1、1,Respiratory disease,放射学院 刘林祥6222136 ,2,特发性肺间质纤维化,原因不明的弥漫性纤维性肺泡炎又称Hamman-Rich综合征为肺泡壁损伤所引起的非感染性炎性反应近认为系免疫性疾病，可能与遗传有关,3,病 理,急性期：肺泡内皮细胞和基底膜受损，肺泡和间质内蛋白样物质渗出，伴透明膜形成，继而淋巴细胞和单核细胞渗出。肺泡内皮细胞再生覆盖在渗出物表面并使其整合入肺间质，肺泡壁增厚，胶原纤维扭曲、紊乱而机化。病变发展，间质纤维化加重晚期：肺泡壁、小叶间隔及胸膜下广泛纤维化，肺体积缩小变硬，毛细血管网和气道的终末部分被破坏。在范围较大的纤维化区域，可有终末气道的代偿性
2、扩张，形成直径数mm至2cm的囊样含气腔隙,4,Hamman-Rich Syndrome,多见于中年，男、女无差别多起病隐匿，初期无症状进行性呼吸困难和干咳进展速度因人而异：快者12年内出现发绀和杵状指，并发肺心病慢者可数年甚或十几年不出现明显缺氧症状，但最终出现缺氧及肺心病易合并肺部感染，反复感染可加快肺纤维化的发展肺功能检查呈限制性通气障碍及低氧血症,5,胸部X线平片,早期两肺中下野细小网织阴影病变发展，不对称性、弥漫性网状、条索状及结节状阴影，可扩展至上肺野晚期，结节影增大，伴广泛厚壁囊状阴影，蜂窝肺并阻塞性肺气肿时，肺野透亮度增强囊肿破裂可发生自发性气胸肺纤维化严重时可发生肺动脉高压和
3、肺心病,6,Idiopathic pulmonary fibrosis,磨玻璃样影及实变影，内见含气支气管影，支气管血管数增粗 Ground-glass attenuation与胸膜面垂直的细线形影，长1-2cm，宽约1mm，多见于两肺下叶两肺中内带小叶间隔增厚，分支状细线形影，Reticular attenuation with interlobular septal thickening胸膜下0.5cm内与胸壁内面弧度一致的弧线状影，长5-10cm，边缘较清或略模糊，见于两下肺后外部,7,Idiopathic pulmonary fibrosis,蜂窝状影，数mm至2cm不等的圆形或椭圆形
4、含气囊腔，壁薄而清楚，与正常肺交界面清楚。分布于两肺基底部胸膜下区. A honeycomb pattern, predominantly basal and peripheral in distribution小结节影，边缘较清楚，纤维条索在横断面的表现，或相互交织而成小叶中心性肺气肿：散在、2-4mm，肺外围部，病变发展可渐见于肺中央部。胸膜下见1-2cm类圆形肺气囊中小支气管扩张，柱状，伴支气管扭曲、并拢 Architectural distortion with associated traction bronchiectasis and bronchiolectasis,8,Idio
5、pathic pulmonary fibrosis,F47peripheral GGO Interlobular septal thickeningIrregularity of the fissuresBronchiectasisEarly interstitial pneumonia22m later, progression of interstitial pneumoniaDiffuse GGO, interlobular septal thickeningA honeycomb pattern,9,鉴别诊断,肺类风湿性病的广泛性肺间质纤维化，最后发展为蜂窝肺，与HRS相似。但前者有渐
6、进性坏死结节即肉芽肿及胸腔积液表现，有别于HRS红斑狼疮的胸部表现以心肌炎所致的心脏增大、间质性肺炎、节段性盘状肺不张和胸腔积液等所见为特征，与HRS不同硬皮病的肺间质纤维化发展至晚期可出现蜂窝肺，如有皮肤的改变以及在食管造影见其张力减低或狭窄等表现，则有助于硬皮病的诊断,10,结节病（Sarcoidosis）,A systemic disorder of unknown causeNoncaseating granulomas with proliferation of epithelioid cells, 多系统肉芽肿性疾病，良性经过，可累及淋巴结、肺、胸膜、皮肤、骨、眼、脾、肝、腮腺及扁
7、桃体等病理特征为非干酪性肉芽肿淋巴结大，但不融合。肺门LN易受累，次为气管旁和AA旁肺内病变沿支气管血管周围结缔组织鞘及小叶间隔发展蔓延，肺内肉芽肿主要分布在间质，小，直径在0.4mm以下，胸膜下肺间质内肉芽肿更密集。小肉芽肿可融合成大结节急性发病者肉芽肿大多经治疗消退或自行消退。慢性发病者常导致进行性肺纤维化,11,Sarcoidosis,见于任何年龄，2040岁多见，女性多Young & middle-aged, slightly higher in womenDistinct geographic and racial predilections, commonly African-Am
8、ericans, Swedes, & Danes病程缓慢，轻者无症状；症状与影像表现不相称咳嗽，少量粘痰、乏力、低热、盗汗、纳差及胸闷肝脾肿大、皮肤结节、关节疼痛、腮腺肿大、外周淋巴结肿大及眼部病变症状,12,Laboratory,Angiotensin converting enzyme (ACE) level elevated and may correlate with activityCD4:CD8 ratio is commonly decreasedHypercalcemia due to increased intestinal absorption of calcium, re
