肺动脉受累大动脉炎误诊为肺栓塞4例分析

大动脉炎（又称Takayasu动脉炎,高安动脉炎）是主要累及弹性动脉的特发性慢性炎症性疾病,以引起不同部位血管的狭窄或闭塞为主,少数患者也可呈动脉扩张或动脉瘤,可累及主动脉及其主要分支,以及肺动脉。肺动脉受累的大动脉炎属少见病。肺栓塞是指血栓进入肺动脉及其分支,     

胸主动脉破损并巨大血肿形成的大动脉炎一例

大动脉炎（takayasu arteritis）是指主动脉及其主要分支的慢性进行性非特异性炎症,常引起不同部位动脉狭窄或闭塞,少数也可引起动脉扩张或动脉瘤,而出现动脉壁破裂并巨大血肿形成的国内外文献未见报道,我院遇到1例.对此患者的发病情况、病理表现及病理与临床的关系分析如下,并进行相关文献复习.     

多发性大动脉炎

多发性大动脉炎可能是一种自身免疫性疾病,它是主动脉及其主要分支的非特异性炎症性疾病,临床表现复杂多样,可引起不同部位的狭窄或闭塞,以致病变处动脉扩张或形成动脉瘤,侵犯以头臂动脉受累最多。此病发病年龄较轻,早期症状可不典型,临床工作中并不少见,应引起重视,早期诊断,早期治疗,对延缓病情的进展有重要意义。     

大动脉炎的诊断和治疗

大动脉炎(Takayasu arteritis,TA)是一种累及主动脉及其主要分支的慢性进行性非特异性炎症,其发病涉及遗传因素、自身免疫机制、内分泌失常等改变,可引起不同部位血管的狭窄或闭塞病变。主要临床表现为全身炎性反应以及受累脏器缺血症状。因受累血管的部位、程度和范围不同,症状轻重不一,可表现为全身不适、易疲劳、发热、食欲减退、恶心、出汗、体质量下降、肌痛关节炎等全身症状和因血管狭窄或闭塞所致的局部缺血症状和体征。对所有 TA 患者均需要进行影像学检查以及大血管造影了解动脉受累情况。对临床上有低热、关节痛及类似感冒样症状时,尤其是青年女性,应仔细进行体格检查,从双侧以及上下肢肢体血压的测定和外周血管的体检中发现线索。男性患者患病比例低,病初反应轻,出现症状晚,发病早期容易误诊或漏诊。多发大动脉炎的治疗分为手术治疗和非手术治疗。活动期的患者应该给与激素和免疫抑制剂治疗。血管障碍危及脏器血运则需要选择手术治疗。大动脉炎早期表现多为非特异性,诊断困难。因此,早期明确诊断,有助于尽快确定合适的治疗方案,抓住时机进行治疗,从而减少大动脉炎引起的损伤。     

大动脉炎的诊断和治疗

大动脉炎(Takayasu arteritis,TA)是一种累及主动脉及其主要分支的慢性进行性非特异性炎症,其发病涉及遗传因素、自身免疫机制、内分泌失常等改变.可引起不同部位血管的狭窄或闭塞病变.主要临床表现为全身炎性反应以及受累脏器缺血症状.因受累血管的部位,程度和范围不同,症状轻重不一,可表现为全身不适、易疲劳、发热、食欲减退、恶心、出汗、体质量下降、肌痛关节炎等全身症状和因血管狭窄或闭塞所致的局部缺血症状和体征.对所有TA患者均需要进行影像学检查以及大血管造影了解动脉受累情况.对临床上有低热、关节痛及类似感冒样症状时,尤其是青年女性,应仔细进行体格检查,从双侧以及上下肢肢体血压的测定和外周血管的体检中发现线索.男性患者患病比例低,病初反应轻,出现症状晚,发病早期容易误诊或漏诊.多发大动脉炎的治疗分为手术治疗和非手术治疗.活动期的患者应该给与激素和免疫抑制剂治疗.血管障碍危及脏器血运则需要选择手术治疗.大动脉炎早期表现多为非特异性,诊断困难.因此,早期明确诊断,有助于尽快确定合适的治疗方案,抓住时机进行治疗,从而减少大动脉炎引起的损伤.     

