small-vessel vasculitis

  • 文章类型: Case Reports
    白细胞碎裂性血管炎(LCV)是一种小血管血管炎,其特征是炎症和对小血管壁的损害。它通常表现为可触及的紫癜,并可能与各种全身状况有关。虽然其病因多样,LCV与全身性疾病相关,感染,药物,和自身免疫性疾病。这里,我们介绍了1例失代偿性心力衰竭患者的LCV病例.一名58岁男子的下肢和阴囊肿胀逐渐恶化,持续的干咳与胫骨上的轻微溃疡性病变有关,和斑片状皮疹,有脓疱和扁平的红色斑点。他三天前因心房颤动和快速心室率住院,他开始服用胺碘酮。这种皮疹持续了三天,然而他否认有任何不适或瘙痒。根据他的临床表现,实验室评估,和影像学发现,他被诊断为胺碘酮诱导的LCV。
    Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis characterized by inflammation and damage to the walls of small blood vessels. It typically presents with palpable purpura and can be associated with various systemic conditions. Although its etiology is diverse, LCV has been associated with systemic diseases, infections, medications, and autoimmune disorders. Here, we present a case of LCV in a patient with decompensated heart failure. A 58-year-old man presented with progressively deteriorating swelling in both his lower limbs and scrotum, a persistent dry cough associated with minor ulcerative lesions on his shins, and a patchy rash with pustules and flat reddish spots. He was hospitalized three days prior due to atrial fibrillation and rapid ventricular rate, for which he was commenced on amiodarone. This rash persisted for three days, yet he denied experiencing any discomfort or itchiness along with it. Based on his clinical picture, laboratory evaluations, and imaging findings, he was diagnosed with LCV induced by amiodarone.
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  • 文章类型: Journal Article
    神经影像学在识别小血管血管炎与它所模仿的常见疾病中具有关键作用,如多发性硬化症。通常,许多这些条件同样存在,因此诊断是困难的。迄今为止,没有标准化的方法来区分这些疾病。这篇综述确定并介绍了现有的评分工具,这些工具可以作为将人工智能/机器学习(AI/ML)整合到这些罕见疾病的临床决策过程中的起点。EMBASE和MEDLINE的范围研究文献综述包括114篇文章,以评估诊断小血管血管炎和常见模拟物的标准。本文介绍了小血管血管炎的现有标准,并对其进行了模拟,以指导AI/ML算法的未来整合,以帮助诊断这些疾病。它们以类似和非特定的方式存在。
    Neuroimaging has a key role in identifying small-vessel vasculitis from common diseases it mimics, such as multiple sclerosis. Oftentimes, a multitude of these conditions present similarly, and thus diagnosis is difficult. To date, there is no standardized method to differentiate between these diseases. This review identifies and presents existing scoring tools that could serve as a starting point for integrating artificial intelligence/machine learning (AI/ML) into the clinical decision-making process for these rare diseases. A scoping literature review of EMBASE and MEDLINE included 114 articles to evaluate what criteria exist to diagnose small-vessel vasculitis and common mimics. This paper presents the existing criteria of small-vessel vasculitis conditions and mimics them to guide the future integration of AI/ML algorithms to aid in diagnosing these conditions, which present similarly and non-specifically.
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  • 文章类型: Systematic Review
    非感染性冷球蛋白性血管炎(NICV)的数据很少,特别是关于复发的管理,这很麻烦。我们旨在调查NICV复发的危险因素。
    CINAHL系统文献检索,Embase,MEDLINE,Scopus,WebofScience数据库一直实施到2023年4月。符合条件的研究包括随机对照试验,观察性研究,以及≥4名患者的病例系列。两名评审员独立提取数据并评估合格研究的质量。
    从数据库搜索中总共检索到3,724篇文章,27项研究符合纳入标准进行审查。大多数研究(n=23)详细的复发,复发时间在1到80个月之间。据报道,I型NICV的复发率为28%,混合NICV的复发率为22%至60%。根据冷球蛋白亚型确定了NICV复发的危险因素,并与不同治疗方案的临床和免疫反应相关。具有相关淋巴增生性疾病的I型NICV表现出反应复发模式。皮肤和关节受累以及对治疗的临床和免疫反应不完全,特别是皮质类固醇单一疗法和偶尔利妥昔单抗,影响II型和III型NICV的复发风险。
    我们的研究结果强调了在NICV中获得临床和免疫反应以及确定复发危险因素的重要性。对NICV患者进行适当的风险分层对于成功实施有效的治疗策略至关重要。
    https://www.crd.约克。AC.英国/普华永道/,标识符CRD42023408140。
    UNASSIGNED: Data on non-infectious cryoglobulinemic vasculitis (NICV) is scarce, especially concerning the management of relapses, which are troublesome. We aimed to investigate risk factors for relapse in NICV.
