Aortitis

主动脉炎
  • 文章类型: Journal Article
    一名81岁的前列腺癌患者(cT3aN0M0),他们已经接受了4年的激素治疗,并且保持了较低的前列腺特异性抗原水平,盆腔淋巴结转移。组织活检显示转移性淋巴结中前列腺癌的神经内分泌分化。因此,开始用卡铂+依托泊苷进行化疗。在第一道菜中,由于中性粒细胞计数下降至230/μl,非格司亭给药2天。在第二个课程中,在第4天给予pegfilgrastim作为预防。然而,在第二道菜的第10天,他开始发烧和疲劳。怀疑感染,使用抗生素,但未能改善他的症状.在第14天,普通计算机断层扫描显示主动脉炎症的迹象。鉴于即使经过一周的抗生素治疗也缺乏改善,类固醇治疗开始怀疑粒细胞集落刺激因子(G-CSF)诱导的主动脉炎,这迅速改善了他的症状。因此,当遇到在使用G-CSF药物进行化疗期间发烧对抗生素无反应的病例时,需要考虑G-CSF药物引起的主动脉炎症的鉴别诊断.
    An 81-year-old man with prostate cancer (cT3aN0M0), who had been undergoing hormonal therapy for 4 years and had maintained low prostate specific antigen levels, developed metastasized pelvic lymph nodes. A tissue biopsy revealed neuroendocrine differentiation of prostate cancer in the metastatic lymph nodes. Consequently, chemotherapy with carboplatin+etoposide was initiated. During the first course, filgrastim was administered for 2 days due to a drop in his neutrophil count to 230/μl. During the second course, pegfilgrastim was administered as prophylaxis on day 4. However, on day 10 of the second course, he started to develop a fever and fatigue. Suspecting infection, antibiotics were administered, but failed to ameliorate his symptoms. On day 14, plain computed tomography revealed signs of aortic inflammation. Given the lack of improvement even after one week of antibiotic therapy, steroid treatment was initiated on the suspicion of granulocyte colony-stimulating factor (G-CSF) -induced aortitis, which rapidly improved his symptoms. Therefore, when encountering a case in which a fever remains unresponsive to antibiotics during chemotherapy with G-CSF agents, a differential diagnosis of aortic inflammation caused by G-CSF agents needs to be considered.
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  • 文章类型: Journal Article
    免疫检查点抑制剂(ICI)被认为是某些癌症的新型治疗方式。由于其显著的功效和对生存率的影响,它们可能很快被广泛使用,甚至作为癌症治疗的一线选择。特别是在晚期转移性癌症的病例中。值得注意的是,这些药物可能揭示新的自身免疫性疾病,并导致先前存在的自身免疫性疾病的爆发。近年来,该领域的数据已经积累。早期检测和协作方法是,因此,对于患有任何这些疾病的患者的管理至关重要。在这里,我们报道了1例诊断为转移性肾细胞癌的患者,其在nivolumab治疗期间表现为主动脉血管炎.在这种情况下,我们的目的是根据文献提高风湿病学家对ICI相关血管炎的认识。
    Immune-checkpoint inhibitors (ICIs) are considered as the novel treatment modality in certain cancers. They may soon be used widely even as the first-line option for cancer treatment due to their remarkable efficacies and impacts on survival rates, particularly in cases of advanced metastatic cancer. Of note, these agents might unveil new autoimmune diseases as well as causing flare-ups of a pre-existing autoimmune disease. Data in this field have been accumulated during recent years. Early detection and a collaborative approach are, therefore, crucial in the management of a patient who presents with any of these conditions. Herein, we report a patient with a diagnosis of metastatic renal cell cancer presented with vasculitis involvement in the aorta during nivolumab treatment. Our aim with this case is to increase the awareness of ICI-related vasculitis involvement among rheumatologists in the light of literature.
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  • 文章类型: Journal Article
    目的:巨细胞动脉炎(GCA-主动脉炎)的主动脉炎是一种常见的并发症,可能导致动脉瘤。Tocilizumab(TCZ)在GCA中获得批准,但迄今为止尚未对GCA-主动脉炎和动脉瘤的疗效进行分析.我们的目的是评估TCZ在一系列GCA主动脉炎和动脉瘤中的有效性和安全性。
    方法:使用TCZ治疗GCA-主动脉炎的多中心观察性研究。GCA诊断为:a)ACR标准,b)颞动脉活检,和/或c)成像技术。主动脉炎主要通过PET/CT诊断。主要结果为EULAR和影像学缓解。其他人是临床缓解,分析标准化,保留皮质类固醇的作用,以及动脉瘤的预防和改善。
    结果:196例GCA主动脉炎患者接受TCZ治疗。六个月后,72.2%达到EULAR缓解,但只有12%的影像学缓解;增加高达81.4%和31.8%,分别,在24个月。快速临床缓解,观察到ESR和CRP正常化在47.4%,84.3%和55.6%,在1个月,增加到89.6%,24个月时分别为85.3%和80.3%,分别。10例(5%)患者存在动脉瘤。其中五人需要早期手术,另外3个扩大了。在随访期间,没有接受TCZ治疗的患者出现动脉瘤。
    结论:在接受TCZ治疗的GCA主动脉炎患者中,观察到快速且持续的临床和分析改善.然而,临床缓解和EULAR缓解与影像学缓解之间存在解耦.
