vasculitis

血管炎
  • 文章类型: Journal Article
    尽管根据病毒细胞侵袭途径的特异性,已经假设内皮损伤与2019年冠状病毒病(COVID-19)相关的脑梗死有关,迄今尚无病例报告。我们在此报告了一名51岁的日本女性,她在感染COVID-19后一周出现颈部疼痛。计算机断层扫描和磁共振成像显示颈动脉和椎动脉发炎。超声检查显示多个皮瓣样结构,被认为是血栓。虽然病人没有脑梗塞,这可能是COVID-19患者血管损伤和血栓形成的重要病例.
    Although endothelial damage has been hypothesized to be associated with coronavirus disease 2019 (COVID-19)-related cerebral infarction based on the specificity of the viral cellular invasion pathway, no case has been reported to date. We herein report a 51-year-old Japanese woman who presented with neck pain one week after COVID-19 infection. Computed tomography and magnetic resonance imaging revealed inflammation of the carotid and vertebral arteries. Ultrasonography revealed multiple flap-like structures that were assumed to be thrombi. Although the patient had no cerebral infarction, this could be an important case of vascular damage and thrombus formation in a COVID-19 patient.
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  • 文章类型: Case Reports
    神经-Behçet病(NBD)是Behçet综合征的重要并发症,可能导致死亡率和残疾率上升。实质性NBD的标准治疗通常需要给予高剂量皮质类固醇以促进快速起效。加上免疫抑制剂,以防止随后的复发。一名患有NBD的48岁男性在9个月内表现出逐渐恶化的构音障碍。该患者在使用糖皮质激素时出现眼内压升高,恶化了他先前的青光眼.该患者先前诊断为NBD,并在9个月内出现进行性构音障碍。导致脑部磁共振成像(MRI)扫描。脑部MRI显示左额顶区域多灶性点状高信号强度,脑岛,和基底神经节.而不是标准的类固醇脉冲疗法,患者接受阿达木单抗-环磷酰胺联合治疗作为替代诱导治疗.随后的系列脑部MRI扫描显示没有出现新的病变,即使在开始诱导治疗后17个月,患者仍然没有临床复发。阿达木单抗-环磷酰胺组合可用作NBD的无皮质类固醇诱导策略。需要进一步的研究以建立最合适的联合方案。
    Neuro-Behçet\'s disease (NBD) represents a significant complication of Behçet\'s syndrome, potentially leading to elevated mortality and disability rates. The standard treatment for parenchymal NBD typically entails administering high-dose corticosteroids to prompt rapid-onset effects, coupled with immunosuppressants to prevent subsequent relapses. A 48-year-old male with NBD presented with progressively worsening dysarthria over 9 months. This patient experienced increased intraocular pressure while using glucocorticoids, which worsened his pre-existing glaucoma. The patient had a prior diagnosis of NBD and presented with progressive dysarthria over a period of nine months, leading to a brain magnetic resonance imaging (MRI) scan. The brain MRI revealed multifocal punctate high signal intensities in the left frontoparietal area, insula, and basal ganglia. Instead of the standard steroid pulse therapy, the patient received adalimumab-cyclophosphamide combination as an alternative induction therapy. Subsequent serial brain MRI scans exhibited no emergence of new lesions, and the patient remained devoid of clinical relapses even after 17 months from the commencement of induction treatment. Adalimumab-cyclophosphamide combination could be used as a corticosteroid-free induction strategy for NBD. Further investigations are warranted to establish the most suitable combination regimen.
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  • 文章类型: Case Reports
    IgA肾病(IgAN)与酒精性肝病相当常见。然而,IgA血管炎(IgAV)与酒精性肝硬化的关系并不常见,文献中仅报道了少数病例。继发性IgAN通常以温顺的方式出现,在大约5-25年内进展缓慢。它通常对类固醇治疗有反应,很少进展为终末期肾病。这里,我们介绍一个50多岁的男人,一种已知的高血压和酒精相关的肝硬化,他因皮疹和快速进行性肾功能衰竭(RPRF)就诊于我们医院。他被诊断为IgA肾炎伴IgA血管炎(IgAVN)。皮肤和肾活检证实了他的诊断。他因肾功能衰竭开始接受肾脏替代治疗,并开始口服类固醇治疗。给予类固醇治疗6个月后,患者康复,且独立于透析,肾脏参数稳定.
