肺血管炎的影像表现.ppt

1、Imaging Findings in Pulmonary Vasculitis,Semin Ultrasound CT MRI 33:567-579 2012,肺原发性血管炎少见，包括大血管炎（多发性大动脉炎(TA)和巨细胞动脉炎(GCA) ）抗中性粒细胞抗体相关性小血管炎（肉芽肿性多血管炎(GPA)、显微镜下多血管炎(MPA)、变应性肉芽肿血管炎），临床症状、体征与肺感染、结缔组织病和恶性肿瘤相似 肺血管炎的影响学征象多样，包括血管壁增厚、肺结节和空洞、磨玻璃影及实变等，须结合临床、影像、实验室及组织病理作出诊断 本文描述常累及肺的原发性血管炎的影像学和临床特征，还讨论了弥漫性肺泡出血DA

2、H的影像学特征，强调了影像和临床综合诊断的重要性,血管炎是指组织病理学上血管损伤为特征，在大多数病例中可继发其他疾病如：感染、结缔组织病、恶性肿瘤和高敏疾病也可以是这些疾病的一部分。原发性血管炎少见全部文献的发生率20-100例/百万，普遍认为150-450/百万 依据受侵及血管的大小是最常见的分类方法，有益于对临床和影像学特征的描述。在原发性大动脉炎（TA、GCA）和原发性ANCA相关性小血管炎（WG、MPA、CSS）常累及胸部。弥漫性肺泡出血是原发性肺血管炎的表现之一，同样也发生在其他情况如：特发性肺泡出血、胶原血管病、药物反应和抗凝血紊乱,Classication,几十年以来依照受累及血

3、管的大小一直是主要的分类依据。1994年Chapel Hill提出了依照血管的大小和实验室结果的更详细的命名 在Chiapel Hill分类中，大血管主动脉及其大的分支，中等血管指内脏血管如肾、肺、冠状、肠系膜血管，小血管指毛细血管和小动静脉 其他血清学实验有：抗中性粒细胞胞浆抗体ANCA和其它免疫标记物如血管免疫球蛋白A沉积物、血清冷球蛋白、抗肾小球基底膜抗体,ANCA相关性血管炎具有相同的临床、组织病理特征，ANCA 阳性以及对免疫抑制剂的类似反应性一组血管炎性疾病 ANCA是针对中性粒细胞和单核细胞内抗原的抗体,包含两种抗体：p-ANCA和c-ANCA 大血管炎可表现为局部缺血症状；小血

4、管炎的症状和体征常无特征性如：发热、关节肌肉痛、周身不适。当曾有过多系统器官症候群（肺泡出血、肾小球肾炎、上呼吸道病变、多发性神经炎、紫癜以及胸部发现结节和空洞），在ANCA相关性血管炎非常常见,Clinical and RadiologicFindings Suggestive of Vasculitis,大血管炎包括多发性大动脉炎(TA)和巨细胞动脉炎(GCA)； 中血管炎包括结节性多动脉炎(PAN)和皮肤黏膜淋巴结综合征； 小血管炎包括肉芽肿性多血管炎(GPA)、显微镜下多血管炎(MPA)、变应性肉芽肿血管炎、过敏性紫癜(HSP)、原发性冷球蛋白血症、皮肤白细胞破碎性血管炎。 其他自身免

5、疫疾病相关的血管炎如系统性红斑狼疮(SLE)、白塞病等血管炎未被列入ChapelHill的分类，也可能累及肺脏。,Large-Vessel Vasculitis,大动脉炎主要侵及主动脉及大分支，主要症状为局部缺血表现 TA和GCA是最常见的大动脉炎 由于Behet disease在主动脉有相似的发现， 故也在此表述 TA几乎仅侵犯40岁以下年轻人，主要是主动脉及主要分支，其次是颅内动脉 GCA,Takayasu Arteritis多发性大动脉炎,世界范围均有发现，但在亚洲很常见。发病通常为年青女性，发生率0.12-0.26/ 100000 TA专门累及主动脉弓及分支 TA是以动脉内、外膜的增殖

6、和纤维化为特征的肉芽肿性炎症，常导致血管腔狭窄、扩张和动脉瘤形成 临床分早期和晚期。早期或发病前表现非系统性特征如：低热不适、疲劳及体重下降。少数病例有经典三阶段即：血管炎阶段、纤维变性阶段和动脉闭塞阶段 TA常复发，表现为各个阶段共存,晚期表现通常为血管狭窄所致：脉搏减弱或消失（96%），典型症候为间歇性跛行和血压的差异。缓慢进展可引发侧枝循环建立，症候更加隐匿 由于临床和实验室无特异性，TA的准确诊断事实上依靠影像学检查 CT和MRI均可显示早期的血管壁增厚、血管腔变窄、瘤样扩张和纤维变性期及晚期的闭塞。在活跃期， CT和MRI均可增强 肺动脉受累发生较晚，主要肺段动脉，而叶动脉和主肺动脉