    UNASSIGNED: A systematic literature search of CINAHL, Embase, MEDLINE, Scopus, and the Web of Science databases was implemented until April 2023. Eligible studies included randomized control trials, observational studies, and case series with ≥4 patients. Two reviewers independently extracted data and assessed the quality of the eligible studies.
    UNASSIGNED: A total of 3,724 articles were retrieved from a database search, with 27 studies meeting the inclusion criteria for review. Most studies (n = 23) detailed relapses, with the time to relapse varying between 1 and 80 months. The relapse rate was reported at 28% in Type I NICV and ranged from 22% to 60% in mixed NICV. Risk factors for relapse in NICV were identified based on the cryoglobulin subtype and correlated with clinical and immunological responses to varying treatment regimens. Type I NICV with an associated lymphoproliferative disorder exhibited a response-relapse pattern. Cutaneous and articular involvement and incomplete clinical and immunological responses to treatment, particularly corticosteroid monotherapy and occasionally rituximab, influence the risk of relapse in Type II and Type III NICV.
    UNASSIGNED: Our findings underscore the significance of attaining both clinical and immunological responses and identifying risk factors for relapse in NICV. Appropriate risk stratification for NICV patients is essential for the successful implementation of effective treatment strategies.
    UNASSIGNED: https://www.crd.york.ac.uk/prospero/, identifier CRD42023408140.
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  • 文章类型: Case Reports
    肉芽肿性多血管炎(GPA)是一种病因不明的自身免疫性肉芽肿性疾病;通常与抗蛋白酶3抗中性粒细胞胞浆抗体(PR3-ANCA)相关。虽然任何器官都可以参与,前列腺参与GPA非常罕见。我们提出一名男性患者的GPA,26岁,进行详尽评估的肺部表现和前列腺受累。患者的实验室检查和影像学扫描显示多个区域有病变的证据,包括前列腺.组织病理学检查证实,病变与肉芽肿性多血管炎一致。患者接受口服类固醇和利妥昔单抗治疗,并表现出显着改善。后来他继续服用硫唑嘌呤,没有任何复发。
    Granulomatosis with polyangiitis (GPA) is an autoimmune granulomatous disease of unknown etiology; frequently associated with anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA). Although any organ can be involved, prostatic involvement in GPA is very rare. We present a male patient with GPA, 26 years old, with pulmonary manifestations and prostatic involvement who underwent exhaustive evaluation. The patient\'s laboratory tests and imaging scans showed evidence of lesions in multiple areas, including the prostate. Histopathological testing confirmed that the lesions were consistent with granulomatosis with polyangiitis. The patient was treated with oral steroids and rituximab and showed significant improvement. He was later maintained on azathioprine without any relapse.
