肺血管炎的影像表現(xiàn)培訓課件

1、肺血管炎的影像表現(xiàn)肺血管炎的影像表現(xiàn)肺原發(fā)性血管炎少見，包括大血管炎（多發(fā)性大動脈炎(TA)和巨細胞動脈炎(GCA) ）抗中性粒細胞抗體相關(guān)性小血管炎（肉芽腫性多血管炎(GPA)、顯微鏡下多血管炎(MPA)、變應性肉芽腫血管炎），臨床癥狀、體征與肺感染、結(jié)締組織病和惡性腫瘤相似肺血管炎的影響學征象多樣，包括血管壁增厚、肺結(jié)節(jié)和空洞、磨玻璃影及實變等，須結(jié)合臨床、影像、實驗室及組織病理作出診斷本文描述常累及肺的原發(fā)性血管炎的影像學和臨床特征，還討論了彌漫性肺泡出血DAH的影像學特征，強調(diào)了影像和臨床綜合診斷的重要性2肺血管炎的影像表現(xiàn)肺原發(fā)性血管炎少見，包括大血管炎（多發(fā)性大動脈炎(TA)和巨血

2、管炎是指組織病理學上血管損傷為特征，在大多數(shù)病例中可繼發(fā)其他疾病如：感染、結(jié)締組織病、惡性腫瘤和高敏疾病也可以是這些疾病的一部分。原發(fā)性血管炎少見全部文獻的發(fā)生率20-100例/百萬，普遍認為150-450/百萬依據(jù)受侵及血管的大小是最常見的分類方法，有益于對臨床和影像學特征的描述。在原發(fā)性大動脈炎（TA、GCA）和原發(fā)性ANCA相關(guān)性小血管炎（WG、MPA、CSS）常累及胸部。彌漫性肺泡出血是原發(fā)性肺血管炎的表現(xiàn)之一，同樣也發(fā)生在其他情況如：特發(fā)性肺泡出血、膠原血管病、藥物反應和抗凝血紊亂3肺血管炎的影像表現(xiàn)血管炎是指組織病理學上血管損傷為特征，在大多數(shù)病例中可繼發(fā)其Classication

3、幾十年以來依照受累及血管的大小一直是主要的分類依據(jù)。1994年Chapel Hill提出了依照血管的大小和實驗室結(jié)果的更詳細的命名在Chiapel Hill分類中，大血管主動脈及其大的分支，中等血管指內(nèi)臟血管如腎、肺、冠狀、腸系膜血管，小血管指毛細血管和小動靜脈其他血清學實驗有：抗中性粒細胞胞漿抗體ANCA和其它免疫標記物如血管免疫球蛋白A沉積物、血清冷球蛋白、抗腎小球基底膜抗體4肺血管炎的影像表現(xiàn)Classication幾十年以來依照受累及血管的大小一直ANCA相關(guān)性血管炎具有相同的臨床、組織病理特征，ANCA 陽性以及對免疫抑制劑的類似反應性一組血管炎性疾病ANCA是針對中性粒細胞和單核細

4、胞內(nèi)抗原的抗體,包含兩種抗體：p-ANCA和c-ANCA大血管炎可表現(xiàn)為局部缺血癥狀；小血管炎的癥狀和體征常無特征性如：發(fā)熱、關(guān)節(jié)肌肉痛、周身不適。當曾有過多系統(tǒng)器官癥候群（肺泡出血、腎小球腎炎、上呼吸道病變、多發(fā)性神經(jīng)炎、紫癜以及胸部發(fā)現(xiàn)結(jié)節(jié)和空洞），在ANCA相關(guān)性血管炎非常常見Clinical and RadiologicFindings Suggestive of Vasculitis5肺血管炎的影像表現(xiàn)ANCA相關(guān)性血管炎具有相同的臨床、組織病理特征，ANCA 6肺血管炎的影像表現(xiàn)6肺血管炎的影像表現(xiàn)7肺血管炎的影像表現(xiàn)7肺血管炎的影像表現(xiàn)大血管炎包括多發(fā)性大動脈炎(TA)和巨細胞動