9、sulting from activation of vitamin D by macrophages in sarcoid granulomas,13,胸部X线平片,纵隔、肺门淋巴结肿大，半数为唯一异常多组淋巴结肿大，两肺门对称性淋巴结肿大，状如土豆，为典型表现肺门淋巴结肿大的程度比其他部位更加显著少有纵隔淋巴结而无肺门淋巴结者淋巴结肿大一般在6-12个月期间可自行消退，恢复正常；或在肺部出现病变过程中，开始缩小或消退；或不继续增大，为结节病的发展规律,14,Sarcoidosis,肺部病变多发生在淋巴结病变之后两肺弥漫性网状结节影，但肺尖或肺底少或无。结节大小不一，多为1-3mm大小，轮廓
10、尚清楚肺内圆形病变，直径约1.0-1.5cm，密度均匀，边缘较清楚，单发者类似肺内良性病变或周围型肺癌，多发者酷似肺转移瘤节段性或小叶性浸润，类似肺部炎性病变，一般伴或不伴胸腔内淋巴结病变少数为单纯粟粒状，似急性粟粒型结核,15,Sarcoidosis,以纤维性病变为主者，不易与其他原因所致的肺纤维化区别，且可引起多种继发性改变胸膜渗液可能为胸膜脏、壁层广泛受累所致。肥厚的胸膜为非干酪性肉芽肿骨病变约占10%。损害一般限于手、足的短管状骨，显示小囊状骨质缺损并伴有末节指（趾）的骨质吸收，变细、变短,16,CT,纵隔、肺门淋巴结肿大，密度均匀，边缘清楚，周围脂肪界面存在。增强扫描呈均匀强化肺内可
11、见大小结节影或块状影晚期支气管血管束扭曲、聚拢或变形，叶间裂、血管支气管移位，支气管扩张和不同程度肺气肿支气管血管束增厚，边缘不规则或结节状，周围可有大小不等的结节状影；小叶间隔增厚和细小蜂窝影，见于胸膜下区胸膜初期为胸腔积液，可自然吸收，少数可发展为胸膜肥厚,17,鉴别诊断,肺门结核：年轻，有轻度中毒症状。气管旁、支气管旁淋巴结肿大，可有钙化。结素反应阳性，痰中找到结核杆菌霍奇金病：常先有颈部、锁骨上淋巴结肿大，然后出现不对称性双侧或单侧纵隔淋巴结肿大，前纵隔较后纵隔多见。纵隔淋巴结肿大的程度常较肺门淋巴结肿大显著非霍奇金淋巴瘤：多为单侧纵隔淋巴结肿大，即使双侧纵隔淋巴结肿大亦不对称。后纵隔
12、多于前纵隔淋巴结肿大，晚期才有肺门淋巴结肿大。纵隔淋巴结多大于肺门淋巴结未分化型小细胞肺癌：多为单侧纵隔或（和）肺门分叶状淋巴结肿大，双侧纵隔淋巴结肿大较少见。部分伴有不同程度的阻塞性肺炎或肺不张。病程发展迅速间质性病变：当病变发展至纤维化期则需与癌性淋巴管炎、间质性肺炎、嗜酸性肉芽肿等鉴别,18,Sarcoidosis,Symptoms and signs are nonspecific, half asymptomaticFatigue, weight loss, general malaise, feverBilateral hilar lymphadenopathy is common
13、 findingOften with associated pulmonary infiltratesSkin and ocular lesions, the liver, spleen, lymph nodes, parotid glands, CNS, genitourinary system, muscles, and bones may be involved,19,Radiologic stages,Stage 0: normal chest radiographstage 1: lymphadenopathy only (1/2)stage 2: lymphadenopathy w
14、ith parenchymal infiltrationstage 3: parenchymal disease onlystage 4: pulmonary fibrosis,20,Hilar adenopathy,M27Chest radiograph Typical bilateral hilar adenopathyAdenopathy in the right paratracheal and left aortic-pulmonary window nodes is also identified,21,Hilar adenopathy,E+ CT clearly depicts
15、the bilateral hilar adenopathy,22,Mediastinal Lymph Nodes,Bilateral hilar adenopathyRight paratracheal adenopathyLeft paratracheal and aortic-pulmonary window nodes are also commonly enlargedCalcification occurs in affected nodes，It can be amorphous, punctate, or eggshell-like; it is closely related
16、 to the duration of the disease and suggests a chronic condition,23,Mediastinal adenopathy,M26Severe back painEnlarged right paratracheal nodesLeft aortic-pulmonary window nodes with associated minimal hilar involvement are also seen,24,Mediastinal adenopathy,Mediastinal adenopathy, 60MCalcification