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  • 文章类型: Case Reports
    一名65岁的男子下肢出现明显的紫癜性丘疹和斑块,在急诊室服用头孢氨苄和强力霉素后,迅速扩散到他的躯干和上肢。这里,我们定义了类似教科书的IgA血管炎的细节,以前被称为过敏性紫癜,在一个成年人。
    A 65-year-old man developed palpable purpuric papules and plaques on his lower extremities, which quickly spread to his trunk and upper extremity after being prescribed cephalexin and doxycycline in the emergency room. Here, we define the details of a textbook-like presentation of IgA vasculitis, formerly referred to as Henoch-Schönlein purpura, in an adult.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    我们先前报道了IgA血管炎和皮肤动脉炎可能与抗磷脂酰丝氨酸/凝血酶原复合物(抗PS/PT)抗体和溶酶体相关膜蛋白2(LAMP-2)的存在相关。皮下组蛋白注射后静脉内给予抗PS/PT抗体诱导的皮肤血管炎大鼠皮肤组织样本中LAMP-2mRNA的拷贝数明显高于无皮肤血管炎大鼠。我们发现,在组蛋白引发皮肤后,静脉内给予抗PS/PT抗体诱导的皮肤血管炎大鼠中,受影响血管的中性粒细胞和血管内皮细胞中LAMP-2蛋白过表达。我们在组蛋白皮肤引发后静脉注射抗PS/PT抗体和抗LAMP-2抗体的大鼠皮肤中发现了典型的皮肤小血管血管炎。我们建议在血清中引入皮肤局部组蛋白和抗PS/PT抗体可以将LAMP-2移动到中性粒细胞和血管内皮细胞的细胞表面,在典型的皮肤小血管血管炎中,抗LAMP-2抗体可以通过抗原特异性结合来桥接这些细胞。
    We previously reported that IgA vasculitis and cutaneous arteritis could be dependently associated with the presence of the antiphosphatidylserine/prothrombin complex (anti-PS/PT) antibody and lysosomal-associated membrane protein-2 (LAMP-2). The copy number of LAMP-2 mRNA in skin tissue samples from rats with cutaneous vasculitis induced by intravenous administration of anti-PS/PT antibody after subcutaneous histone injection was significantly higher than in those without cutaneous vasculitis. We found LAMP-2 protein overexpression in neutrophils and vascular endothelial cells of the affected blood vessels in rats with cutaneous vasculitis induced by intravenous administration of anti-PS/PT antibody after cutaneous priming by histones. We found typical cutaneous small-vessel vasculitis in the skin of rats given intravenous injection of both anti-PS/PT antibody and anti-LAMP-2 antibody after cutaneous priming by histones. We suggested that the introduction of skin local histones and anti-PS/PT antibody in serum could move LAMP-2 to the cell surface of neutrophils and vascular endothelial cells, and that anti-LAMP-2 antibody could bridge these cells through antigen-specific binding in typical cutaneous small-vessel vasculitis.
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  • 文章类型: Case Reports
    白细胞碎裂性血管炎(超敏反应性血管炎)被定义为由于感染引起的皮肤或其他全身表现的小血管炎症,毒品,或肿瘤性疾病。该临床病例报告强调了头孢曲松与白细胞碎裂性血管炎之间的关联,该患者49岁,有青霉素过敏史。米氮平治疗焦虑症.还综述了有关抗生素诱导的白细胞碎裂性血管炎的文章。患者在5天前报告了上呼吸道感染和发烧的症状,为此她接受了头孢曲松2天,然后到急诊科就诊,上肢和下肢出现瘙痒性皮疹,嘴唇肿胀1天。皮疹是红斑,斑丘疹,发痒,和非招标,没有粘膜受累.实验室检查显示白细胞增多(白细胞,22.3×109/L),主要为嗜酸性粒细胞(18.4%)。经验停止头孢曲松后,患者给予泼尼松龙和抗组胺药。皮肤活检证实了白细胞碎裂性血管炎的诊断。治疗开始后观察到显著的临床改善。在后续行动中,皮疹完全缓解,没有疤痕;然而,下肢有脱皮现象。识别抗生素诱导的白细胞碎裂性血管炎至关重要,因为许多类别的抗生素都可能导致这种情况。继续服用有问题的药物可能会导致危及生命的并发症。
    Leukocytoclastic vasculitis (hypersensitivity vasculitis) is defined as small blood vessel inflammation with skin or other systemic manifestations due to infections, drugs, or neoplastic disease. This clinical case report highlights an association between ceftriaxone and leukocytoclastic vasculitis in a 49-year-old female patient with a history of penicillin allergy, on mirtazapine for anxiety disorder. Articles concerning antibiotic-induced leukocytoclastic vasculitis are also reviewed. The patient reported a symptom of upper respiratory tract infection and fever 5 days previously for