大动脉炎尸检病例的临床病理分析

为探讨大动脉炎的病理学改变与临床表现之间的关系，收集了该院建院以来的７例大动脉炎尸检病例。将７例尸检材料的大体解剖所见、组织学观察、临床表现及其并发症等几个方面进行整理、分类和对比。结果发现大动脉炎的病理改变主要以受累动脉壁增厚、僵硬和管腔狭窄，甚至闭塞为主，并随着缩窄和阻塞的部位和程度不同即出现复杂的临床症状。因此，大动脉炎病理改变的不同阶段、不同程度及不同部位都将直接影响患者的临床表现、并发症、预后以及存活期     

18例顽固性肾血管性高血压患者介入治疗的疗效分析

<正>肾血管性高血压是继发性高血压的常见病因之一,是由于单侧或双侧肾动脉主干或分支狭窄引起的高血压。常见病因有多发性大动脉炎,肾动脉纤维肌性发育不良和动脉粥样硬化。我国肾动脉狭窄(Renal artery stenosis,RAS)的主要病因为动脉粥样硬化,大动脉炎次之[1]。在成年恶性高血压患者中,肾血管性高血压约占6%～27%[2]。我院自2004年以来采用介入治疗RAS18例,现分析如下。     

多发性大动脉炎1例

<正>多发性大动脉炎(TA)是一种病因未明,主要累及主动脉及其主要分支的慢性进行性非特异性炎症,可引起不同部位的动脉狭窄或闭塞,表现为全身炎症及受累器官的缺血症状。该病发病率低,病因及发病机制受多重因素影响,诊治难度大,临床危害高。为提高对该病的认识,本文对我院收治的1例多发性大动脉炎病例进行临床特征、影像学检查、实验室检查及治疗等方面讨论。1病例患者女性,65岁,主因"胸闷气喘半月,剑突下疼痛伴呕吐5 d,加重1 d"于2018年3月27日至我院心血管内科就诊。患者半月前安静状态下偶发胸闷气喘、咳嗽。6 d前出     

107例大动脉炎的眼底改变与临床分析

目的：探讨大动脉炎与眼底改变的关系。方法：１０７例住院大动脉炎病人用直接眼底镜检查眼底，并在不同体位下观察其动态变化。结果：１０７例中有眼底改变者６３例（５８．９％），其中呈慢性缺血性改变１８例（２８．６％），全部为４０岁以下的女性，病变均累及头臂动脉；呈高血压性改变４５例（７１．４％），在女性和男性病例中各占３８．７％和６４．３％，病变均累及胸腹主动脉和（或）肾动脉。结论：眼底改变主要与动脉受累部位和程度有关，与性别、年龄、病程等也有一定关系。掌握大动脉炎的眼底特征，有助于该病的临床诊断。     

新型支架在大动脉炎累及冠状动脉中的应用

<正>大动脉炎是指主动脉及其主要分支的慢性进行性非特异性炎症,亦可累及肺动脉以及更远的外周动脉,病因未明。1大动脉炎累及冠状动脉临床特点以及介入治疗大动脉炎主要引起管腔狭窄或闭塞,少数导致动脉扩张、假性动脉瘤或夹层动脉瘤,临床症状复杂多样。文献报道,大动脉炎冠状动脉受累的比例差异较大为10%~30%,大部分为冠状动脉开口及近段病变,受累患者中仅5%~20%有相应症状[1-2]。患者可出现严重心肌缺血,有典型心     

Distinct profiles of endothelial gene expression in hyperpermeable regions of the porcine aortic arch and thoracic aorta