    IgA nephropathy (IgAN) is a fairly common association with alcoholic liver disease. However, IgA vasculitis (IgAV) is quite an uncommon association with alcoholic liver cirrhosis and only a handful of cases have been reported in literature. Secondary IgAN usually presents in a docile manner, progressing slowly in about 5-25 years. It is usually responsive to steroid therapy, very rarely progressing to End-Stage Renal Disease. Here, we present a man in his late 50s, a known hypertensive and alcohol related liver-cirrhotic, who presented to our hospital with rash and rapidly progressive renal failure (RPRF). He was diagnosed with IgA nephritis with IgA vasculitis (IgAVN). His diagnosis was confirmed with skin and renal biopsy. He was started on renal replacement therapy for his renal failure and began oral steroid therapy. After administration of steroid therapy for 6 months, the patient recovered and was dialysis independent with stable renal parameters.
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  • 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,身体对自身抗原失去耐受性,特别是核抗原。T细胞和B细胞的异常反应导致自身抗体的产生和组织中免疫复合物的形成,触发补体激活,炎症,和不可逆的器官损伤。SLE可以影响身体的任何部位,导致不同的临床症状。SLE的一种罕见表现是狼疮肠系膜血管炎(LMV),表现出模糊的症状,异常的实验室发现,和特定的成像特征。LMV,虽然不常见,可以发展为严重的并发症,如肠穿孔,出血,甚至死亡率。这里,我们报告了一例累及多器官系统的LMV(包括皮肤粘膜,肌肉骨骼,浆膜腔,和血液系统),最初出现危及生命的顽固性胃肠道出血,并发严重的肺部感染.通过分享这个案例,我们的目标是增强临床医生对严重SLE病例的管理信心,并提高对疾病监测的认识.
    Systemic lupus erythematosus (SLE) is a complex autoimmune disease where the body loses tolerance to its own antigens, particularly nuclear antigens. Abnormal responses from T and B cells lead to the production of autoantibodies and the formation of immune complexes in tissues, triggering complement activation, inflammation, and irreversible organ damage. SLE can affect any part of the body, resulting in diverse clinical symptoms. One rare manifestation of SLE is lupus mesenteric vasculitis (LMV), which presents with vague symptoms, abnormal laboratory findings, and specific imaging features. LMV, although uncommon, can progress to severe complications such as bowel perforation, haemorrhage, and even mortality. Here, we report a case of LMV with the involvement of multiple organ systems (including mucocutaneous, musculoskeletal, serosal cavities, and haematological systems), presenting initially with life-threatening intractable gastrointestinal bleeding, and complicated by severe pulmonary infection. By sharing this case, we aim to enhance clinicians\' confidence in managing critical SLE cases and raise awareness about disease surveillance.
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  • 文章类型: Case Reports
    VEXAS综合征是UBA1基因突变继发的罕见实体,位于X染色体上。这种突变产生,因此,造血干细胞上的特征性空泡。它的特点是多种自身炎症和血液学表现,有反应并最终依赖皮质类固醇治疗。在本出版物中,我们介绍了在我们医院诊断的2例病例系列,并对迄今为止已发表的证据进行了简短的文献回顾。
    VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far.