which she received ceftriaxone for 2 days before presenting to the emergency department with a pruritic skin rash in the upper and lower extremities and swollen lips for 1 day. The rash was erythematous, maculopapular, itchy, and non-tender, with no mucus membrane involvement. Laboratory investigations revealed leukocytosis (white blood cells, 22.3 × 109/L) that was mainly eosinophilic (18.4%). The patient was administered prednisolone and antihistamine after stopping ceftriaxone empirically. A skin biopsy confirmed the diagnosis of leukocytoclastic vasculitis. Significant clinical improvement was observed after treatment initiation. Upon follow-up, the skin rash was resolved entirely with no scars; however, there was skin-peeling over the lower extremities. Recognition of antibiotic-induced leukocytoclastic vasculitis is crucial as many classes of antibiotics can contribute to this condition. Continuation of the offending drug may lead to life-threatening complications.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    对南美国家系统性血管炎的流行病学知之甚少。这项研究的目的是比较巴西和秘鲁两个血管炎转诊中心的全身血管炎患病率。进行了一项横断面研究,所有18岁以上的患者,至少6个月的随访,谁符合最常见形式的血管炎的分类或诊断标准,包括在内。对562例系统性血管炎患者进行分析,345人(61.4%)来自巴西,217人(38.6%)来自秘鲁。Behçet病的发生频率(37.9%vs.1.8%;p<0.0001),大动脉炎(TAK)(25.2%vs.6.9%;p<0.0001),和巨细胞动脉炎(9.8%vs.0.9%;p<0.0001)巴西中部高于秘鲁中部。另一方面,显微镜下多血管炎(MPA)的发生率(67.3%vs.2.8%;p<0.0001)和肾限制性血管炎(2.8%vs.0.0%;p=0.009)在秘鲁中心较高。关于其他形式的血管炎没有发现差异。诊断时,巴西TAK患者,肉芽肿性多血管炎,MPA比秘鲁患者年轻。在巴西的血管炎转诊中心和秘鲁的血管炎转诊中心之间,观察到系统性血管炎发生频率的流行病学差异。要点•观察到巴西和秘鲁之间系统性血管炎的流行病学特征存在显著差异。•MPA是秘鲁血管炎的主要形式,而BD和TAK是巴西最常见的血管炎形式。•TAK的诊断年龄,MPA,巴西患者的GPA低于秘鲁患者。
    Little is known about the epidemiology of systemic vasculitis in South American countries. The aim of this study is to compare the prevalence of systemic vasculitides in two vasculitis referral centers from Brazil and Peru. A cross-sectional study was performed and all patients above 18 years of age, with at least 6 months of follow-up and who met classification or diagnosis criteria for the most common forms of vasculitis, were included. A total of 562 patients with systemic vasculitis were analyzed, 345 (61.4%) from Brazil and 217 (38.6%) from Peru. The frequency of Behçet\'s disease (37.9% vs. 1.8%; p < 0.0001), Takayasu arteritis (TAK) (25.2% vs. 6.9%; p < 0.0001), and giant cell arteritis (9.8% vs. 0.9%; p < 0.0001) was higher in the Brazilian center than the Peruvian one. On the other hand, the frequency of microscopic polyangiitis (MPA) (67.3% vs. 2.8%; p < 0.0001) and renal-limited vasculitis (2.8% vs. 0.0%; p = 0.009) was higher in the Peruvian center. No differences were found concerning other forms of vasculitis. At diagnosis, Brazilian patients with TAK, granulomatosis with polyangiitis, and MPA were younger than Peruvian patients. Epidemiologic differences in the frequency of systemic vasculitis are observed between a vasculitis referral center from Brazil and another from Peru. Key Points • Significant differences are observed regarding the epidemiologic profile of systemic vasculitis between Brazil and Peru. • MPA is the predominant form of vasculitis in Peru while BD and TAK are the most frequent forms of vasculitis in Brazil. • The age at diagnosis of TAK, MPA, and GPA was lower in Brazilian patients than in Peruvian patients.
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