Among the early events associated with atherosclerotic lesion development are increased macromolecular permeability of the endothelium and expression of genes that affect inflammation and oxidative state. The purpose of this study was to measure the expression of several atherosclerosis-related genes in endothelial cells scraped from arch and thoracic regions of the porcine aorta exhibiting elevated permeability. Aortae were collected from six swine that were exposed to circulating Evans blue dye (EBD), a marker of transendothelial albumin permeability. Endothelial cells were scraped from (1) white regions in the thoracic aorta, (2) light blue streaks and blue regions near ostia in the thoracic aorta, and (3) dark blue regions in the aortic arch. Expression levels of several genes were analyzed by real-time quantitative PCR. There were modest differences between the expression levels of several genes in cells from the light blue regions relative to those from white regions. In the dark blue regions, eNOS was drastically downregulated and MCP-1 was upregulated relative to their expression in both the white and light blue regions. The distinct levels of permeability and differences in gene expression profiles exhibited by cells from these different regions of the aorta may reflect corresponding differences in their hemodynamic environments.     

Chronische Periaortitis

Chronic periaortitis is a rare fibroinflammatory disorder which affects the abdominal aorta and may spread into the retroperitoneum, often encasing the ureters. An aneurysma of the abdominal aorta and vasculitis of the thoracic aorta and of supra-aortic vessels may also coexist. Chronic periaortitis can be idiopathic or secondary to different triggers such as drugs, tumors and infections. Abdominal and/or low back pain is the hallmark symptom. Laboratory markers of inflammation are usually increased. The diagnosis rests on computerized tomography or magnetic resonance imaging, which typically show a retroperitoneal mass displacing the aorta anteriorly and the ureters medially. Positron-emission tomography may assist in defining disease activity and extension. Chronic periaortitis should be differentiated from other fibrosing disorders of various origins. Histology is required in atypical cases to secure the diagnosis. Treatment is based on high-dose steroids with a tapering scheme combined with immunosuppressive agents in refractory or relapsing disease. In case of ureter obstruction early DJ-catheter placement is required. Operative interventions to relieve ureter obstruction are rarely necessary provided immunosuppressive treatment is timely instituted.     

Diagnosis and follow up of aortitis in the elderly

OBJECTIVES: To evaluate the correlation of MRI and [(18)F]FDG-PET scans with the clinical course and inflammatory markers in patients with aortitis. METHODS: Eight patients with aortitis presenting with unspecific GCA-like symptoms were examined. Aortitis was diagnosed and followed up by [(18)F]FDG-PET and MRI. The aorta was divided into three vascular regions (ascending aorta, aortic arch, and descending aorta) to localise the aortic inflammation and compare both imaging techniques. RESULTS: were correlated with clinical and laboratory examinations. RESULTS: At diagnosis, 20/24 vascular regions from eight patients were positive by [(18)F]FDG-PET scan and 15/21 aortic regions by MRI. Patients were treated with corticosteroids and followed up for a mean (SD) of 13.3 (4.7) months. In [(18)F]FDG-PET, 11/20 (55%) initially pathological aortic regions returned to normal in the follow up examination, which correlated closely with the clinical and laboratory follow up examination. Conversely, in MRI, 14/15 initially affected vascular regions were unchanged. CONCLUSIONS: [(18)F]FDG-PET and MRI are both effective techniques for detecting early aortitis and have a high correlation with laboratory inflammatory measures. However, during the follow up examination, [(18)F]FDG-PET uptake decreased in line with the clinical symptoms and inflammatory serum markers, whereas MRI scans gave more static results.     

