大动脉炎临床特征及治疗转归173例分析

目的 探讨大动脉炎临床症状、受累血管分布特征及治疗转归。方法 回顾性分析173例大动脉炎患者的临床症状、血管影像学、炎性指标及治疗随诊情况。采用f检验进行统计学分析。结果 173例患者中女性136例,男性37例,中位发病年龄23岁。81例(46.6％)患者起病有高血压,72例(41.1％)患者无脉症/脉搏不对称,50例(28.7％)患者有发热的症状;受累血管分布情况：主动脉112例(64.7％),肺动脉17例(9.8％),无名动脉33例(19.1％),颈总动脉114例(65.9％),锁骨下动脉113例(65.3％),肾动脉63例(36.2％),椎动脉21例(12.1％),腹腔干10例(5.8％)。105例(61.0％)患者红细胞沉降率( ESR)升高。合并活动性/既往结核者45例,占26.0％,乙型肝炎病毒携带者10例,占5.8％。其中105例患者接受随诊,98例(94.2％)在治疗后病情平稳,17例在激素减量过程中病情反复。结论 糖皮质激素及（或）免疫抑制剂以及血管严重狭窄或闭塞者适时联合介入与手术,可有效控制大动脉炎患者的临床症状及炎性指标,改善患者的生存质量。     

13例儿童大动脉炎临床资料分析

目的研究儿童大动脉炎（takayasu arteritis，TA）的临床表现和治疗方案。方法对1999年1月——2008年8月住院的TA患儿临床资料进行回顾性分析。结果13例中，男6例，女7例，年龄3岁～12岁，平均9．3岁。按照Ishikawa’s criteria标准诊断大动脉炎。临床表现有高血压61．5％、心功能不全38．5％、脉搏减弱30．8％、心肌病23．1％、心脏瓣膜受累23．1％。主要的血管成像类型是Ⅱ型61．5％。抗高血压药，口服激素和控制充血性心力衰竭的药是主要的医疗措施。2例内科治疗无效患儿子经皮血管成形术（PCTA）疗效好。结论大动脉炎有脉搏减弱期，有高血压和其他并发症。TypeⅡ最常见。抗高血压药和激素治疗可控制症状。     

大动脉炎致脑梗死临床分析

目的 探讨大动脉炎致脑梗死患者的临床特点和诊治情况.方法 回顾性分析2002年1月～2010年4月在我院神经内科住院的12例大动脉炎所致脑梗死患者的临床资料.结果 12例患者均有颈总动脉或锁骨下动脉或椎动脉及其分支的狭窄或闭塞,查弓上颅外段动脉磁共振血管造影、CT血管成像均有阳性发现.均为青中年患者,以女性为主,经Essen卒中风险评分量表评分均为0～2分,属于脑卒中低危患者.经激素或加用小剂量免疫抑制剂治疗均有效.结论 大动脉炎致脑梗死容易漏诊.对年轻的脑卒中低危患者,特别是女性,需要明确有无大动脉炎,而弓上血管磁共振血管造影、CT血管成像有较高的诊断价值.明确诊断后应早期给予激素或加用小剂量免疫抑制剂治疗.     

大动脉炎合并脑梗死的临床分析

目的 探讨大动脉炎合并脑梗死患者的临床特点和治疗情况。方法 回顾性分析1998-2003年在我院住院的14例大动脉炎合并脑梗死患者的临床资料。结果 大动脉炎合并脑梗死患者占同期住院的大动脉炎患者10．6％(14／132)。出现脑梗死距大动脉炎首发症状时间为1个月～15年。14例均为3支以上主动脉弓上分支动脉狭窄或闭塞并表现为偏瘫，经CT证实有脑梗死，梗死部位最多见于基底节区。激素、细胞毒药物等治疗有效。结论 大动脉炎合并脑梗死动脉病变范围广泛，临床上要予以重视，避免误诊．治疗的关键是控制原发病。     