5、脈炎(GCA)；中血管炎包括結(jié)節(jié)性多動脈炎(PAN)和皮膚黏膜淋巴結(jié)綜合征；小血管炎包括肉芽腫性多血管炎(GPA)、顯微鏡下多血管炎(MPA)、變應性肉芽腫血管炎、過敏性紫癜(HSP)、原發(fā)性冷球蛋白血癥、皮膚白細胞破碎性血管炎。其他自身免疫疾病相關(guān)的血管炎如系統(tǒng)性紅斑狼瘡(SLE)、白塞病等血管炎未被列入ChapelHill的分類，也可能累及肺臟。8肺血管炎的影像表現(xiàn)大血管炎包括多發(fā)性大動脈炎(TA)和巨細胞動脈炎(GCA)；Large-Vessel Vasculitis大動脈炎主要侵及主動脈及大分支，主要癥狀為局部缺血表現(xiàn)TA和GCA是最常見的大動脈炎由于Behet disease在主動脈

6、有相似的發(fā)現(xiàn)， 故也在此表述TA幾乎僅侵犯40歲以下年輕人，主要是主動脈及主要分支，其次是顱內(nèi)動脈GCA9肺血管炎的影像表現(xiàn)Large-Vessel Vasculitis大動脈炎主要侵Takayasu Arteritis多發(fā)性大動脈炎世界范圍均有發(fā)現(xiàn)，但在亞洲很常見。發(fā)病通常為年青女性，發(fā)生率0.12-0.26/ 100000TA專門累及主動脈弓及分支TA是以動脈內(nèi)、外膜的增殖和纖維化為特征的肉芽腫性炎癥，常導致血管腔狹窄、擴張和動脈瘤形成臨床分早期和晚期。早期或發(fā)病前表現(xiàn)非系統(tǒng)性特征如：低熱不適、疲勞及體重下降。少數(shù)病例有經(jīng)典三階段即：血管炎階段、纖維變性階段和動脈閉塞階段TA常復發(fā)，表現(xiàn)為

7、各個階段共存10肺血管炎的影像表現(xiàn)Takayasu Arteritis多發(fā)性大動脈炎世界范圍均晚期表現(xiàn)通常為血管狹窄所致：脈搏減弱或消失（96%），典型癥候為間歇性跛行和血壓的差異。緩慢進展可引發(fā)側(cè)枝循環(huán)建立，癥候更加隱匿由于臨床和實驗室無特異性，TA的準確診斷事實上依靠影像學檢查CT和MRI均可顯示早期的血管壁增厚、血管腔變窄、瘤樣擴張和纖維變性期及晚期的閉塞。在活躍期， CT和MRI均可增強肺動脈受累發(fā)生較晚，主要肺段動脈，而葉動脈和主肺動脈不常見。CT表現(xiàn)包括早期病變的增厚和增強，慢性階段的管壁鈣沉積和管腔狹窄、閉塞11肺血管炎的影像表現(xiàn)晚期表現(xiàn)通常為血管狹窄所致：脈搏減弱或消失（96%

8、），典型癥12肺血管炎的影像表現(xiàn)12肺血管炎的影像表現(xiàn)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 shows diffuse w

9、all 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.13肺血管炎的影像表現(xiàn)Figur

10、e 2 TA in a 35-year-old wFigure 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 arrow

11、s). Both common carotid arteries are patent. A fusiform aneurysm of the thoracic descending aorta is also seen.14肺血管炎的影像表現(xiàn)Figure 3 TA in a 32-year-old aFigure 4 Late-stage TA with right pulmonary artery involvement in a63-year-old woman. Contrast-enhanced CT scan showsmarked stenosis of the right pu

12、lmonary artery (arrow). 15肺血管炎的影像表現(xiàn)Figure 4 Late-stage TA with riGiant Cell ArteritisGCA常侵及50歲以上成人的大中血管，尤其是顱外頸動脈及分支和主動脈，肺動脈少見。病理上GCA比TA少見GCA主要侵及顳動脈、視神經(jīng)和視網(wǎng)膜動脈，典型癥狀如捏動脈減弱和腫脹、短暫頭痛、偏盲和視力下降。超過一半病人有如下全身肌肉骨骼系統(tǒng)癥狀中的一種或多種組合： 乏力、體重下降、低熱、風濕性多發(fā)肌肉疼痛、關(guān)節(jié)痛和腱鞘炎顱外GCA最常見于主動脈弓和鎖骨下和腋動脈。有或無典型顳動脈炎及無典型癥候的GCA易誤診為動脈粥樣硬化性疾病16肺