7、不常见。CT表现包括早期病变的增厚和增强，慢性阶段的管壁钙沉积和管腔狭窄、闭塞,Figure 2 TA in a 35-year-old woman, presenting with diminished left arm pulse and left arm weakness. Contrast-enhanced CT shows concentric wall thickening of thesupra-aortic trunks with obliteration of left subclavian artery . (B) Sagittal-reformatted image sh

8、ows diffuse wall thickening of the thoracic and abdominal aorta .There is occlusion of the left subclavian artery , ostial occlusion of the celiac trunk, and severe stenosis of the mesenteric artery . Note the variations in aortic diameter, with some stenosis and dilations in the abdominal aorta.,Fi

9、gure 3 TA in a 32-year-old asymptomatic woman. MRA with maximum intensity projection reconstructions shows complete occlusion of both prevertebral subclavianarteries (arrowheads); there is collateral ow from the right verteb- ral artery and left cervical artery (white arrows). Both common carotid ar

10、teries are patent. A fusiform aneurysm of the thoracic descending aorta is also seen.,Figure 4 Late-stage TA with right pulmonary artery involvement in a 63-year-old woman. Contrast-enhanced CT scan showsmarked stenosis of the right pulmonary artery (arrow).,Giant Cell Arteritis,GCA常侵及50岁以上成人的大中血管，尤

11、其是颅外颈动脉及分支和主动脉，肺动脉少见。病理上GCA比TA少见 GCA主要侵及颞动脉、视神经和视网膜动脉，典型症状如捏动脉减弱和肿胀、短暂头痛、偏盲和视力下降。超过一半病人有如下全身肌肉骨骼系统症状中的一种或多种组合： 乏力、体重下降、低热、风湿性多发肌肉疼痛、关节痛和腱鞘炎 颅外GCA最常见于主动脉弓和锁骨下和腋动脉。有或无典型颞动脉炎及无典型症候的GCA易误诊为动脉粥样硬化性疾病,CT和MRI主要表现与TA相似：动脉壁增厚、狭窄和动脉瘤；主动脉GCA早期无症状，晚期造成严重的并发症如：动脉瘤及其破裂 与动脉硬化不同，GCA动脉瘤常发生在胸主动脉并更倾向破裂 PET有利于显示活动性病变和随

12、访。因为GCA病人年长且常并发动脉粥样硬化，故诊断较TA更困难,Behet Disease,Behet Disease 以临床表现为复发性口腔溃疡、生殖器溃疡和眼葡萄膜炎三联征为代表的多系统血管炎。还可以累及关节、胃肠道、中枢神经系统、心血管系统、和肺 通常20-40岁，男女比例相等，胸部受累报道1-8%，血管系统受累25-30%，也是死亡的常见原因 Behet Disease动脉瘤呈梭形囊袋状，通常局限在双下肺动脉或主肺动脉，肺动脉瘤常伴有部分或完全性血栓形成 肺动脉瘤直接导致高死亡率（2年内提高到30%，咳血发生后平均10个月） 免疫抑制剂治疗对75%动脉瘤的完全有效,血管炎的管壁增厚也见

13、于主动脉和上腔静脉窦，Behet Disease的上腔静脉窦及纵膈静脉血栓相当常见 心内血栓位于右心，常伴有肺动脉血栓、肺静脉血栓及心内膜纤维变性 肺实质病变为胸膜下肺泡浸润和楔形或边缘模糊的类圆形密度增高影，提示肺内灶性血管炎以及血栓形成导致的梗死、出血和灶性肺不张,Figure 5 A 67-year-old woman with giant cell arteritis who presented with headaches and visual loss. Contrast-enhanced CT shows concentric wall thickening and dilati

14、on (arrows) of the ascending and descending thoracic aorta (A) and abdominal aorta (B).,Figure 6 A 26-year-old woman with Behet disease who presented with dyspnea. (A, B) Contrast-enhanced CT images showing increased diameter of both interlobar and lower lobe pulmonary arteries; the aneurysm is part

15、ially thrombosed on the right (arrow) and completely thrombosed on the left (asterisk).,(C) Contrast-enhanced CT image obtained 6 months after immunosuppressive treatment show resolution of the right interlobar pulmonary artery aneurysm and decrease in size of the left interlobar pulmonary artery de