17、 in the affected hilar nodes, huge subcarinal lymph nodesAn unusual finding in other granulomatous diseases such as tuberculosis,25,Pulmonary sarcoidosis,Lung involvement in 20% of patientsStrong predilection for the upper lungDyspnea and dry cough are commonSarcoid granulomas in lung are distribute
18、d along lymphatic vessels in interstitial tissues of bronchovascular bundles and subpleural and perilobular spacesMultiple miliary nodules, bronchial wall thickening, and ground-glass attenuationPerivascular or subpleural distribution Coalescing granulomas can form multiple masslike nodulesNodules h
19、ave irregular margins and may contain an air bronchogram,26,Pulmonary sarcoidosis,Stage IV disease may manifest as conglomerated masses with marked traction bronchiectasisUsually predominantly in the central and upper lung. this distribution is typical of sarcoidosis but can also be seen in tubercul
20、osis and silicosisExtensive calcification may be encountered within fibrotic granulomasCavitation or cyst formation may also be seen,27,Pulmonary sarcoidosis,M37Small nodules with a perivascular distribution and irregular thickening of bronchovascular bundles and interlobular septa,28,Pulmonary sarc
21、oidosis,M24HRCTMultiple miliary nodules and diffuse thickening of the bronchial wallSimultaneous presence of small nodules with a perivascular distribution and along the interlobular pleura,29,Pulmonary sarcoidosis,F31Hazy ground-glass opacity with a lower lung predominance, both of which are unusua
22、l findings.,30,Pulmonary sarcoidosis,HRCTWidespread ground glass attenuationReticulonodular Mild bronchiectasis peripherally,31,Pulmonary sarcoidosis,F26Multiple nodules bilaterallyMinimal hilar adenopathySimulate metastates,32,Pulmonary sarcoidosis,A nodular consolidation with ill-defined bordersAi
23、r bronchogram within the nodules,33,Stage IV pulmonary sarcoidosis,M60, Extensive fibrotic change and cavitary lesions with a central distribution distorting lung parenchymaIrregular thickening of pleura and overinflation of peripheral lung parenchyma,34,Primary Bronchogenic Cancer,Macro-pathologyCe
24、ntral type：主支气管、肺叶支气管及肺段支气管的肺癌Peripheral type：肺段以下支气管直到细支气管以上的肺癌Alveolar type：发生于细支气管或肺泡上皮的肺癌,35,Histological types of lung cancer,来自支气管表面上皮的癌鳞状上皮癌 Squamous cell carcinoma腺癌 Adenocarcinoma腺鳞癌大细胞癌 Large cell carcinoma来自神经内分泌细胞的癌高分化：类癌 carcinoid中分化：不典型类癌 atypical carcinoid低分化：小细胞癌 Small cell carcinoma
25、来自细支气管Clara细胞和型肺细胞的癌细支气管肺泡癌 bronchial alveolar carcinoma,36,中心型肺癌,临床表现：刺激性干咳、痰中带血，胸痛、发热直接征象：肺门肿块，支气管狭窄与阻塞间接征象：支气管阻塞或狭窄后引起的阻塞性肺炎、肺不张、肺气肿转移征象,37,右肺上叶中心型肺癌,38,右肺上叶中心型肺癌,39,右肺上叶中心型肺癌,40,中心型肺癌,纵隔增宽、肺门增大，为肺癌的淋巴结转移,41,中心型肺癌：右肺门及右上纵隔不规则肿块，中上肺野片絮状密度增高影。断层示上叶支气管鼠尾状狭窄,42,中心型肺癌,左肺肺癌，右肺代偿性肺气肿,43,Central type-CT