13、血管炎的影像表現(xiàn)Giant Cell ArteritisGCA常侵及50歲以CT和MRI主要表現(xiàn)與TA相似：動脈壁增厚、狹窄和動脈瘤；主動脈GCA早期無癥狀，晚期造成嚴重的并發(fā)癥如：動脈瘤及其破裂與動脈硬化不同，GCA動脈瘤常發(fā)生在胸主動脈并更傾向破裂PET有利于顯示活動性病變和隨訪。因為GCA病人年長且常并發(fā)動脈粥樣硬化，故診斷較TA更困難17肺血管炎的影像表現(xiàn)CT和MRI主要表現(xiàn)與TA相似：動脈壁增厚、狹窄和動脈瘤；主Behet DiseaseBehet Disease 以臨床表現(xiàn)為復發(fā)性口腔潰瘍、生殖器潰瘍和眼葡萄膜炎三聯(lián)征為代表的多系統(tǒng)血管炎。還可以累及關(guān)節(jié)、胃腸道、中樞神經(jīng)系統(tǒng)、心血

14、管系統(tǒng)、和肺通常20-40歲，男女比例相等，胸部受累報道1-8%，血管系統(tǒng)受累25-30%，也是死亡的常見原因Behet Disease動脈瘤呈梭形囊袋狀，通常局限在雙下肺動脈或主肺動脈，肺動脈瘤常伴有部分或完全性血栓形成肺動脈瘤直接導致高死亡率（2年內(nèi)提高到30%，咳血發(fā)生后平均10個月）免疫抑制劑治療對75%動脈瘤的完全有效18肺血管炎的影像表現(xiàn)Behet DiseaseBehet Disease 以血管炎的管壁增厚也見于主動脈和上腔靜脈竇，Behet Disease的上腔靜脈竇及縱膈靜脈血栓相當常見心內(nèi)血栓位于右心，常伴有肺動脈血栓、肺靜脈血栓及心內(nèi)膜纖維變性肺實質(zhì)病變?yōu)樾啬は路闻萁櫤?/p>

15、楔形或邊緣模糊的類圓形密度增高影，提示肺內(nèi)灶性血管炎以及血栓形成導致的梗死、出血和灶性肺不張19肺血管炎的影像表現(xiàn)血管炎的管壁增厚也見于主動脈和上腔靜脈竇，Behet DiFigure 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 dilation (arrows) of the ascending and descending thora

16、cic aorta (A) and abdominal aorta (B).20肺血管炎的影像表現(xiàn)Figure 5 A 67-year-old woman wFigure 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 partially thr

17、ombosed on the right (arrow) and completely thrombosed on the left (asterisk).21肺血管炎的影像表現(xiàn)Figure 6 A 26-year-old woman w(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

18、 interlobar pulmonary artery devoid of contrast (arrow) because of organization of thethrombus and evolution toward chronic pulmonary thromboembolism. (D) Contrast-enhanced CT showing a thrombus in the right atrium (asterisk). 22肺血管炎的影像表現(xiàn)(C) Contrast-enhanced CT image(E) Lung window setting CT image

19、 showing wedge-shaped subpleural opacities suggestive of pulmonary infarction associated with pulmonary thromboembolism23肺血管炎的影像表現(xiàn)(E) Lung window setting CT imaSmall-Vessel Vasculitis雖然小血管炎定義在小動脈、靜脈和毛細血管，也可在中大動脈發(fā)生肺部受累在ANCA相關(guān)性小血管炎很常見（WG、CSS和MPA）全部年齡段均可受累， 但最多見于50-60歲成人，男略多于女24肺血管炎的影像表現(xiàn)Small-Vessel Va

20、sculitis雖然小血管炎定Wegener GranulomatosisWG是最常見的ANCA相關(guān)性血管炎，幾乎所有病例都有上呼吸道癥狀，大多數(shù)累及肺（90%）、腎（80%）典型的臨床三聯(lián)征包括上呼吸道：鼻竇炎、耳炎、鼻粘膜潰瘍、骨缺損和聲門下狹窄； 下呼吸道：咳嗽、胸痛和咳血；和腎小球腎炎初始階段無典型三聯(lián)征25肺血管炎的影像表現(xiàn)Wegener GranulomatosisWG是最常見的ANodules, Masses, and Consolidation超過90%患者影像學最常見的表現(xiàn)是肺結(jié)節(jié)和腫塊組織病理學上活動期肺結(jié)節(jié)和腫塊為肉芽腫性炎癥。病變?nèi)诤蠅乃啦⑿纬煽斩吹内厔菔荳G的特征結(jié)節(jié)