16、void of contrast (arrow) because of organization of the thrombus and evolution toward chronic pulmonary thromboembolism. (D) Contrast-enhanced CT showing a thrombus in the right atrium (asterisk).,(E) Lung window setting CT image showing wedge-shaped subpleural opacities suggestive of pulmonary infa

17、rction associated with pulmonary thromboembolism,Small-Vessel Vasculitis,虽然小血管炎定义在小动脉、静脉和毛细血管，也可在中大动脉发生 肺部受累在ANCA相关性小血管炎很常见（WG、CSS和MPA）全部年龄段均可受累， 但最多见于50-60岁成人，男略多于女,Wegener Granulomatosis,WG是最常见的ANCA相关性血管炎，几乎所有病例都有上呼吸道症状，大多数累及肺（90%）、肾（80%） 典型的临床三联征包括上呼吸道：鼻窦炎、耳炎、鼻粘膜溃疡、骨缺损和声门下狭窄； 下呼吸道：咳嗽、胸痛和咳血；和肾小球肾炎

18、 初始阶段无典型三联征,Nodules, Masses, and Consolidation,超过90%患者影像学最常见的表现是肺结节和肿块 组织病理学上活动期肺结节和肿块为肉芽肿性炎症。病变融合坏死并形成空洞的趋势是WG的特征 结节和肿块常多发、于双肺胸膜下而不是支气管血管树区域，上下肺无偏爱。边缘可光滑，不规则少见 结节和肿块随着病变的进展增多、增大可融合，直径由几毫米至大于10cm；在CT上2cm的结节多形成空洞 厚壁空洞的内缘不规则，治疗后可变小或变成薄壁,Figure 7 A 57-year-old man with Wegener granulomatosis (WG) who p

19、resented with malaise, recurrent episodes of epistaxis, and shortness of breath. Posteroanterior chest radiograph shows well-dened multiple bilateral nodules predominantly affecting the upper lobes, some of which cavitated, and some with an air-uid level secondary to infection,Figure 8 Schematic rep

20、resentation of the 3 major histologic features that characterize WG: (1) vasculitis with inammation of medium-sized and small vessels, frequently located within inammatory nodules. Neutrophilic inltration and microabscess formation (black arrow) may be present. (2) Areas of necrosis (white arrow). (

21、3) Necrotizing and non-necrotizing granulomatous inammation (arrowhead),Figure 9 WG in a 34-year-old woman with pulmonary masses and nodules, progressive renal failure, and dysphonia. (A) CT image (lung window) shows an irregular thick-walled cavitated mass in the right upper lobe and a better-dened

22、 cavitated nodule in the left upper lobe (arrow). The mass in the right upper lobe is surrounded by a halo of ground-glass attenuation caused by hemorrhage. (B) Late contrast-enhanced CT shows a mass in the right lower lobe with low attenuation and small cavities because of central necrosis and a ri

23、m of peripheral enhancement (arrowheads). (C) CT image shows circumferential tracheal wall thickening (arrows) in the upper thoracic trachea.,Figure 10 Same patient as in Figure 7, after immunosuppressive treatment. (A) CT image (lung window) shows a cavitated mass in the right upper lobe, a well-de

24、ned nodule in the left lower lobe, and a scarring lesion in the left upper lobe. (B) CT image (lung window) obtained 1 year later shows a healing residual lesion from the cavity of the right upper lobe. The well-dened nodule in the left lower lobe and scarring lesion in the left upper lobe are stabl

25、e.,Figure 11 WG in a 56-year-old woman who presented with malaise and chronic sinusitis. (A, B), CT images (lung window) show patchy small ill-dened nodules (arrowheads), some with air bronchogram; in (A) note the marked bronchial wall thickening in the right upper lobe bronchi (arrows) and right ma

26、in bronchus.,在CT上约15%的病例见到晕征，为出血所致；增强CT上多数无空洞的结节或肿块中心呈低密度区、伴或不伴周边强化。治疗后大约50%的肿块/结节分解，40%变小，10%无变化 其次的影像学发现（20-50%病例）气腔实变和斑片状磨玻璃影，可伴/不伴肺结节和肿块，既反映了肺血管炎性病变中的局限性肺炎也反映肺泡出血,肺实变表现为随机分布的类似肺栓塞的肺外周楔形阴影，也可沿支气管血管树分布 双肺弥漫性磨玻璃样影提示肺泡出血（10%）,以胸膜下结节和肿块为主WG的影像学鉴别诊断包括感染（脓毒栓塞，多发脓肿）、肿瘤（血性转移瘤、淋巴瘤）和机化性肺炎；沿支气管血管树分布的为主病变要与K