26、findings,管壁型：管壁增厚，管腔不规则狭窄管内型：腔内软组织肿块，偏心性狭窄或闭塞，杯口状截断管外型：管壁环形增厚，腔外软组织肿块管腔狭窄或闭塞，继发远端肺炎症、不张或气肿肺门纵隔淋巴结转移，呈软组织样等密度，单个或多个融合成分叶状，增强扫描无强化肺内，胸膜及远处转移,44,中心型肺癌,45,Central type-CT findings,46,Central type-CT findings,47,Central type,48,Squamous cell carcinoma,49,Squamous cell carcinoma,50,Squamous cell carcinoma
27、,51,Lung cancer-peripheral type,临床表现：可无症状或胸痛、咳嗽直接征象：肺内结节和肿块边缘分叶，脐样切迹，毛刺小于2cm的孤立结节，内部密度不均匀。大于3cm时，密度较均匀厚壁空洞间接征象：小叶范围的阻塞性肺炎、肺不张，彗尾征。胸膜凹陷转移征象,52,右肺见结节性病灶，CT示分叶与毛刺,53,厚壁空洞，体层示空洞内壁凹凸不平,54,周围型肺癌-癌性空洞,55,Peripheral type,肺内肿块，下缘与胸膜间有牵拽，箭头所指为慧尾征,56,Peripheral typeCT findings,肺内结节，肿块软组织密度，均匀或不均匀，空泡征，钙化少肺窗：边缘毛
28、刺，胸膜凹陷征，血管集束征纵隔窗：深分叶，偏心空洞，小棘状突起，脐征肺门纵隔淋巴结转移远处转移,57,周围型肺癌,58,Peripheral carcinoma,59,Peripheral carcinoma,ground-glass opacity,60,Peripheral carcinoma,Soft tissue massSpicula,61,Peripheral carcinoma,62,Peripheral carcinoma,63,Peripheral carcinoma-lobulation,64,Hilar metastases,65,Peripheral carcinoma
29、,66,Peripheral carcinoma,67,Peripheral carcinoma,68,Peripheral carcinoma,69,Peripheral carcinoma-cavity,70,Pancoast tumors,Arise from lung apexInvade costovertebral groove in superior sulcus Invade parietal pleura, causing nonspecific shoulder pain radiating down medial aspect of scapulaInvolve T1 n
30、erve root, causing pain radiating along medial aspect of arm and forearm as far as wristInvade stellate ganglion, causing Horner syndrome, include ptosis (narrowing of the palpebral fissure), miosis (pupillary constriction), and anhidrosis (absence of sweating on one side of the face),71,Pancoast tu
31、mors,Invade first, second, and third ribs posteriorlySuperior extension encasing C8 nerve root, with resultant pain in medial two digits of hand and atrophy of intrinsic muscles of handAnterior extension into tracheoesophageal groove result in vagal or recurrent laryngeal nerve palsySuperomedial ext
32、ension may result in involvement of vertebral artery, vertebral bodies, neurovertebral foramina, and spinal canal, with a consequent risk of paraplegia,72,右肺上沟癌,右肺上野片状阴影，第一、二肋骨溶骨性破坏,73,Pancoast tumor,74,M48，left interscapular chest wall pain suggeste T1 nerve root invasion,75,Superior sulcus tumor,M
33、48 w/o neurologic signs in left upper extremity but with left interscapular chest wall pain suggestive of T1 nerve root invasion,M48，left interscapular chest wall pain suggeste T1 nerve root invasion,76,Alveolar cancinoma,双肺布满结节性病灶病理证实为肺泡癌,77,肺泡癌,78,Alveolar cancinoma,79,Alveolar cancinoma,80,Alveol