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  • 文章类型: Case Reports
    此病例报告详细介绍了一名88岁男子的诊断挑战和管理,该男子因败血症样症状出现在日本农村社区医院,根据他的物理和实验室检查结果,最初怀疑是急性细菌性胆管炎。尽管他唑巴坦和哌拉西林的抗生素治疗,病人的症状持续存在,导致进一步的调查显示,没有感染的迹象,但在对比增强的计算机断层扫描中,主动脉弓壁明显增厚。这些发现,结合患者的临床表现和缺乏抗生素反应,将诊断重定向到巨细胞动脉炎(GCA)。每天60mg泼尼松龙的给药可显著缓解症状并防止潜在的严重并发症,例如失明和不可逆的神经损伤。该病例强调了在出现全身炎症症状的老年患者中考虑GCA的重要性以及及时干预的必要性。它还强调了在老年患者中管理高剂量类固醇治疗的挑战,并提出了整合免疫抑制剂以减少类固醇依赖的潜在益处。本报告强调需要在GCA的非典型表现中提高意识和全面的诊断方法,特别是在资源有限的医疗保健环境中的老年人群中。
    This case report details the diagnostic challenge and management of an 88-year-old man who presented to a rural Japanese community hospital with sepsis-like symptoms, initially suspected of acute bacterial cholangitis based on his physical and laboratory findings. Despite the antibiotic treatment of tazobactam and piperacillin, the patient\'s symptoms persisted, leading to further investigations that revealed no signs of infection but notable aortic arch wall thickening on contrast-enhanced computed tomography scans. These findings, combined with the patient\'s clinical presentation and lack of antibiotic response, redirected the diagnosis toward giant cell arteritis (GCA). The administration of prednisolone of 60 mg daily significantly alleviated symptoms and prevented potential severe complications such as blindness and irreversible neurological damage. This case underscores the importance of considering GCA in elderly patients presenting with systemic inflammatory symptoms and the necessity of timely intervention. It also highlights the challenges in managing high-dose steroid therapy in elderly patients and suggests the potential benefits of integrating immunosuppressants to reduce steroid dependency. This report emphasizes the need for heightened awareness and a comprehensive diagnostic approach in atypical presentations of GCA, particularly in geriatric populations within resource-limited healthcare settings.
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  • 文章类型: Case Reports
    机械血栓切除术(MT)对急性缺血性卒中的疗效已经确立,但很少有关于MT对患有胶原蛋白病的中风患者的有效性的报道。我们报告了患有脑梗死的系统性红斑狼疮(SLE)患者接受MT的病例。一名48岁的妇女被诊断患有SLE30年。她因为前一天的头晕而去了我们医院,但当她到达医院停车场时,她出现了呕吐和意识障碍。MRI显示小脑半球梗塞增加,磁共振血管造影(MRA)未显示右椎动脉或基底动脉。紧急做了脑血管造影,右椎动脉造影显示椎动脉V4段闭塞。除了这些血管造影结果,患者的意识也受损,被认为需要紧急血运重建治疗。我们使用支架取出器进行了MT。血管造影检查后,发现基底动脉再灌注和右椎动脉严重狭窄。因此,经皮腔内血管成形术(PTA)和支架置入术治疗椎动脉狭窄。该程序成功地维持了椎动脉的通畅和流向基底动脉的血液。她的意识得到了改善;她只有轻度恶心,没有明显的神经系统发现。
    The efficacy of mechanical thrombectomy (MT) for acute ischemic stroke has been established, but there are few reports on the effectiveness of MT for stroke patients with collagen disease. We report the case of a systemic lupus erythematosus (SLE) patient with cerebral infarction who underwent MT. A 48-year-old woman had been diagnosed with SLE for 30 years. She visited our hospital because of dizziness from the day before, but when she arrived at the hospital parking lot, she developed vomiting and impaired consciousness. An MRI revealed increased cerebellar hemisphere infarction and magnetic resonance angiography (MRA) did not visualize the right vertebral artery or basilar artery. Urgent cerebral angiography was performed, and angiography of the right vertebral artery revealed occlusion of the V4 segment of the vertebral artery. In addition to these angiographic findings, the patient also had impaired consciousness and was judged to be in need of emergency revascularization treatment. We performed an MT using a stent retriever. Immediately after the angiography examination, reperfusion to the basilar artery and severe stenosis of the right vertebral artery were noted. Therefore, percutaneous transluminal angioplasty (PTA) and stent placement for vertebral artery stenosis were done. This procedure successfully maintained the patency of the vertebral artery and blood flow to the basilar artery. Her consciousness improved; she only had mild nausea and no remarkable neurological findings.