    OBJECTIVE: Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms.
    METHODS: Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms.
    RESULTS: 196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up.
    CONCLUSIONS: In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission.
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  • 文章类型: Journal Article
    目的:系统评价现有证据,使用18F-FDGPET/CT分析风湿性多肌痛(PMR)或巨细胞动脉炎(GCA)患者颅外大血管血管炎(LVV)的患病率。
    方法:检索PubMed和EMBASE,并由两名评审员筛选结果。使用改良版本的纽卡斯尔-渥太华量表评估研究质量。使用I2统计量和Q检验评估研究之间的异质性。按疾病类型进行了进一步的亚组分析,学习质量,和18F-FDGPET/CT摄取标准。通过漏斗图和Egger检验评估发表偏倚。
    结果:确定了268种出版物,其中17项符合选择标准,并纳入荟萃分析.通过18F-FDGPET/CT检查,颅外LVV的总体合并患病率为54.5%[95%CI:42.6%至66.1%]。GCA患者的患病率明显高于PMR患者(60.1%vs.41.8%,p=0.006)。同样,偏倚风险较低的研究报告颅外LVV患病率较高(61.1%vs.46.9%;p=0.010)。未观察到发表偏倚。
    结论:18F-FDGPET/CT检查可用于检测颅外LVV,PMR或GCA患者。这种参与在GCA患者中更为常见,并且可能根据研究的质量而有所不同。
    OBJECTIVE: Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using 18F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA).
    METHODS: PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I2 statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and 18F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger\'s test.
    RESULTS: 268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by 18F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed.
    CONCLUSIONS: The 18F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies.
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  • 文章类型: Case Reports
    一名54岁的女性在接受dabrafenib治疗时被诊断为Erdheim-Chester病,表现出心力衰竭的临床症状。在停止使用有问题的药物并开始指导指南的药物治疗射血分数降低的心力衰竭后,临床表现有所改善。
    A 54-year-old woman with a diagnosis of Erdheim-Chester disease under therapy with dabrafenib presents with clinical signs of heart failure. After discontinuing the offending medication and initiating guideline-directed medical therapy for heart failure with reduced ejection fraction, the clinical picture improved.
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  • 文章类型: Journal Article
    目的:巨细胞动脉炎(GCA)是最常见的大血管(LVV)血管炎之一,与复发和心血管并发症的高风险相关。改善风险分层仍然是该患者群体中的重要问题。我们的目的是在GCA中进行聚类分析,以识别聚类并评估其预后价值。
    方法:在一项多中心队列研究中,我们对283名GCA患者的混合数据坐标结果进行了因子分析,以产生聚类并评估复发率,心血管事件和死亡。
    结果:确定了三个簇:“血管复发特征”(23.0%),“典型GCA档案”(47.7%),和“眼科老年人概况”(29.3%)。“血管复发特征”群包括复发和心血管事件更频繁的年轻患者,特别是胸主动脉瘤。“典型的GCA简介”是最大的,有典型的颅骨表现和经常相关的风湿性多肌痛。“眼科老年人概况”是年龄最大的患者,视力丧失更多,死亡率最高。
    结论:我们的发现强调了GCA中不同的预后景观,强调年轻左心室受累患者的心血管预后不良,老年患者的死亡率较高。这加强了对主动脉异常筛查以及这些患者是否可能从生物治疗和心血管危险因素管理的强化治疗中受益的进一步研究的需要。
    OBJECTIVE: Giant cell arteritis (GCA) is one of the most common large vessel (LVV) vasculitis and is associated with a high risk of relapse and cardiovascular complications. Improving risk stratification remains a significant issue in this patient population. We aimed to perform a cluster analysis among GCA to identify clusters and evaluate their prognostic value.
    METHODS: In a multicenter cohort study, we performed hierarchical cluster analysis on the factor analysis of mixed data coordinates results with 283 GCA patients\' characteristics to generate clusters and assess incidence of relapse, cardiovascular events and death.
    RESULTS: Three clusters were identified: \"Vascular relapsing profile\" (23.0%), \"Typical GCA profile\" (47.7%), and \"Ophthalmologic elderly profile\" (29.3%). The \"Vascular relapsing profile\" cluster included younger patients with more frequent relapses and cardiovascular events, particularly thoracic aortic aneurysms. The \"Typical GCA profile\" was the largest, with classic cranial manifestations and frequently associated polymyalgia rheumatica. The \"Ophthalmologic elderly profile\" had the oldest patients with more visual loss and the highest mortality rate.
    CONCLUSIONS: Our findings underline the varied prognostic landscape within GCA, emphasizing the poor cardiovascular prognosis of younger patients with LV involvement and the higher mortality among elderly patients. This reinforces the need for further research regarding the screening of aortic abnormalities and whether those patients might benefit from intensive treatment with biotherapy and cardiovascular risk factors management.
