polyarteritis nodosa

结节性多动脉炎
  • 文章类型: Journal Article
    结节性多动脉炎(PAN)是一种影响中型动脉的全身性风湿性疾病。PAN通常与抗嗜中性粒细胞胞浆抗体无关,并且没有血清学替代标志物。因此,它的诊断需要病理结果。然而,活检诊断PAN的阳性率不高,活检区域通常是有限的。一些研究人员报道了影像学发现在诊断PAN中的有用性,独立于病理结果。18F-氟代脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(FDG-PET)/CT最近在日本被批准用于诊断大血管血管炎。一些研究还证明了FDG-PET/CT在诊断中血管血管炎中的有用性。然而,与其他方式相比,没有研究评估FDG-PET/CT诊断PAN的有用性,目前尚不清楚FDG-PET/CT是否优于其他诊断PAN的方法。在这里,我们报告了1例PAN,并比较了FDG-PET/CT与其他诊断PAN的有效性.
    Polyarteritis nodosa (PAN) is a systemic rheumatic disease that affects medium-sized arteries. PAN is typically not associated with anti-neutrophil cytoplasmic antibodies and has no serological surrogate markers. Therefore, its diagnosis requires pathological findings. However, the positive rate of biopsy in diagnosing PAN is not high, and the biopsy area is often limited. Several investigators have reported the usefulness of imaging findings in diagnosing PAN, independent of pathological findings. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET)/CT has recently been approved for the diagnosis of large-vessel vasculitis in Japan. Several studies have also demonstrated the usefulness of FDG-PET/CT in diagnosing medium-vessel vasculitis. However, no studies have evaluated the usefulness of FDG-PET/CT for diagnosing PAN compared to other modalities, and it is not clear whether FDG-PET/CT is superior to other modalities for diagnosing PAN. Herein, we report a case of PAN and compare the usefulness of FDG-PET/CT with other modalities in diagnosing PAN.
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  • 文章类型: Case Reports
    在接受抗TNF-α药物治疗强直性脊柱炎的患者中,监测紫癜性和缺血性皮肤病变至关重要。此病例强调了识别和解决药物引起的血管炎的重要性,同时强调了迅速评估和探索替代治疗方案以保障患者健康的必要性。
    该病例讨论了一名38岁女性,有强直性脊柱炎(AS)病史,并伴有皮肤病变,包括紫癜性皮肤损伤和右脚手指缺血,在开始使用阿达木单抗治疗后。排除其他潜在原因后,如感染和恶性肿瘤,该患者接受了与使用阿达木单抗相关的中度血管血管炎的诊断.停用阿达木单抗和高剂量糖皮质激素和环磷酰胺静脉脉冲治疗导致她的缺血性病变消退。该病例强调了在新的皮肤病变患者中考虑药物相关副作用的重要性,特别是在风湿性疾病如AS的背景下。
    UNASSIGNED: In patients receiving anti-TNF-α drugs for ankylosing spondylitis, monitoring purpuric and ischemic skin lesions is crucial. This case underscores the significance of identifying and addressing drug-induced vasculitis while stressing the necessity for prompt evaluation and exploration of alternative treatment options to safeguard patient well-being.
    UNASSIGNED: The case discusses a 38-year-old female with a history of ankylosing spondylitis (AS) who presented with skin lesions, including purpuric skin lesions and ischemia of her right foot digits, after initiating treatment with adalimumab. After excluding other potential causes, such as infections and malignancies, the patient received a diagnosis of moderate-sized vascular vasculitis associated with adalimumab use. Discontinuation of adalimumab and treatment with high dose glucocorticoids and intravenous pulse of cyclophosphamide resulted in the resolution of her ischemic lesions. This case underscores the importance of considering drug-related side effects in patients with new skin lesions, particularly in the context of rheumatic diseases such as AS.