21、和腫塊常多發(fā)、于雙肺胸膜下而不是支氣管血管樹區(qū)域，上下肺無偏愛。邊緣可光滑，不規(guī)則少見結(jié)節(jié)和腫塊隨著病變的進展增多、增大可融合，直徑由幾毫米至大于10cm；在CT上2cm的結(jié)節(jié)多形成空洞厚壁空洞的內(nèi)緣不規(guī)則，治療后可變小或變成薄壁26肺血管炎的影像表現(xiàn)Nodules, Masses, and ConsolidaFigure 7 A 57-year-old man with Wegener granulomatosis (WG) who presented with malaise, recurrent episodes of epistaxis, and shortness of breath

22、. 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 infection27肺血管炎的影像表現(xiàn)Figure 7 A 57-year-old man witFigure 8 Schematic representation of the 3 major histologic f

23、eatures 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).(3) Necrotizing and non-necrotizing granu

24、lomatous inammation (arrowhead) 28肺血管炎的影像表現(xiàn)Figure 8 Schematic representatFigure 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-dene

25、d 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 r

26、im of peripheral enhancement (arrowheads). (C) CT image shows circumferential tracheal wall thickening (arrows) in the upper thoracic trachea. 29肺血管炎的影像表現(xiàn)Figure 9 WG in a 34-year-old wFigure 10 Same patient as in Figure 7, after immunosuppressive treatment. (A) CT image (lung window) shows a cavitat

27、ed mass in the right upper lobe, a well-dened 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 scarri

28、ng lesion in the left upper lobe are stable.30肺血管炎的影像表現(xiàn)Figure 10 Same patient as in FFigure 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 mark

29、ed bronchial wall thickening in the right upper lobe bronchi (arrows) and right main bronchus.31肺血管炎的影像表現(xiàn)Figure 11 WG in a 56-year-old 在CT上約15%的病例見到暈征，為出血所致；增強CT上多數(shù)無空洞的結(jié)節(jié)或腫塊中心呈低密度區(qū)、伴或不伴周邊強化。治療后大約50%的腫塊/結(jié)節(jié)分解，40%變小，10%無變化其次的影像學發(fā)現(xiàn)（20-50%病例）氣腔實變和斑片狀磨玻璃影，可伴/不伴肺結(jié)節(jié)和腫塊，既反映了肺血管炎性病變中的局限性肺炎也反映肺泡出血32肺血管炎的影像表現(xiàn)在C

30、T上約15%的病例見到暈征，為出血所致；增強CT上多數(shù)無肺實變表現(xiàn)為隨機分布的類似肺栓塞的肺外周楔形陰影，也可沿支氣管血管樹分布雙肺彌漫性磨玻璃樣影提示肺泡出血（10%）33肺血管炎的影像表現(xiàn)肺實變表現(xiàn)為隨機分布的類似肺栓塞的肺外周楔形陰影，也可沿支氣以胸膜下結(jié)節(jié)和腫塊為主WG的影像學鑒別診斷包括感染（膿毒栓塞，多發(fā)膿腫）、腫瘤（血性轉(zhuǎn)移瘤、淋巴瘤）和機化性肺炎；沿支氣管血管樹分布的為主病變要與Kaposi sarcoma鑒別WG腫塊和結(jié)節(jié)變化快是與惡性腫瘤的明顯區(qū)別；上呼吸道癥狀、化驗提示腎小球腎炎和血清c-ANCA陽性（活動期90%)可排除鑒別34肺血管炎的影像表現(xiàn)以胸膜下結(jié)節(jié)和腫塊為主W

31、G的影像學鑒別診斷包括感染（膿毒栓塞35肺血管炎的影像表現(xiàn)35肺血管炎的影像表現(xiàn)ChurgStrauss Syndrome 以哮喘、嗜酸性粒細胞增多和壞死性血管炎為三聯(lián)征。下列6項中超過4項應診斷CSS:哮喘外周血嗜酸性粒細胞10系統(tǒng)性血管炎引起的單/多神經(jīng)病變游走性肺陰影鼻竇炎活檢標本血管外嗜酸性細胞增多36肺血管炎的影像表現(xiàn)ChurgStrauss Syndrome 以哮喘、遲發(fā)哮喘（平均32歲）是CSS與普通哮喘的區(qū)別，肺是最常受累的器官，其次是皮膚；肺出血和腎小球腎炎較其他的小血管炎疾病少見心臟是CSS主要器官，冠脈炎和心肌炎是主要死因組織病理學表現(xiàn)壞死性小血管炎和伴有壞死性肉芽腫的嗜