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  • 文章类型: Journal Article
    Syphilitic aortitis is a rare disease caused by Treponema pallidum affecting the aorta and leading to inflammation. Syphilitic aortitis is one of the causes of aortic aneurysms. This article presents surgical treatment of a patient with syphilitic aortitis and thoracic aortic aneurysm. This clinical case confirms the difficulties of surgical treatment.
    Сифилитический аортит является редким заболеванием, вызванным инфекцией Treponema pallidum, которая воздействует на аорту и приводит к ее воспалению и повреждению. Сифилитический аортит является одной из причин формирования аневризмы аорты. В данной статье представлен клинический случай хирургического лечения пациента с сифилитическим аортитом и аневризмой грудной аорты. Клинический случай подтверждает сложности хирургического лечения.
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  • 文章类型: Journal Article
    Scedosporiumspp.和长龙孢菌是新兴的非曲霉丝状真菌。我们以前进行的Scedosporiosis/lomentosporiosis观察性研究报告了频繁的真菌血管受累,包括主动脉炎和外周动脉炎。对于这篇文章,我们回顾了7例Scedosporiumspp。和产乳杆菌性动脉炎来自头孢孢子菌病/lomentosporiosis观察研究和13例来自已发表文献。据报道,70%(14/20)的病例患者存在潜在的免疫抑制,主要是那些有实体器官移植(10/14)。在50%(10/20)的病例中观察到骨关节感染的定位;感染经常(7/10)与血管感染部位相邻。Scedosporiumspp./20例患者中有9例在完成非血管性scedosporiosis/lomentosporiosis治疗后3个月内被诊断出感染。在8/11主动脉炎和6/10周围动脉炎病例中发现动脉瘤。侵袭性真菌疾病相关死亡人数较高(12/18[67%])。头孢孢子菌属的血管嗜性。产乳杆菌显示血管成像,比如计算机断层扫描血管造影,需要管理感染,特别是对于骨关节位置。
    Scedosporium spp. and Lomentospora prolificans are emerging non-Aspergillus filamentous fungi. The Scedosporiosis/lomentosporiosis Observational Study we previously conducted reported frequent fungal vascular involvement, including aortitis and peripheral arteritis. For this article, we reviewed 7 cases of Scedosporium spp. and L. prolificans arteritis from the Scedosporiosis/lomentosporiosis Observational Study and 13 cases from published literature. Underlying immunosuppression was reported in 70% (14/20) of case-patients, mainly those who had solid organ transplants (10/14). Osteoarticular localization of infection was observed in 50% (10/20) of cases; infections were frequently (7/10) contiguous with vascular infection sites. Scedosporium spp./Lomentospora prolificans infections were diagnosed in 9 of 20 patients ≈3 months after completing treatment for nonvascular scedosporiosis/lomentosporiosis. Aneurysms were found in 8/11 aortitis and 6/10 peripheral arteritis cases. Invasive fungal disease--related deaths were high (12/18 [67%]). The vascular tropism of Scedosporium spp. and L. prolificans indicates vascular imaging, such as computed tomography angiography, is needed to manage infections, especially for osteoarticular locations.
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  • 文章类型: Journal Article
    山羊原发性心血管疾病的报告很少见,最常见的是室间隔缺损,瓣膜性心内膜炎,外伤性心包炎,离子载体中毒和营养性心肌病。我们现在报告67公斤的病理结果,6岁,成年雌性波尔山羊表现出神经系统症状(即,压头,不稳定和划桨)和高温2天前死亡。对美洛昔康和青霉素-链霉素缺乏治疗反应,预后不良导致动物安乐死。尸检时,主要发现包括严重的主动脉夹层与腔内血栓形成和狭窄,肺充血和水肿.主动脉的组织学检查显示严重的慢性肉芽肿和纤维化夹层主动脉炎伴矿化。受影响的主动脉段的细菌培养导致多杀性巴氏杆菌的大量生长和葡萄球菌的适度生长。中枢神经系统的组织病理学发现与神经李斯特菌病一致。
    Reports of primary cardiovascular disease in goats are rare and most commonly include ventricular septal defect, valvular endocarditis, traumatic pericarditis, ionophore poisoning and nutritional cardiomyopathies. We now report the pathological findings in a 67 kg, 6-year-old, adult female Boer goat that presented with neurological signs (ie, head pressing, unsteadiness and paddling) and hyperthermia 2 days prior to death. Lack of therapeutic response to meloxicam and penicillin‒streptomycin and poor prognosis led to euthanasia of the animal. At necropsy, the main findings included severe aortic dissection with luminal thrombosis and stenosis, and pulmonary congestion and oedema. Histological examination of the aorta revealed severe chronic granulomatous and fibrosing dissecting aortitis with mineralization. Bacterial culture of the affected aortic segment resulted in isolation of a profuse growth of Pasteurella multocida and a moderate growth of Staphylococcus spp. Histopathological findings in the central nervous system were consistent with neurolisteriosis.
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  • 文章类型: Journal Article
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