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  • 文章类型: Case Reports
    动脉瘤是导致病理性扩张的动脉壁的局部弱化。所有腹内动脉瘤都标记为内脏动脉瘤(VAA),除了主动脉到髂动脉的动脉瘤.VAA很罕见,胃十二指肠动脉瘤(GDAA),占内脏动脉瘤的1.5%。一个80多岁的女人出现了慢性上腹痛,减肥,和恶心。保守管理不成功。成像显示GDAA,提示血管内弹簧圈栓塞。随后的评估证实结节性多动脉炎(PAN),用利妥昔单抗治疗。该报告强调了诊断挑战,强调需要使用成像和血管造影的多学科方法。GDAA的潜在危及生命的破裂需要及时干预,正如在这种情况下所说明的那样。与PAN的罕见关联,虽然不常见,强调了在多发性内脏动脉瘤中考虑潜在病因的重要性。早期诊断和干预对于这种罕见但可能致命的疾病至关重要。
    An arterial aneurysm is a localized weakening of the artery wall that results in pathological dilatation. All intra-abdominal artery aneurysms are labeled as visceral artery aneurysms (VAA), apart from the aorto-iliac artery aneurysms. VAA´s are rare, gastroduodenal artery aneurysms (GDAA), constituting 1.5% of visceral artery aneurysms. A woman in her early 80s´ presented with chronic epigastric pain, weight loss, and nausea. Conservative management was unsuccessful. Imaging revealed a GDAA, prompting endovascular coil embolization. Subsequent evaluation confirmed Polyarteritis Nodosa (PAN), treated with rituximab. The report underscores the diagnostic challenges, emphasizing the need for a multidisciplinary approach using imaging and angiography. GDAA\'s potential life-threatening rupture necessitates prompt intervention, as illustrated in this case. The rare association with PAN, although infrequent, underscores the importance of considering underlying etiologies in multiple visceral aneurysms. Early diagnosis and intervention are pivotal for this uncommon yet potentially lethal condition.
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  • 文章类型: Case Reports
    背景:结节性多动脉炎(PAN)是一种系统性血管炎(SV),历史上被认为可以节省冠状动脉。冠状动脉造影和当代影像学显示冠状动脉狭窄和扩张,这与显著的发病率和死亡率有关。PAN中的冠状动脉由于全身性炎症而加重了动脉粥样硬化,从而增加了固有的动脉炎过程。传统的动脉粥样硬化危险因素无法近似风险。很少有报告记录冠状动脉病理学和最佳治疗方法。
    方法:1990-2022年英文文献数据库出版物查询。
    结果:冠状动脉受累的严重程度不包括实验室监测,但是冠心病与一些临床症状有关。弗雷明汉危险因素不足以近似疾病负担。将动脉粥样硬化与动脉炎分开需要先进的血管造影方法。治疗包括抗凝治疗,免疫抑制和血运重建。PCI一直是主流,尽管支架置入被管腔直径的变化和放置后不久新内膜化的报道所混淆。
    结论:当移植物选择避免SV的血管区域时,CABG提供明确的治疗。除了审查之外,我们还提供了一种新颖的CABG配置的报告,更新和讨论文献。越来越多的证据表明,离散的临床症状值得怀疑冠状动脉受累。
    BACKGROUND: Polyarteritis Nodosa (PAN) is a systemic vasculitis (SV) historically thought to spare the coronary arteries. Coronary angiography and contemporary imaging reveal coronary stenosis and dilation, which are associated with significant morbidity and mortality. Coronary arteries in PAN are burdened with accelerated atherosclerosis from generalized inflammation adding to an inherent arteritic process. Traditional atherosclerotic risk factors fail to approximate risk. Few reports document coronary pathology and optimal therapy has been guarded.
    METHODS: Database publication query of English literature from 1990-2022.
    RESULTS: Severity of coronary involvement eludes laboratory monitoring, but coronary disease associates with several clinical symptoms. Framingham risk factors inadequately approximate disease burden. Separating atherosclerosis from arteritis requires advanced angiographic methods. Therapy includes anticoagulation, immunosuppression and revascularization. PCI has been the mainstay, though stenting is confounded by vagarious alteration in luminal diameter and reports of neointimization soon after placement.
    CONCLUSIONS: When graft selection avoids the vascular territory of SV\'s, CABG offers definitive therapy. We have contributed report of a novel CABG configuration in addition to reviewing, updating and discussing the literature. Accumulating evidence suggests discrete clinical symptoms warrant suspicion for coronary involvement.