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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
    Takayasu动脉炎是一种病因不明的炎症性疾病,会影响大血管。中等船只的参与也有很好的记录;然而,作为表现的神经病是罕见的。在这个案例报告中,一名20多岁的年轻女性,有8个月的右上肢间歇性跛行病史,进展为静息疼痛,C5-C8分布异常疼痛,无痛性右腋窝肿块。在检查中,她的右桡动脉没有脉搏,右锁骨下动脉和腹主动脉有杂音。CT血管造影显示有提示Takayasu动脉炎的特征,右腋窝动脉引起部分血栓形成的动脉瘤,导致右臂丛神经受压。该患者接受甲氨蝶呤和口服皮质类固醇治疗。随访3个月时,动脉瘤的大小缩小了,压缩症状的解决和炎症标志物的正常化。
    Takayasu arteritis is an inflammatory disease of unknown aetiology affecting large vessels. Medium vessel involvement is also well documented; however, neuropathy as a presenting manifestation is rare. In this case report, a young woman in her 20s presented with an 8-month history of intermittent claudication in the right upper limb progressing to rest pain with allodynia in C5-C8 distribution and painless right axillary mass. On examination, she had absent pulses in the right radial, brachial and subclavian artery with audible bruit in the right subclavian and abdominal aorta. CT angiogram showed features suggestive of Takayasu arteritis with a partially thrombosed aneurysm arising from the right axillary artery leading to compression of the right brachial plexus. This patient received treatment with methotrexate and oral corticosteroids. At 3 months follow-up, there was a reduction in the size of the aneurysm, resolution of compressive symptoms and normalisation of inflammatory markers.
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  • 文章类型: Case Reports
    一个75岁的女人,有高血压和心房颤动,但没有既往肾脏病史,因胸部不适和呼吸困难就诊。她被发现患有急性肾衰竭,血清肌酐为5.1,从基线的0.9增加,尿液分析显示蛋白尿和血尿伴有异形红细胞。随后的检查对于核周抗中性粒细胞胞浆抗体(p-ANCA)和髓过氧化物酶抗体阳性具有重要意义。她做了肾活检,这显示了14个肾小球中有12个坏死性新月,由于显微镜下多血管炎,她被诊断为快速进展性肾小球肾炎。尽管使用血浆置换进行了积极的治疗,大剂量泼尼松,利妥昔单抗输注,肾功能恶化,她需要开始血液透析.她入院三周后最终出院,计划在门诊继续进行利妥昔单抗输注和每周三次血液透析。由于她对传统疗法的反应不佳,一种名为avacopan的新靶向免疫调节剂的启动,补体5a受体拮抗剂,被考虑。这种靶向免疫调节剂作为降低与当前广泛免疫抑制方式相关的严重感染风险的可能方式也是特别感兴趣的。此外,当用于代替类固醇时,它们降低了与累积糖皮质激素毒性相关的发病率.对于标准疗法难以治疗的ANCA相关性血管炎患者,靶向免疫调节剂,如阿瓦科潘,应考虑作为替代或辅助治疗。
    A 75-year-old woman, with hypertension and atrial fibrillation but no prior renal history, presented to the hospital for chest discomfort and dyspnea. She was found to be in acute renal failure, with a serum creatinine of 5.1, increased from a baseline of 0.9, and urine analysis revealing proteinuria and hematuria with dysmorphic red blood cells. Subsequent work up was significant for positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA) and myeloperoxidase antibodies. She underwent a renal biopsy, which revealed necrotizing crescents in 12 of 14 glomeruli, and she was diagnosed with rapidly progressive glomerulonephritis due to microscopic polyangiitis. Despite aggressive treatment with plasmapheresis, high-dose prednisone, and rituximab infusions, renal function worsened, and she required initiation of hemodialysis. She was ultimately discharged after a three-week admission, with plans to continue rituximab infusions and three times weekly hemodialysis in the outpatient setting. Due to her poor response to traditional therapies, initiation of a new targeted immunomodulator known as avacopan, a complement 5a receptor antagonist, was considered. Such targeted immunomodulators are also of particular interest as possible ways to reduce the risk of severe infection associated with current broad immunosuppressive modalities. In addition, when used in place of steroids, they reduce the morbidity associated with cumulative glucocorticoid toxicity. For patients with ANCA-associated vasculitis refractory to standard therapies, targeted immunomodulators such as avacopan should be considered as alternative or adjunct therapy.
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