多发大动脉炎的介入治疗

晚期大动脉炎药物治疗效果极差，外科手术治疗并发症多，死亡率高，介入治疗因为相对无创，并发症发生率低，已经成为晚期大动脉炎所致动脉狭窄或闭塞的首选治疗方法。     

大动脉炎累及冠状动脉7例临床分析

目的 :分析大动脉炎累及冠状动脉的患者的发病率、临床特点、冠状动脉病变性质及治疗效果 ,为临床提供参考。方法 :1990～ 2 0 0 2年 5 2例大动脉炎住院患者中 7例 (女 5例、男 2例 )患者 ,根据其临床表现、辅助检查、大血管造影及冠状动脉造影 ,证实为大动脉炎累及冠状动脉 ,予以激素、扩管等对症治疗 ,随访观察患者症状、心功能、炎症指标等病情变化。结果 :7例患者中 6例有不同程度的心肌缺血症状 ,2例出现心肌梗死 ,3例出现心力衰竭症状。 1例冠状动脉造影显示为左旋支中段中度狭窄 ,1例为左冠状动脉前降支开口处重度狭窄 ,另1例MRI和磁共振血管造影示广泛前壁心肌梗死。经对症治疗后 ,患者血沉、C反应蛋白均能回复正常 ,而症状与心功能无明显改善。结论 :多发性大动脉炎累及冠状动脉并不少见 ,可发生于冠状动脉开口与近端 ,亦可累及中段 ,随病程延长 ,患者心脏情况恶化 ,临床上应对有心肌缺血症状的大动脉炎患者积极行冠状动脉造影 ,早期诊断 ,积极治疗。     

冠状动脉及肺动脉同时受累的大动脉炎2例并文献复习

目的:探讨冠状动脉、肺动脉同时受累的多发性大动脉炎的临床特点,以提高对该病的认识。方法:对2例诊断为冠状动脉、肺动脉同时受累的多发性大动脉炎患者的进行回顾性分析并文献复习。结果:2例患者均有主动脉及分支不同程度、部位的受累,例1患者合并右冠状动脉主干开口管腔重度狭窄,右肺动脉闭塞。例2患者左冠状动脉主干管腔次全闭塞,右冠状动脉开口处管壁不规则增厚,右上肺动脉及其分支闭塞,2例患者均经激素免疫抑制剂等治疗,症状改善。经文献检索国外报道2例冠状动脉、肺动脉同时受累的大动脉炎病例,复习文献发现:计算机断层扫描血管成像(CTA)、磁共振血管成像(MRA)和正电子发射计算机断层/计算机断层扫描(PET/CT)可用于早期诊断及炎症活动的评估,术前术后的免疫抑制治疗是手术成功的关键。结论:冠状动脉、肺动脉同时受累的大动脉炎患者,血管病变广泛,心血管事件发生率高,预后极差。因此应提高临床医师对该病的早期诊断意识,规范免疫抑制治疗,必要时介入及手术干预,改善患者预后。     

青少年自身免疫性肝病患者的临床观察

目的分析青少年自身免疫性肝病患者的临床病理学特征及治疗转归,提高对该病的认识。方法回顾性分析2016年9月至2019年10月于上海交通大学医学院附属仁济医院消化科住院及门诊随访的32例青少年自身免疫性肝病患者的分型、临床特点、生物化学指标、肝脏病理、治疗效果及药物不良反应。结果 32例患者中自身免疫性肝炎(AIH)28例(1型AIH 25例,2型AIH 3例),自身免疫性硬化性胆管炎(ASC)4例。临床表现为急性肝炎型起病7例(21.9%),慢性隐匿型起病22例(68.8%),肝硬化及其并发症型起病3例(9.4%)。有13例患者单用激素治疗,2例因治疗时间未满1年故疗效是否应答不确切,1例不完全应答,10例完全应答,其中1例已成功停药,2例在完全应答缓解数年后复发,在激素基础上加用免疫抑制剂(这2例归为联合治疗)。21例根据病情应用激素联合免疫抑制剂治疗,11例不完全应答,10例完全应答。结论青少年AIH的临床表现多样,诊断困难,但对激素单药或联合硫唑嘌呤的免疫抑制治疗反应良好。AIH可转归至ASC,应定期对AIH患儿行磁共振胰胆管造影(MRCP)或逆行胰胆管造影(ERCP)检查以排查ASC,尤其是出现胆汁淤积症状者。     