Aortitis

Inflammatory or noninfectious aortitis may be idiopathic or it may be part of a systemic autoimmune disease, such as Takayasu's arteritis, Beh?et's disease, or giant cell arteritis. At the acute stage, there is thickening of the aortic wall with dilatation of the aorta, more commonly in the thoracic aorta. If it involves the aortic root, there may be annuloaortic ectasia or aortic regurgitation. At a later stage, there may be aneurysmal dilatation of the aorta and rarely dissection or rupture of the aorta. In Takayasu's arteritis, stenosing lesions can occur as well as aneurysmal dilatation of the aorta or arteries. Stenosing lesions may be treated with angioplasty with or without stenting, whereas aneurysmal dilatation of the aorta is treated by aneurys-mectomy with arterial reconstruction or conduit. Severe aortic regurgitation may require aortic valve surgery with or without replacement of the ascending aorta. Irrespective of the interventional procedure undertaken as appropriate for the lesion, control of inflammation with steroid therapy with or without other immunosuppressive agents is of paramount importance. Otherwise, prosthetic valve or graft dehiscence may occur after aortic surgery, and restenosis rate is also higher after percutaneous transluminal angioplasty or stenting.     

Vaskulitiden der gro?en Gef??e

Large vessel vasculitis is characterized by local inflammation of the vessel wall of the aorta and its first and second degree branches. Giant cells are found regularly. The process leads to stenoses and occlusions as well as to aneurysmatic dilatation. Recent progress in vascular imaging (colour Doppler ultrasound, MRI and PET) allows to detect Takayasu arteritis at an earlier stage and to reveal the involvement of larger arteries in temporal arteritis (giant cell arteritis, GCA). With increasing frequency, a subtype of predominantly female and less aged GCA patients without cranial symptoms is noticed in whom obstructions are prevailing in the proximal arm vessels or in the aorta. The latter may be affected exclusively. Initial therapy is based on corticosteroids. In GCA, methotrexate hitherto is the only other immunosuppressive drug that has proved to be helpful. Disease activity has to be controlled both by laboratory parameters and vascular imaging.     

Ascending to descending aorta bypass for middle aortic syndrome.

Though stenoses of the descending aorta and its branches are seen with congenital anomalies or systemic inflammation, occlusion of the descending aorta is extremely rare. A patient with an occluded hypoplastic descending thoracic aorta required re-operation because of graft failure between the descending thoracic aorta and the infrarenal abdominal aorta. The etiology of the aortic occlusion in this case is unknown, but inflammation, such as Takayasu disease, is speculated.     

Correlation between 18-fluorodeoxyglucose accumulation in large vessels and serological markers of inflammation in polymyalgia rheumatica: a quantitative PET study

OBJECTIVE: To quantify 18-fluorodeoxyglucose (FDG) accumulation in large vessels in patients with polymyalgia rheumatica by positron emission tomography (PET), and to compare these data with serological markers of inflammation. METHODS: 13 untreated patients with active polymyalgia rheumatica underwent FDG positron emission tomography; eight were analysed in a second PET when in clinical remission. Six patients with other highly inflammatory conditions served as controls. For quantitative analysis, FDG uptake over nine defined vascular regions, divided by an individual background value, was expressed as a region of interest (ROI) index. These data were compared with the clinical status of the patient and with erythrocyte sedimentation rate (ESR), C reactive protein, haemoglobin, and platelet and leucocyte counts. RESULTS: By visual evaluation, 12 of the 13 patients showed an increased tracer uptake of the aorta or its major branches. By quantitative analysis, FDG uptake was significantly increased in polymyalgia rheumatica. In patients with active disease, the mean ROI index for all vascular regions exceeded that of controls by 70% (mean (SD): 1.58 (0.37) v 0.93 (0.12); p<0.001). In the eight patients who underwent follow up PET, the index declined substantially. In active polymyalgia rheumatica, FDG uptake was significantly correlated with C reactive protein (r = 0.8), ESR (r = 0.79), and platelet counts (r = 0.68). CONCLUSIONS: The observed FDG accumulation in the aorta and its branches and a strong correlation between tracer uptake and markers of inflammation is suggestive of large vessel arteritis. Quantitative ROI analysis appears to be a sensitive tool for detecting such inflammation.     

