Aortitis no infecciosa: experiencia con tocilizumab en un hospital comarcal

ObjectivesDescribe patients with noninfectious aortitis and their response to treatment in a regional hospital.MethodsReview of patients with noninfectious aortitis, diagnostic technique used and immunosuppressive therapy received.ResultsWe report 8 patients (7 women and one man) diagnosed with aortitis by positron emission tomography (PET). The mean age was 69 years (interquartile range [IQR] 62-72.2). Three months of treatment with tocilizumab improved symptoms, erythrocyte sedimentation rate and C-reactive protein level (P < .001 and P < .012, respectively) in the 6 patients in whom it was used.ConclusionsTocilizumab was an effective and safe treatment in those patients diagnosed with aortitis refractory to steroids and conventional immunosuppressive therapy.     

大动脉炎合并脑梗死49例临床分析

目的 总结大动脉炎合并脑梗死患者的临床特点，分析此类患者脑梗死复发的相关因素。 方法 回顾性分析2010年1月-2020年6月随访超过3年的大动脉炎合并脑梗死患者的临床资料，对 此类患者脑梗死复发的相关因素进行分析。 结果 共纳入49例患者，首次脑梗死中位年龄30.0（22.0～45.0）岁，中位随访时间3.4（3.2～3.7）年。 11例（22.4%）出现复发性脑梗死，两次脑梗死间隔中位时间8.0（5.0～88.0）个月。与脑梗死无复发 患者相比，复发性脑梗死患者合并糖尿病比例（27.3% vs 2.6%，P =0.031）以及首次脑梗死后随访 期间平均TG水平（3.65±0.96 mmol/L vs 1.14±0.54 mmol/L，P =0.001）升高。大动脉炎受累动脉以颈 总动脉最为常见（48/49，98%），其次是椎动脉（40/49，81.6%），受累血管病变性质以狭窄（49/49， 100%）和闭塞（36/49，73.5%）最常见。脑梗死复发组动脉闭塞比例（100% vs 65.8%，P =0.024）和 动脉血栓发生率（45.5% vs 13.2%，P =0.033）均高于脑梗死无复发组。治疗方面，脑梗死复发组抗 血小板治疗患者比例（63.6% vs 97.4%，P =0.007）及接受血管重建术的患者比例（27.3% vs 68.4％， P =0.033）均低于脑梗死无复发组。 结论 大动脉炎合并脑梗死的患者，如合并糖尿病、血脂控制欠佳、主动脉弓分支动脉有闭塞、血 栓形成者，脑梗死复发风险高。     

Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.     

Survey of Japanese dermatological vasculitis specialists on cases of cutaneous arteritis (cutaneous polyarteritis nodosa)

We developed a questionnaire to examine the findings of cutaneous arteritis among dermatological specialists experienced in vasculitis as certified by the Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. We sent a questionnaire to 12 dermatological facilities identified through the revised Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. Retrospective data obtained from 84 patients at the 12 dermatological facilities between 2012 January 2016 December were evaluated. The 84 patients were categorized into two groups, a systemic steroid treatment group (group 1, n = 52) and a no systemic steroid treatment group (group 2, n = 32). C-reactive protein in group 1 patients was significantly higher than that in group 2 patients. Frequency of fever, arthritis, myalgia- and peripheral neuropathy in group 1 was significantly higher than that in group 2. We propose that these symptoms could serve as early markers for the transfer from cutaneous arteritis to systemic polyarteritis nodosa. We further suggest that patients who are subsequently associated with cerebral hemorrhage and infarction, who are originally diagnosed as having cutaneous arteritis, could progress to systemic polyarteritis nodosa. The study demonstrated that it is important for dermatologists to detect these findings early in order to establish an accurate diagnosis and a timely treatment.     

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??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.     

Polyarteritis Nodosa-Induced Pancreaticoduodenal Artery Aneurysmal Rupture

Polyarteritis nodosa (PAN) is a systemic, necrotizing vasculitis of small- and medium-sized arteries typically with multiorgan involvement. Most cases of PAN are idiopathic, although hepatitis B or C virus infections and hairy cell leukemia are important in the pathogenesis of some cases. PAN is characterized as segmental transmural inflammation of muscular arteries. Diagnosis is based on clinical suspicion, a negative immunofluorescence test for antineutrophil cytoplasmic antibodies, and whenever possible, biopsy conformation. Angiographic images may reveal microaneurysms affecting the renal, hepatic, or mesenteric vasculature. Aneurysmal formation and rupture are important complications that can be fatal. Treatment may warrant immunosuppression with steroids and cyclophosphamide. If left untreated, PAN can be fatal. To our knowledge, we report the second documented case of PAN-induced ruptured inferior pancreaticoduodenal artery aneurysm.     

儿童风湿病国际相关诊治指南系列解读之七——儿童大动脉炎分类标准解读

大动脉炎（TA）是由免疫机制介导的慢性、肉芽肿性、炎症性疾病，病变主要累及主动脉及其一级分支。现今儿童TA诊断多采用由欧洲抗风湿病联盟、欧洲儿童风湿病学会和儿科风湿病国际研究组织共同制定的分类标准。影像学检查是诊断TA及评估疾病活动程度的重要方法。欧洲儿童风湿病单中心及访问点所发布的共识对该标准做了进一步补充。