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  • 文章类型: Case Reports
    自发性包膜下和肾周出血,被称为Wunderlich综合征(WS),是结节性多动脉炎(PAN)的罕见临床表现。我们报告了一例48岁的男性,有腿部肌肉压痛和感觉障碍的反复发作史,双侧侧腹疼痛,下肢疼痛的结节性皮肤病变,减肥,和难以控制的动脉高血压。腹肾盂CT血管造影显示左侧大的肾周血肿,导致病人进入重症监护病房。排除传染性或肿瘤性病灶后,患者被诊断为PAN,并开始静脉注射甲基强的松龙脉冲,反应良好.由于WS是PAN的罕见初始临床表现,早期诊断和积极治疗将显著改善临床结局.
    Spontaneous subcapsular and perirenal hemorrhage, known as Wunderlich syndrome (WS), is a rare clinical manifestation of polyarteritis nodosa (PAN). We report a case of a 48-year-old male with a history of recurrent episodes of leg muscle tenderness and dysesthesia, bilateral flank pain, painful nodular skin lesions in the lower limbs, weight loss, and difficult-to-control arterial hypertension. The abdominopelvic computed tomography angiography showed a large left perirenal hematoma, leading to the patient\'s admission to the intensive care unit. After the exclusion of infectious or neoplastic foci, the patient was diagnosed with PAN and started intravenous methylprednisolone pulses with a good response. Since WS is a rare initial clinical manifestation of PAN, an early diagnosis and aggressive treatment will significantly improve clinical outcomes.
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  • 文章类型: Journal Article
    目标:疫苗接种对于最大限度地减少包括COVID-19大流行在内的全球健康危机的影响至关重要。这项研究调查了COVID-19疫苗接种与中等血管血管炎发生之间的潜在关联。
    方法:利用几个数据库进行综合文献综述。仔细评估这些研究以确保其质量并消除任何潜在的偏见。
    结果:在查看了935个搜索结果并删除了重复项之后,我们选择了10例病例报告。我们发现接种COVID-19疫苗后可能会发生中等血管血管炎,通常出现在疫苗接种后约16.2天。研究中的患者中位年龄为43.5岁,主要为男性(80%)。此外,一半的病例是在第二剂疫苗接种后报告的。
    结论:疫苗相关血管炎是COVID-19疫苗接种的一种罕见但可能的并发症,缺乏明确的治疗方案。
    OBJECTIVE: Vaccinations are essential in minimizing the effects of global health crises including COVID-19 pandemic. This study investigates the potential association between COVID-19 vaccination and the occurrence of medium vessel vasculitis.
    METHODS: Several databases were utilized to conduct a comprehensive literature review. The studies were carefully evaluated to ensure their quality and eliminate any potential bias.
    RESULTS: After reviewing 935 search results and removing duplicates, we selected 10 case reports. We discovered that medium vessel vasculitis may occur after COVID-19 vaccination, typically appearing around 16.2 days after vaccination. The patients in the study had a median age of 43.5 years and were predominantly males (80%). Additionally, half of the cases were reported after the second dose of vaccination.
    CONCLUSIONS: Vaccination-associated vasculitis is a rare yet possible complication of COVID-19 vaccination and lacks a clear treatment protocol.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    背景:结节性皮肤多动脉炎(cPAN)是一种中型血管坏死性血管炎。这是一种罕见的,结节性多动脉炎的皮肤限制性变异,以真皮和皮下组织受累为特征。cPAN中最常见的发现包括数字坏疽,网状livedo,和皮下结节。然而,虽然仅限于皮肤,由于伴随的皮肤缺血和坏死,cPAN导致显著的发病率和死亡率,这样患者就容易受到双重感染。这里,我们描述了与肺动脉高压(PAH)相关的cPAN的独特表现。
    方法:一位78岁的女性患者出现与PAH相关的指缺血和腿部溃疡。皮肤活检显示真皮中小血管的坏死性纤维蛋白样坏死。诊断为cPAN和PAH。患者接受糖皮质激素治疗,血管扩张剂,和环磷酰胺.
    结果:她死于严重的脓毒症并发症。
    结论:迄今为止,这是首例描述cPAN和PAH之间关联的病例报告.在这种情况下,PAH是皮肤血管炎的并发症,表明血管病变可能在PAH的病理生理学中起作用。然而,潜在的病理生理机制仍需牢固建立。
    BACKGROUND: Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH).
    METHODS: A 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide.
    RESULTS: She died due to severe sepsis complications.