34、ar cancinoma,81,Bronchioloalveolar carcinomaA53-year-old man,Initial (1mm) CT at the level of the bronchus intermedius a small nodular area of ground-glass attenuation in the right upper lobeFollow-up CT (5mm) 48 months later increased nodule size.,82,肺转移瘤,宫颈癌术后，肺内数个转移病灶,83,Lung metastases,84,Lung m
35、etastases, rectal cancer,85,Lung metastases,86,食道癌肺转移,87,Lung metastases, colon cancer,88,错构瘤(hamartoma),非真性肿瘤，内胚层与间胚层发育异常形成根据部位，分为周围型和中央型局限于某一肺叶或肺段的反复发作感染肺内球形软组织肿块，见爆米花样钙化CT扫描：钙化、钙化、脂肪,89,错构瘤,左肺门外上肿块内可见爆米花样钙化,90,Hamartoma,91,错构瘤,92,Hamartoma,93,肺栓塞(pulmonary emboli),肺动脉分支被栓子堵塞引起的肺供血障碍常见栓子是深静脉脱落的血栓久
36、病卧床、妊娠、大手术后和心功能不全可发生深静脉血栓风心病，原发于肺动脉的血栓进入血循环的脂肪、肿瘤栓子和气体,94,Pulmonary embolism,The third most common acute cardiovascular disease after myocardial infarction and strokeResults in thousands of deaths each year because it often goes undetectedCTA has sensitivities of 53%100% and specificities of 83%100%
37、Pulmonary angiography, the diagnostic standard of reference for confirming or refuting diagnosis,95,病理,双重供血，正常时两组有丰富的吻合支当肺动脉的某一分支栓塞后，肺组织因支气管动脉的侧枝供血而不发生异常，栓子较小未能完全堵塞肺动脉分支时也不易发生供血障碍多数小栓子进入肺循环可引起肺动脉小分支多发性栓塞,96,临床表现,多无明显症状，或有轻微不适可为突发的呼吸困难和胸痛肺动脉大分支或主干栓塞或广泛的肺动脉小分支栓塞可出现严重的呼吸困难、发绀、休克或死亡较大的栓子堵塞肺动脉大分支或主干可引起急性
38、右心衰竭或心肌梗死而致死亡,97,X线表现,肺动脉较大分支栓塞或多发性小分支栓塞X线平片可出现异常阴影，较小分支栓塞即使出现症状并经血管造影证实，X线仍可正常肺缺血又称Westermark征，当肺叶或肺段动脉栓塞时，相应区域内肺血管纹理减少或消失，透亮度增加多发性肺小动脉栓塞引起广泛性肺缺血，显示肺纹理普遍减少和肺野透亮度增加，但无肺体积膨胀现象,98,X线表现,嵌塞在肺动脉内的血栓使相应部位血管阴影增宽，阻塞远端致血流减少而变细多发于下叶且以右下叶多见，下叶体积缩小，膈肌升高，叶间裂下移。并盘状肺不张较大肺动脉栓塞或多发性小动脉栓塞可引起心影增大，主要是右心室增大，同时有肺动脉高压右心功能不
39、全时心影增大更为显著，奇静脉和上腔静脉增粗,99,X线血管造影,肺动脉分支内充盈缺损或截断局限性血管减少或无血管区，血灌流缓慢小分支多发性栓塞引起肺动脉外围分支迂曲，突然变细，呈剪枝样细小分支的栓塞血管造影不能显示血栓24hs后开始溶解，故48hs后造影可正常继发肺动脉高压和肺心病时，肺动脉干和大分支扩张，周围分支变细,100,CT,平扫：较大肺动脉栓塞见血管内高密度或低密度病灶。高密度为新鲜血栓，低密度为陈旧性血栓增强：血栓为长条状及不规则形充盈缺损栓塞的肺动脉可有不同程度的扩张较大肺血管栓塞可见相应区域肺血管分布减少,101,Acute pulmonary embolism,Arteria
40、l occlusion with failure to enhance the entire lumen due to a large filling defectArtery may enlarged vs. adjacent patent vessels A partial filling defect surrounded by contrast material, polo mint sign, railway track signPeripheral wedge shaped areas of hyperattenuation represent infarcts, along wi
41、th linear bands are significant ancillary findings,102,Acute occlusive pulmonary embolism,F32Chest painA pulmonary embolus within posterobasal segment of right lower lobe arteryArtery enlarge vs. adjacent patent vessels,103,Acute pulmonary embolism,F45chest painA pulmonary embolus affects the segmen