27、aposi sarcoma鉴别 WG肿块和结节变化快是与恶性肿瘤的明显区别；上呼吸道症状、化验提示肾小球肾炎和血清c-ANCA阳性（活动期90%)可排除鉴别,ChurgStrauss Syndrome ,以哮喘、嗜酸性粒细胞增多和坏死性血管炎为三联征。下列6项中超过4项应诊断CSS: 哮喘 外周血嗜酸性粒细胞10 系统性血管炎引起的单/多神经病变 游走性肺阴影 鼻窦炎 活检标本血管外嗜酸性细胞增多,迟发哮喘（平均32岁）是CSS与普通哮喘的区别，肺是最常受累的器官，其次是皮肤；肺出血和肾小球肾炎较其他的小血管炎疾病少见 心脏是CSS主要器官，冠脉炎和心肌炎是主要死因 组织病理学表现坏死性小血管

28、炎和伴有坏死性肉芽肿的嗜酸性粒细胞性炎症,CSS最常见的影像学表现为类似单纯性嗜酸性细胞肺炎的双侧游走性、非肺段分布、无区域偏好的实变影，或与慢性嗜酸性肺炎或机化性肺炎相似的肺外周实变影 高达90%的CT有双肺外周对称分布的磨玻璃影或实变影，50%病人可见线状小叶间隔增厚，提示心脏受累引起的肺水肿或小叶间隔的嗜酸性细胞浸润,提示与哮喘有关的气道受累征象包括小叶中心结节、树芽征、支气管扩张、支气管和细支气管壁增厚 10-50%的病例CT可见单/双侧胸腔积液，提示心肌炎导致的左心衰或嗜酸性胸膜炎 哮喘伴有以肺外周分布为主的实变影时，应考虑特发性嗜酸性肺炎、CSS和机化性肺炎 依靠系统性损害如皮疹、

29、外周神经病变和p-ANCA阳性（活动期大约35-70%）做出CSS诊断,Figure 12 Schematic representation of themain histologic features found in ChurgStrauss syndrome. (A) The small box shows a normal secondary pulmonary lobule with the bronchus (blue structure in the online version) and the artery (red structure in the online versi

30、on) in the middle; the white dots represent the alveoli. In the prodromal stage隐匿期, bronchiolitis with eosinophilic and neutrophilic inltration of the bronchial wall (black arrow) and septal inltration by eosinophils (black arrowhead) can be seen.,(B) Eosinophilic inltration in the alveoli; black ar

31、row points to an inltrated alveolus. Once the vasculitic phase is established, granulomatous necrosis of medium-sized arteries, veins，and capillaries is apparent.血管炎期，小叶中央动、静脉和毛细血管可见到肉芽肿型坏死 Extravascular granulomas (black arrowheads), brinoid necrosis (white arrow), and thrombosis (white arrowhead)

32、are common ndings常见血管外肉芽肿、纤维素样坏死和血栓,Figure 13 ChurgStrauss syndrome in a 38-year-old woman with asthma diagnosed 7 years before who presented with a 2-month history of fever and cough. She had a history of persistent eosinophilia and sinusitis. 有过嗜酸性细胞增多症和鼻窦炎表现 (A) Chest radiograph shows bilateral p

33、atchy perihilar and basal consolidation.双肺门周围和下肺斑片状影,(B, C) CT images (lung window) demonstrate patchy bilateral areas of consolidation; some are distributed in the periphery and some (arrowheads) along the bronchovascular bundles. Note the bronchial wall thickening (arrows in B). Thickening of the

34、interlobular septa is seen in (C) (arrows).,Microscopic Polyangiitis,MPA是非肉芽肿性系统性血管炎，是引起肺-肾综合征的最常见原因。临床最常累及肾，其次是肺。超过90%病人有快速进展的肾小球肾炎，仅10-30%出现肺泡出血，病理上为肺毛细血管炎 胸部症状有咳血、呼吸急促，其他相关表现有皮疹、末梢神经炎和胃肠道出血 快速进展的肾小球肾炎、p-ANCA阳性（40-80%） 以及肺泡出血的临床和影像学表现应考虑MPA,Figure 14 Diffuse pulmonary hemorrhage in a 62-year-old m

35、an with coagulation disorder凝血障碍 and respiratory failure. Chest radiograph shows diffuse bilateral areas of consolidation. Note the endobronchial and nasogastric tube.,Figure 15 Variety of CT patterns in diffuse alveolar hemorrhage. (A) Microscopic polyangiitis in 43-year-old man showing patchy areas of ground-glass opacities. (B) WG in a 62-year-old woman: CT shows multiple pulmonary nodules (asterisks) coexisting with extensive areas of consolidation.,(C) Systemic lupus erythematosus in a 35-year-old patient. CT shows diffuse ground-glass opacities, ill-dened c