    CONCLUSIONS: To date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.
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  • 文章类型: Case Reports
    结节性多动脉炎(PAN)是一种罕见的血管炎。急性肢体缺血是PAN的罕见表现和并发症。普通的旧球囊血管成形术(POBA)是解决PAN相关的严重威胁肢体缺血(CLTI)的治疗策略之一。然而,狭窄和闭塞的复发是常见的,使POBA成为不良的治疗选择,正如我们描述的临床病例所证明的。因此,考虑到西罗莫司的抗炎和免疫抑制特性,我们使用西罗莫司涂层球囊治疗PAN诱导的CLTI。一名37岁的女性首先表现为急性肢体缺血作为她的最初症状。诊断性血管造影显示她的胫骨血管闭塞,并进行POBA恢复灌注。在她生病的后期,尽管接受了多个疗程的免疫抑制药物,并且多次尝试使用POBA来挽救肢体,但她还是出现了足部坏疽。因为她的疾病轨迹,MagicTouch(概念医疗)在她的第三次血管成形术期间,将西罗莫司涂层球囊部署到她的前胫骨动脉。在她最后一次血管成形术后17个月,她仍然没有溃疡,和监测扫描显示胫骨血管无闭塞。使用西罗莫司涂层球囊血管成形术是一种有希望的治疗方法,可成功挽救PAN血管炎和CLTI患者的肢体。
    Polyarteritis nodosa (PAN) is a rare form of vasculitis. Acute limb ischemia is a rare presentation and complication of PAN. Plain old balloon angioplasty (POBA) is one of the treatment strategies for addressing PAN-related critical limb threatening ischemia (CLTI). However, recurrence of stenosis and occlusion is frequent, making POBA a poor treatment choice, as evidenced in our described clinical case. Consequently, with consideration of sirolimus\'s anti-inflammatory and immunosuppressive properties, we used a sirolimus-coated balloon in the treatment of PAN-induced CLTI. A 37-year-old woman first presented with acute limb ischemia as her initial symptom. Diagnostic angiography demonstrated occlusion of her tibial vessels, and POBA was performed to restore perfusion. Later in the course of her illness, she developed foot gangrene despite multiple courses of immunosuppressive drugs and several attempts with POBA to achieve limb salvage. Because of her disease trajectory, a MagicTouch (Concept Medical) sirolimus-coated balloon was deployed to her anterior tibial artery during her third angioplasty. At 17 months after her last angioplasty, she remained ulcer free, and surveillance scans demonstrated occlusion-free tibial vessels. The use of sirolimus-coated balloon angioplasty is a promising treatment approach for successful limb salvage in patients with PAN vasculitis and CLTI.
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  • 文章类型: Case Reports
    我们部门收治了一名33岁的女性,抱怨过去三个月中出现的多灶性感觉异常和上下肢无力。她还一直在接受dupilumab治疗特应性皮炎,抗白细胞介素4/13受体抗体。神经传导研究显示双侧肢体多灶性轴索感觉运动神经病。一入场,她的右前臂上出现了一个小红斑,但是由于其位置和时间过程,它对于血管性皮肤病变是非典型的。尽管如此,活检显示中型血管血管炎。因此,患者被诊断为皮肤动脉炎引起的血管神经病。甲泼尼龙冲击治疗泼尼松龙和硫唑嘌呤可显着改善她的症状。当怀疑多发性单神经病时,皮肤活检是有用的,即使皮肤发现不典型的血管炎皮疹。
    A 33-year-old female was admitted to our department complaining of multifocal paresthesia and weakness of the upper and lower extremities that had developed over the previous three months. She had also been undergoing treatment for atopic dermatitis with dupilumab, an anti-interleukin 4/13 receptor antibody. A nerve conduction study revealed multifocal axonal sensorimotor neuropathy of bilateral limbs. On admission, a small erythema appeared on her right forearm, but it was atypical for vasculitic skin lesions due to its location and time course. Nonetheless, a biopsy revealed medium-sized vessel vasculitis. The patient was therefore diagnosed with vasculitic neuropathy caused by cutaneous arteritis. Methylprednisolone pulse therapy with prednisolone and azathioprine markedly improved her symptoms. A skin biopsy is useful when mononeuropathy multiplex is suspected, even if the skin findings are atypical for vasculitic rash.
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