, livedo reticularis, and a transthoracic echocardiogram showing severe mitral regurgitation and an atrial thrombus. A transesophageal echocardiogram confirmed the presence of a pedunculated lesion in the right atria, challenging differential diagnosis.
    UNASSIGNED: ANA-negative SLE, though rare, was observed in this patient, highlighting diagnostic complexities. APS compounded the clinical presentation, emphasizing the importance of identifying specific autoantibodies and recurrent thrombotic events. In the case of atrial thrombus, differentiation from other cardiac conditions, such as myxoma or vegetation, is a key.
    UNASSIGNED: This case underscores the critical importance of recognizing and managing atrial thrombus, a rare but life-threatening complication in patients with systemic lupus erythematosus and antiphospholipid syndrome. Additionally, the diagnostic challenge of ANA-negative SLE warrants careful consideration in patients presenting with characteristic features of the disease.
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  • 文章类型: Case Reports
    视神经脊髓炎谱系疾病(NMOSD)是一种罕见的中枢神经系统自身免疫性疾病,以视神经炎为特征,脊髓炎,或者脑部损伤.其症状与多发性硬化症(MS)的症状重叠,对NMOSD的诊断具有挑战性。这里,我们报告了一例罕见的COVID-19疫苗接种后NMOSD引起的完全性脊髓损伤。一名52岁的女性患者在接受第三剂辉瑞-BioNTechCOVID-19疫苗(BNT162b2)后出现了NMOSD诱导的完全脊髓损伤。尽管最初诊断为完全性脊髓损伤,病人接受了强化治疗,包括利妥昔单抗治疗和康复。因此,她完全康复,并从ASIA减值量表(AIS)-A过渡到AIS-E。从完全脊髓损伤到功能独立的显着神经功能恢复凸显了综合治疗方法的有效性。此外,该病例强调需要认识到NMOSD是COVID-19疫苗接种的潜在不利结果,并强调了早期诊断的重要性,及时干预,和彻底的康复,以优化患者的结果。需要进一步的病例报告和研究来调查COVID-19疫苗接种与NMOSD发生之间的关系。
    Neuromyelitis optica spectrum disease (NMOSD) is a rare autoimmune disorder of the central nervous system characterized by optic neuritis, myelitis, or brain lesions. Its symptoms overlap with those of multiple sclerosis (MS), making a diagnosis of NMOSD challenging. Here, we report a rare case of NMOSD-induced complete spinal cord injury following COVID-19 vaccination. A 52-year-old female patient developed NMOSD-induced complete spinal cord injury after receiving their third dose of the Pfizer-BioNTech COVID-19 vaccine (BNT162b2). Despite the initial diagnosis of complete spinal cord injury, the patient underwent intensive treatment, including rituximab therapy and rehabilitation. As a result, she made a full recovery and transitioned from the ASIA Impairment Scale(AIS)-A to AIS-E. The remarkable neurological recovery from complete spinal cord injury to functional independence highlights the efficacy of a comprehensive treatment approach. In addition, this case emphasizes the need to recognize NMOSD as a potential adverse outcome of COVID-19 vaccination and emphasizes the importance of early diagnosis, timely intervention, and thorough rehabilitation for optimizing patient results. Further case reports and studies are needed to investigate the association between COVID-19 vaccination and the occurrence of NMOSD.
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  • 文章类型: Case Reports
    Lambert-Eaton肌无力综合征(LEMS)是一种罕见的自身免疫性疾病,其特征是针对突触前神经肌肉接头的自身抗体。它导致肌肉无力和自主神经功能障碍。LEMS可以是特发性的或与肿瘤疾病相关的,通常是小细胞肺癌。此病例报告描述了一名患有非霍奇金淋巴瘤的男性中罕见的副肿瘤LEMS。
    一名57岁的非霍奇金淋巴瘤男性,表现为进行性肌无力,反射减弱,和自主神经症状。诊断显示LEMS具有针对电压门控钙通道的自身抗体。免疫抑制治疗和淋巴瘤治疗导致他的病情显着改善。
    该病例突出了非霍奇金淋巴瘤患者中罕见的副肿瘤LEMS的发生。识别和及时管理LEMS以及淋巴瘤治疗可以导致显著的临床改善,强调需要提高对这种复杂关联的认识。
    UNASSIGNED: Lambert-Eaton myasthenia syndrome (LEMS) is a rare autoimmune disorder characterized by autoantibodies targeting presynaptic neuromuscular junctions. It results in muscle weakness and autonomic dysfunction. LEMS can be idiopathic or associated with neoplastic diseases, often small-cell lung cancer. This case report describes a rare instance of paraneoplastic LEMS in a man with non-Hodgkin lymphoma.
    UNASSIGNED: A 57-year-old male with non-Hodgkin lymphoma presented with progressive muscle weakness, diminished reflexes, and autonomic symptoms. Diagnosis revealed LEMS with autoantibodies against voltage-gated calcium channels. Immunosuppressive therapy and lymphoma treatment led to significant improvement in his condition.
    UNASSIGNED: This case highlights the rare occurrence of paraneoplastic LEMS in a patient with non-Hodgkin lymphoma. Recognition and timely management of LEMS alongside lymphoma treatment can lead to significant clinical improvement, emphasizing the need for increased awareness of such complex associations.
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  • 文章类型: Case Reports
    非霍奇金淋巴瘤是由淋巴细胞引起的各种淋巴增殖性疾病,具有广泛的组织学特征和临床表现,通常使准确诊断复杂化。在淋巴增殖性疾病中已观察到较高频率的自身抗体,然而,免疫系统的确切作用和潜在的致病因素仍然是个谜。间变性大细胞淋巴瘤(ALCL),侵袭性非霍奇金淋巴瘤变种,通常以类似于其他侵袭性淋巴瘤的方式出现,周围和/或腹膜后腺病迅速进展,伴有全身症状,如发烧,盗汗,和减肥。此病例报告深入研究了经过组织学验证的ALCL实例,惊人地模仿系统性红斑狼疮。本报告的目的是强调淋巴瘤可以表现出与自身免疫相关的临床或生物学特征。
    Non-Hodgkin lymphomas are a diverse group of lymphoproliferative disorders rising from the lymphocytes with a broad spectrum of histological characteristics and clinical manifestations that often complicate accurate diagnosis. Autoantibodies have been observed at higher frequencies in lymphoproliferative diseases, yet the precise role of the immune system and the underlying causative factors remain enigmatic. Anaplastic large cell lymphoma (ALCL), an aggressive non-Hodgkin\'s lymphoma variant, commonly presents in a manner akin to other aggressive lymphomas, featuring swift progression of peripheral and/or retroperitoneal adenopathy, accompanied by systemic symptoms like fever, night sweats, and weight loss. This case report delves into a histologically verified instance of ALCL, which strikingly emulates systemic lupus erythematosus. This report\'s objective is to underscore the concept that lymphoma can manifest clinical or biological features reminiscent of autoimmunity.
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