大动脉炎累及心脏32例临床分析

目的 调查大动脉炎累及心脏患者的临床特点、病变分类及其性质，为临床提供参考。方法 回顾性分析同济医院1990～2002年间住院32例大动脉炎患者的临床资料，根据心脏临床症状、心电图、超声和冠状动脉造影等检查结果分析其心脏受累的类型及特点。结果 32例大动脉炎患者中有18例明显累及心脏，包括高血压心脏损害4例，主动脉瓣关闭不全8例(25．0％)，不同程度上冠状动脉的受累6例(18．8％)，伴有扩张型心肌病2例(6．3％)。结论 多发性大动脉炎累及心脏的影响除了继发性高血压心脏病外，还有冠状动脉狭窄、瓣膜病变以及心肌病变，这些均能造成心脏功能的损害，影响病人的预后，临床上应予以重视。     

老年肾病综合征的临床表现和病理类型分析

目的：进一步了解老年肾病综合征的临床表现和病理特点。方法：对北京协和医院1980－1999年底住院老年肾病综合征病人进行临床表现和病理类型分析。结果：住院成人肾病综合征病人共513例,其中老年肾病综合征61例,占11．9％,原发病为原发性肾小球肾炎者49例,占80．3％,糖尿病肾病者7例,占11．5％。临床表现以浮肿、血尿为主,贫血的发生率为54．1％,慢性肾功能不全（CRF）为32．8％。肾活检发现,在非糖尿病肾病综合征病人用激素或激素加免疫抑制剂治疗的缓解率为47．85,有效率为30．4％,未缓解率为21．7％,一般激素或激素加免疫抑制剂效果较好,其临床表现与年轻人的肾病综合征相似,但合并贫血、急性肾功能不全（ARF）、CRF和感染的比例较高,最常见的病因是慢性肾小球肾炎,其病理类型以系膜增殖型肾小球肾炎最常见,其次是局灶增殖型肾小球肾炎,老年肾病综合征患者对激素或激素加免疫抑制剂治疗的反应尚可。     

Severe chronic aortic insufficiency requiring valve replacement: an infrequent complication of Takayasu's disease

Takayasu's arteritis (TA) is a granulomatous vasculitis of medium and large arteries, which most often presents as pulseless disease due to widespread arterial stenoses. Only the minority of TA patients have aortic valve insufficiency, which is due to aortic root dilatation following aortitis and aneurysm formation. No other cardiac valve is involved. We report a case of Takayasu's disease-related severe aortic insufficiency (AI) in a Filipino woman, which necessitated aortic valve replacement. It is important to consider TA in the differential diagnosis of AI in young women, particularly those with early-onset systemic hypertension and pulse deficits. Early diagnosis and therapy of TA can improve outcomes.     

Polymyalgia rheumatica and temporal arteritis: A retrospective study of 111 patients

Summary There is no unanimity as to whether polymyalgia rheumatica (PMR) and temporal arteritis (TA) are two distinct diseases or different features of one disease.The objective of this study was to assess the value of histological findings of temporal artery biopsy and the efficacy and complications of drug therapy as well as the frequency of malignancies. It was carried out as a retrospective follow-up study. One hundred eleven patients (89 PMR, 14 TA and 8 PMR+TA) were studied. In 56 patients with PMR a temporal artery biopsy was performed; in none of these biopsies was active arteritis found. Of the 19 patients with TA or PMR+TA, where a temporal artery biopsy was performed, arteritis was found in 15 patients. Reactivation occurred in 27 patients: 4 patients using NSAIDs and 23 patients using corticosteroids. Side effects of the medication included vertebral compression in 10 patients, most of whom were using corticosteroids.Malignancies were diagnosed in 12 of the 111 patients. Most malignancies were diagnosed long before or after the diagnosis of PMR.In case of a PMR diagnosed by the clinician a biopsy of the temporal artery has no value, while the yield of this diagnostic procedure is high in TA. Reactivation was seen quite often and warrants a prolonged period of medical treatment.     