32、酸性粒細胞性炎癥37肺血管炎的影像表現(xiàn)遲發(fā)哮喘（平均32歲）是CSS與普通哮喘的區(qū)別，肺是最常受累肺血管炎的影像表現(xiàn)培訓課件提示與哮喘有關(guān)的氣道受累征象包括小葉中心結(jié)節(jié)、樹芽征、支氣管擴張、支氣管和細支氣管壁增厚10-50%的病例CT可見單/雙側(cè)胸腔積液，提示心肌炎導致的左心衰或嗜酸性胸膜炎哮喘伴有以肺外周分布為主的實變影時，應考慮特發(fā)性嗜酸性肺炎、CSS和機化性肺炎依靠系統(tǒng)性損害如皮疹、外周神經(jīng)病變和p-ANCA陽性（活動期大約35-70%）做出CSS診斷39肺血管炎的影像表現(xiàn)提示與哮喘有關(guān)的氣道受累征象包括小葉中心結(jié)節(jié)、樹芽征、支氣管Figure 12 Schematic represen

33、tation 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 version) in the middle; the white dots represent the alveoli. In the

34、prodromal stage隱匿期, bronchiolitis with eosinophilic and neutrophilic inltration of the bronchial wall (black arrow) and septal inltrationby eosinophils (black arrowhead) can be seen. 40肺血管炎的影像表現(xiàn)Figure 12 Schematic representa(B) Eosinophilic inltration in the alveoli; black arrow points to aninltrate

35、d alveolus. Once the vasculitic phase is established, granulomatous necrosis of medium-sized arteries, veins，and capillaries is apparent.血管炎期，小葉中央動、靜脈和毛細血管可見到肉芽腫型壞死Extravascular granulomas (black arrowheads), brinoid necrosis (white arrow), and thrombosis (white arrowhead) are common ndings常見血管外肉芽腫、

36、纖維素樣壞死和血栓41肺血管炎的影像表現(xiàn)(B) Eosinophilic inltration iFigure 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. 有過嗜酸性細胞增多癥和鼻竇炎表現(xiàn)(A) Chest radiograph shows

37、 bilateral patchy perihilar and basal consolidation.雙肺門周圍和下肺斑片狀影42肺血管炎的影像表現(xiàn)Figure 13 ChurgStrauss syndro(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

38、 wall thickening (arrows in B). Thickening of the interlobular septa is seen in (C) (arrows).43肺血管炎的影像表現(xiàn)(B, C) CT images (lung window)Microscopic PolyangiitisMPA是非肉芽腫性系統(tǒng)性血管炎，是引起肺-腎綜合征的最常見原因。臨床最常累及腎，其次是肺。超過90%病人有快速進展的腎小球腎炎，僅10-30%出現(xiàn)肺泡出血，病理上為肺毛細血管炎胸部癥狀有咳血、呼吸急促，其他相關(guān)表現(xiàn)有皮疹、末梢神經(jīng)炎和胃腸道出血快速進展的腎小球腎炎、p-ANCA陽性（

39、40-80%） 以及肺泡出血的臨床和影像學表現(xiàn)應考慮MPA44肺血管炎的影像表現(xiàn)Microscopic PolyangiitisMPA是非肉Figure 14 Diffuse pulmonary hemorrhage in a 62-year-old man withcoagulation disorder凝血障礙 and respiratory failure. Chest radiograph shows diffuse bilateral areas of consolidation. Note the endobronchial and nasogastric tube.45肺血管炎的

40、影像表現(xiàn)Figure 14 Diffuse pulmonary heFigure 15 Variety of CT patterns in diffuse alveolar hemorrhage. (A) Microscopic polyangiitis in 43-year-old man showing patchyareas of ground-glass opacities. (B) WG in a 62-year-old woman: CT shows multiple pulmonarynodules (asterisks) coexisting with extensive areas of consolidation. 46肺血管炎的影像表現(xiàn)Figure 15 Variety of CT patter(C) Systemic lupus erythematosus in a 35-year-old patient. CT shows diffuse ground-glass opacities, ill-dened centrilobular nodules, and septal thickening (arrows).(D) Microscopic polyangiitis in a 72-year-old w