Cannulation and cardiopulmonary bypass produce selective brain lesions in pigs

Whether cardiopulmonary bypass alone or together with manipulation of the aorta produces neurologic complications remains controversial. Using a pig model, the immediate effects of aortic cannulation and cardiopulmonary bypass on neural injury in different brain regions were investigated in 3 experimental groups: non-operated controls; operated controls with aortic cannulation without cardiopulmonary bypass; and operated animals undergoing cardiopulmonary bypass. Immunohistochemistry using a monoclonal antibody against calretinin was used to show possible ischemic damage in the hippocampal formation which is one of the most vulnerable regions to ischemia. Both cannulation of the aorta alone and cardiopulmonary bypass resulted in numerous argyrophilic neurons in discrete regions of the prefrontal and cerebellar cortex. Decreased calretinin immunoreaction and a reduced number of calretinin-positive neurons were observed following aortic cannulation or cardiopulmonary bypass compared to the non-operated controls. This suggests that both cannulation of the aorta alone and cardiopulmonary bypass affect a selected population of neurons. Therefore, off-pump, aorta no-touch technique may prevent neurologic complications.     

Impaired endothelium-dependent relaxations in rabbits subjected to aortic coarctation hypertension

Rabbits were rendered hypertensive by suprarenal coarctation of the abdominal aorta. Seven days later, endothelium-dependent and endothelium-independent vascular relaxations were examined in vascular rings taken from hypertensive (thoracic aorta, carotid artery) and normotensive (abdominal aorta) regions. Relaxation of phenylephrine-contracted rings in response to endothelium-dependent agonists (acetylcholine, A23187) was impaired, compared with that in sham-operated and intact controls, in regions exposed to the elevated blood pressure (i.e., above the coarctation). Responses to acetylcholine and A23187 in the abdominal aorta, below the coarctation, were not altered. The diminished endothelium-dependent responses in the thoracic aorta were not affected by pretreatment with the cyclooxygenase inhibitor indomethacin. In contrast to acetylcholine and A23187, responses to the endothelium-independent agonist nitroprusside were not attenuated in vessels from hypertensive regions, indicating that the defect occurred in the endothelium. The EC50 for acetylcholine-induced relaxations of thoracic aorta correlated significantly with mean arterial pressure above the coarctation, indicating that the extent to which endothelium-dependent relaxation is impaired is in proportion to the degree of blood pressure elevation. This study suggests that the diminished relaxations by endothelium-dependent agonists is a local response to the elevation of blood pressure and is not due to a circulating factor.     

Characteristic change in local pulse wave velocity in different segments of the atherosclerotic aorta in KHC rabbits

BACKGROUND: Pulse wave velocity, conventionally determined between the carotid and femoral arteries, is a useful measure to estimate stiffness of the aorta. We investigated local pulse wave velocity (LPWV) in different segments in the aorta with relatively early-stage atherosclerosis in relation to the extent and severity of atherosclerotic lesions. METHODS: Pressure waves were recorded in eight aortic positions using two catheters with one or two micromanometers to determine LPWV in the ascending aorta, distal end of the aortic arch, proximal, middle, and distal thoracic aortas, and proximal, middle, and distal abdominal aortas in Kurosawa and Kusanagi-hypercholesterolemic (KHC) and normal rabbits aged 10 to 12 months. RESULTS: The LPWV in the KHC rabbit was greatest in the aortic arch, decreased almost to the normal level in the middle and distal thoracic aorta, increased in the proximal abdominal aorta, and showed almost identical change to that in the normal rabbit in the middle and distal abdominal aortic regions. There was significant difference in LPWV in the aortic arch, proximal thoracic, and proximal abdominal aortas between the two rabbit groups. The sclerotic lesion was prominent in the aortic arch, proximal thoracic aorta, and proximal abdominal aortas. The wall was severely thickened with abundant foam cells. The significant increase in LPWV would be mainly related to the increased wall thickness in these aortic regions. CONCLUSIONS: We can conclude that LPWV reflects well the distribution and severity of atherosclerotic lesion and the increased wall thickness in the local aortic region in which pulse waves were traveled.