Takayasu's arteritis and its therapy

Twenty patients with Takayasu's arteritis were followed prospectively for an average of 4.6 years. Sixteen patients with active inflammatory Takayasu's arteritis were treated with glucocorticosteroids; eight responded to therapy. Six patients had clinical or angiographic progression of their vasculitis on daily corticosteroid therapy. These patients were then given cyclophosphamide together with prednisone on alternate days. Four of these 6 patients had no progression of vascular lesions while receiving cyclophosphamide; two had progression of vascular lesions after 30 and 48 months of therapy. Vascular reconstructive surgery was successful in 7 patients who tolerated a total of 13 vascular surgical procedures without major complications. One bypass graft occluded after 13 months and was revised. With corticosteroid therapy, cytotoxic therapy, and surgery, no deaths due to Takayasu's arteritis or its treatment have occurred.     

Takayasu＇s arteritis following Crohn＇s disease in a young woman： Any evidence for a common pathogenesis？

Takayasu＇s arteritis and Crohn＇s disease are chronic inflammatory diseases of uncertain aetiology. They rarely occur together, with only twenty nine cases of co-existent Takayasu＇s arteritis and Crohn＇s disease reported in the literature. In 88% of these cases, Takayasu＇s arteritis was diagnosed simultaneously or following a diagnosis of Crohn＇s disease. We present a case of a young Caucasian medical student, incidentally found to have bilateral carotid bruits on auscultation by a colleague. Magnetic resonance angiography revealed stenoses of the common carotid arteries with established collaterals, and a diagnosis of Type 1 Takayasu＇s arteritis was made. An 18F-fluorodeoxyglucose positron emission tomography scan revealed no active disease. Nine months later, she presented with a short history of abdominal pain, vomiting and abdominal distension. Barium follow-through and computer tomography revealed a terminal ileal stricture and proximal small bowel dilation. An extended right hemicoloectomy was performed and histopathology supported a diagnosis of Crohn＇s disease. This case report is presented with a particular focus on the temporal relationship between these two disease processes and explores whether their concurrence is more than just co-incidence.     

Role of C-C chemokines in Takayasu's arteritis disease

BACKGROUND: Takayasu's arteritis (TA) is a chronic obliterative inflammatory disease. Inflammatory cell infiltration and destruction of the vessel wall in TA strongly suggest that cell mediated immunological mechanisms play an important role in the pathogenesis of this disease. Therefore, in the present study our aim was to focus on the role of chemokines and adhesion molecules in patients with Takayasu's disease. METHODS: Twenty-one patients with clinically defined TA and 21 healthy control volunteers were recruited by using the standard criteria. Patients with TA were divided into those with clear-cut clinically active or inactive disease based on vasculitis activity score. RESULTS: MCP-1 and hRANTES were significantly increased in patients with TA as compared to controls. MCP-1 and hRANTES values were reliably able to distinguish between patients with active disease vs. subjects in remission. sVCAM-1 levels remained unaltered between patients and controls. CONCLUSIONS: C-C chemokines can be used as reliable markers/diagnostic tools in determining the activity of Takayasu's arteritis.     

Echocardiographic follow-up of patients with Takayasu's arteritis: five-year survival

Takayasu's arteritis (TA) is a primary vasculitis that causes stenosis or occlusion, rarely aneurysm and distal ischemia. This study was undertaken to examine cardiovascular damage using echocardiography and determine the causes of morbid-mortality in Mexican Mestizo patients with TA. Seventy-six patients were studied by transthoracic echocardiography. Left ventricular diameters, parietal thickness, systolic function, and wall motion were analyzed, also, valvular lesions and aorta features were assessed. Thickness of the interventricular septum was 12 mm +/- 3 (8-19), and that of posterior wall was 12 mm +/- 2 (9-18). The average left ventricular diastolic diameter was 47 mm +/- 7 (33-68) and the left ventricular systolic diameter 32 mm +/- 8 (16-64). The left ventricular ejection fraction was of 57 +/- 11%. Left ventricular concentric hypertrophy was found in 28 (50%) of the 56 hypertensive patients. The five-year survival of patients with left ventricular concentric hypertrophy was 80%, compared to 95% in patients without hypertrophy (P = 0.00). Abnormal wall motion was found in 15 patients. Thirty-one patients had aortic regurgitation, 19 had mitral regurgitation, 13 had tricuspid regurgitation, and 10 and pulmonary hypertension. Six patients had aneurysms of ascending aorta and 7 stenosis of descending aorta. Thirteen of 76 patients died (17%), 85% were hypertensive, and 9% also had acute myocardial infarction (AMI). Echocardiography, a noninvasive technique, shows a great utility in detection and follow-up of cardiovascular manifestations in patients with TA. New techniques, more sensitive toward detecting the early stages of left ventricular dysfunction, are promising to limit left ventricular hypertrophy development.     

