MPA, microscopic polyangiitis

  • 文章类型: Case Reports
    血管炎是一组异质性疾病,其特征是中枢神经系统中小血管的多灶性节段炎症。脑血管炎的主要症状是中风,头痛,和脑病。其他症状包括癫痫发作,颅神经麻痹,和脊髓病。成像技术在确定血管炎的诊断和证明大脑受累中起着至关重要的作用。一名患有永久性心房颤动的89岁女性发生了栓塞性中风。在治疗中,左大脑中动脉完全顺行再灌注静脉溶栓和取栓,没有临床效果。头颅MRI显示在眼眶内侧有双侧卵圆形病变,最初被误解为眼眶肿瘤。最终诊断证实,由于炎症性动脉炎引起的眼眶变化,动脉壁增厚。十天后,进行了随访MRI,显示眼眶肿块完全消退.原发性中枢神经系统血管炎,表现为急性缺血性中风,早期全身溶栓治疗可能是可逆的。
    Vasculitis is a heterogeneous group of disorders characterized by multifocal segmental inflammation of the small and medium vessels of the central nervous system. The predominant symptoms of cerebral vasculitis are stroke, headache, and encephalopathy. Additional symptoms include seizures, cranial nerve palsies, and myelopathy. Imaging techniques play a crucial role in identifying the diagnosis of vasculitis and demonstrating brain involvement. An 89-year-old woman with permanent atrial fibrillation developed an embolic stroke. In treatment, intravenous thrombolysis and thrombectomy with complete antegrade reperfusion of the left middle cerebral artery was used, without the clinical effectiveness. Brain MRI revealed bilateral oval lesions in medial parts of the orbits, which were initially misinterpreted as orbital tumors. Final diagnosis confirmed thickened arterial walls as orbital changes due to inflammatory arteritis. Ten days later, follow-up MRI was performed and showed complete regression of the orbital masses. Primary central nervous system vasculitis, manifesting as acute ischemic stroke, may be reversible with early systemic thrombolytic treatment.
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  • 文章类型: Case Reports
    一名30岁女性出现心绞痛。冠状动脉造影显示左主干和右冠状动脉严重狭窄,冠状动脉内硝酸甘油并未改善。冠状动脉计算机断层扫描血管造影和正电子发射断层扫描显示冠状动脉口炎症和主动脉根脂肪绞合。她被诊断为血管炎和瓣膜炎,并接受了免疫治疗和冠状动脉搭桥术。(难度等级:高级。).
    A 30-year-old woman presented with angina pectoris. Coronary angiography revealed severe stenosis in the left main and right coronary arteries that did not improve with intracoronary nitroglycerin. Coronary computed tomography angiography and positron emission tomography revealed coronary ostia inflammation and aortic root fat stranding. She was diagnosed with vasculitis and valvulitis and received immunotherapy and coronary bypass. (Level of Difficulty: Advanced.).
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  • 文章类型: Case Reports
    抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)构成了一组自身免疫起源的血管炎症疾病。髓过氧化物酶(MPO)ANCA与ANCA相关的AAV密切相关。MPO-ANCA阳性AAV患者的肺部受累率很高;然而,只有少数报道的机化性肺炎(OP)病例。尽管经过了整整一个月的抗生素治疗,但由于38°C的发烧,一名78岁的男子被送往我们医院。胸部计算机断层扫描图像显示右肺中叶和左肺上叶可见限制性巩固,这建议了一个OP模式。MPO-ANCA和尿潜血试验阳性。经支气管活检的组织病理学检查显示OP和粘液栓。肾活检的组织学发现显示与AAV有关的坏死性肾小球肾炎。患者被诊断为MPO-ANCA阳性AAV,并接受全身性皮质类固醇治疗,他很快康复了。因此,诊断OP时,应通过订购患者血清ANCA和隐匿性血尿试验来考虑AAV的可能性。
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of blood vessel inflammation diseases of autoimmune origin. Myeloperoxidase (MPO) ANCA is closely related to ANCA associated AAV. The MPO-ANCA positive AAV patients have lung involvement at high rates; however, there are only a few reported cases with organizing pneumonia (OP). A 78-year-old man was presented to our hospital due to a fever of 38 °C despite a whole month of antibiotics treatment. Chest computed tomography image revealed restricted consolidations visible in the middle lobe of the right lung and the upper lobe of the left lung, which suggested an OP pattern. MPO-ANCA and urine occult blood tests were positive. Histopathological examination of the transbronchial biopsy revealed OP and mucus plug. Histological findings on renal biopsy showed necrotizing glomerulonephritis related to AAV. The patient was diagnosed with MPO-ANCA positive AAV and was treated with systemic corticosteroid therapy, from which he recovered rapidly. Thus, when diagnosing OP, the possibility of AAV should be considered by ordering patients\' serum ANCA and occult hematuria tests.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    已显示两种嗜肝病毒与全身性血管炎-乙型肝炎与经典结节性多动脉炎和丙型肝炎与冷球蛋白性血管炎有因果关系。本文提供了有关这些血管炎的临床表现和管理的最新概述。HBV相关的PAN患者有较高的体重减轻,周围神经病变,多发性单神经炎,腹痛,需要手术的胃肠道表现,心肌病,睾丸炎,高血压,和/或转氨酶水平升高。微动脉瘤在肠系膜动脉中也更常见。皮肤表现,然而,不太常见。这些患者还患有严重的疾病,如通过较高的五因子评分和较高的BVAS所提示的。虽然复发不太常见,与非HBVPAN相比,HBVPAN患者的死亡率更高。血浆置换在清除免疫复合物的治疗中具有作用。HCV相关冷球蛋白血症性血管炎的常见临床表现为皮肤损害,周围神经病变,肾小球肾炎,关节炎,还有干燥症状.尽管聚乙二醇干扰素α和利巴韦林的联合治疗是一线治疗,对于严重或危及生命的表现,需要免疫治疗。最近的随机试验表明利妥昔单抗在这种情况下的疗效。
    Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides. HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes. The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon α and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations.
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