42、tal artery of the laterobasal segment of right lower lobePartial filling defect surrounded by contrast material produces the polo mint sign,104,Acute pulmonary embolism,M66Chest pain and dyspneaAcute pulmonary embolus causes a partial filling defect surrounded by contrast material Another acute pulm
43、onary embolus affects the left main pulmonary artery,105,Acute pulmonary embolism,F58Chest pain and dyspneaA pulmonary embolus results in an eccentrically positioned partial filling defectSurrounded by contrast material and forms acute angles with the arterial wall,106,Acute pulmonary embolism,F58ch
44、est pain and dyspneaAncillary findings of a peripheral wedge-shaped area of hyperattenuation,107,Acute pulmonary embolism,Morphologic abnormalities suggest right ventricular failure can be quantified with CT pulmonary angiographyRV dilatation (RV cavity is wider than LV cavity in the short axis), w/
45、o contrast material reflux into hepatic veinsDeviation of interventricular septum toward LVA pulmonary embolism index greater than 60%,108,Acute pulmonary embolism,M42chest pain and severe dyspnea,short axis of RV is wider than that of LV, caused by acute pulmonary embolism and created RV strain,109
46、,Acute central pulmonary embolism,F87 asymptomatic Subtle regions of hyperattenuation CTA: acute pulmonary embolism within the right main and left interlobar pulmonary arteries,110,Chronic Pulmonary EmbolismDiagnostic criteria,Complete occluded vessel is smaller than adjacent patent vesselsA periphe
47、ral, crescent shaped intraluminal defect forms obtuse angles with vessel wallContrast material flowing through thickened, often smaller arteries due to recanalizationA web or flap within a contrast filled artery Secondary signsExtensive bronchial collateral vesselsAn accompanying mosaic perfusion pa
48、tternCalcification within eccentric vessel thickening,111,Chronic pulmonary embolism,27MdyspneaComplete occluded vessels in left lung are smaller than adjacent patent vesselsCollateral blood supply from a branch of right hemidiaphragmatic artery,112,Chronic pulmonary embolism,M62DyspneaAn eccentrica
49、lly located thrombus forms obtuse angles with vessel wallDilated collateral bronchial artery,113,Chronic pulmonary embolism,A small, recanalized pulmonary artery with contrast material in the central lumen,114,Chronic pulmonary embolism,M56dyspneaA flap within a small right interlobar pulmonary arteryCollateral bronchial artery dilatation is also noted,