Antimonocyte antibodies in Takayasu's arteritis: prevalence of and relation to disease activity

OBJECTIVE: To investigate the prevalence of antimonocyte antibodies (AMA) in Takayasu's arteritis (TA) and their relationship with disease activity. METHODS: IgG-AMA were studied in the sera of 60 patients with TA (29 active disease, 31 inactive) and 43 controls by a cellular ELISA using glutaraldehyde fixed U-937 cells or peripheral blood monocytes as antigen. Relationship of AMA with disease activity was evaluated by measuring titers of these antibodies in followup sera of 15 AMA positive patients with active TA undergoing immunosuppressive therapy. RESULTS: Twenty-six of 60 TA patients (43%) compared to 4 of 43 controls (9%) (p < 0.001) and 20 of 29 patients with active disease (69%) compared to 6 of 31 patients with inactive disease (19%) (p < 0.001) were positive for AMA. The antibody titers were significantly higher in patients with active disease than those with inactive disease (0.396 +/- 0.172 vs 0.232 +/- 0.096; p < 0.001). In the followup study of 15 patients with active disease who received immunosuppressive therapy, we observed normalization of AMA titers in 6 of the 7 patients who became inactive, compared to only one of the 8 patients whose disease remained active during followup (p < 0.01). CONCLUSION: AMA are present in a significant proportion of patients with TA and correlate with disease activity, suggesting a possible pathogenic role of these antibodies in TA.     

Male siblings with Takayasu's arteritis suggest genetic etiology

Summary Takayasu's arteritis is a nonspecific arteritis involving the aorta and its major branches. The disease mainly affects young females and familial incidence is uncommon. In this paper, two rare cases of male siblings with Takayasu's arteritis and the results of their HLA typing are described. The HLA haplotype of the two cases was completely identical—A2-B40-Cwl and A24(9)-Bw59-Cwl, DR2, and DR4. It is reported that BW52(5) is strongly associated with the disease. However, in our cases, Bw52(5) was not found, while DR2 and DR4, which have been reported in association with several autoimmune disease, were detected. Accordingly, in these cases, genetic factors might be associated with the pathogenesis of the disease through an autoimmune mechanism.     

老年大动脉炎患者的临床特点分析

目的 总结分析老年大动脉炎患者的临床特点,加强对老年大动脉炎患者的综合治疗.方法 入选阜外心血管病医院就诊的60岁以上大动脉炎病例,回顾性分析其病史、临床特点.结果 26例大动脉炎患者女性22例,男性4例.发病年龄均<40岁,其中确诊年龄≤40岁者15例,41～50岁者1例,51～60岁者7例,>60岁者3例;26例患者中9例合并冠状动脉粥样硬化性心脏病,7例合并高血压,4例合并2型糖尿病,2例合并心律失常,1例合并亚急性感染性心内膜炎;临床表现伴高血压者21例(81%),头晕12例(46%),胸痛9例(35%),上肢无脉或脉弱者7例(27%);临床分型为头臂动脉型8例(30%),胸腹主动脉型4例(15%),广泛型11例(58%),肺动脉型3例(12%);患者血沉(ESR)、C反应蛋白(CRP)、抗链球菌溶血素(ASO)升高分别为10例(50%)、6例(32%)、14例(78%);2例死亡病例均合并糖尿病,其余患者药物治疗控制病情活动性及其他疾病和危险因素.结论 老年大动脉炎患者常处于疾病活动期且可能并存其他心血管疾病和危险因素,应加强对老年大动脉炎患者的综合治疗.