特发性肺纤维化IPF.

1、2021-12-1812021-12-182 肺实质是指各级支气管及肺泡结构肺实质是指各级支气管及肺泡结构 肺泡主要指肺泡腔及肺泡上皮细胞。肺泡主要指肺泡腔及肺泡上皮细胞。2021-12-1832021-12-1842021-12-1852021-12-1862021-12-1872021-12-188DPLDKnown causese.g. drugs, CT dsPost-RT, occupation,HPIdiopathicInterstitialpneumoniasGranulomatouse.g. sarcoidosisOther formse.g. LAM, HXIPFOther

2、than IPFDIPRB-ILDAIPCOPLIPNSIP2021-12-1892021-12-18102021-12-18112021-12-18122021-12-18132021-12-18142021-12-181550%YearsRespiratoryFunction/Symptoms1234FVCTraditional View of UIP/IPF ProgressionFVC = forced vital capacity2021-12-181650%YearsRespiratoryFunction/Symptoms123Acute exacerbationStep Theo

3、ry of UIP/IPF ProgressionFVC042021-12-18172021-12-18182021-12-1819Estimated 83,000 CurrentPatients in the United StatesEstimated 31,000 New Patients per Year in the United States05010015020025030045-5455-6465-7475+MaleFemale02040608010012045-5455-6465-7475+MaleFemaleWeycker D, et al. Prevalence, Inc

4、idence, and Economic Costs of Idiopathic Pulmonary Fibrosis. Paper presented at: CHEST 2002, November 2-7, 2002; San Diego, California.PrevalenceIncidence2021-12-18202021-12-18210.01.00 1 2 3 4 5 6 7 8 9Cumulative Proportion SurvivingSurvival Curve for IIP Patients Grouped

5、 by Histologic ClassificationP 0.0003NSIP (n = 30)Discordant UIP (n = 28)Concordant UIP (n = 51)Flaherty KR, et al. Am J Respir Crit Care Med. 2001;164:1722-1727.2021-12-1822ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2000;161:646-664.2021-12-18232021-12-18242021-12-18252021-12-18262021-12

6、-18272021-12-18282021-12-18292021-12-18302021-12-18312021-12-18322021-12-18332021-12-1834InjuryEpithelial cellsSlide courtesy of Paul Noble, MDCapillary2021-12-1835Epithelial cellsCollagenMyofibroblastCell deathGrowth factors and other products of epithelial cell InjurySlide courtesy of Paul Noble,

7、MDCapillary2021-12-18362021-12-18372021-12-18382021-12-18392021-12-18402021-12-18412021-12-1842Courtesy of Kevin O. Leslie, MD.a. Peripheral accentuation of the diseaseb. Transition into uninvolved lungc. Low power pathologyd. High power image of fibroblastic focus Courtesy of Kevin O. Leslie, MD.20

8、21-12-1843ff = fibroblastic fociCourtesy of Fernando J. Martinez, MD.2021-12-18442021-12-18452021-12-1846NSIP FibroticUIPCourtesy of Kevin O. Leslie, MD.2021-12-1847A Potential Natural History of the Pathogenesis of NSIP UIPBeginningNSIPEnd StageUIPFlaherty KR, et al. Am J Respir Crit Care Med. 2001

9、;164:1722-1727. Slide courtesy of Robert Strieter, MDIntermediateNSIP-FStage/Phase2021-12-18482021-12-18492021-12-1850Clinical History Physical Laboratory PFTsPrimary care physiciansPulmonologistsRadiologistsPathologistsMultidimensional and multidisciplinaryRadiology Chest X-ray HRCTPathology Surgic

10、al lung biopsy2021-12-18511970sInterstitial Lung DiseasesSarcoidosisHypersensitivity PneumonitisA heterogeneous group that included a number of diseases2002AsbestosisLAMetcUIP/IPFDIPRB-ILDNSIPCOPLIPCellularFibroticAdapted from ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.AI

11、P2021-12-18522021-12-18532021-12-1854ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2000;161:646-664. 2021-12-18552021-12-18562021-12-1857Chang AC, et al. Ann Thorac Surg. 2002.74;1942-1946.Rena O, et al. Eur J Cardiothora

12、c Surg. 1999;16:624-627. VATS is the preferred procedure for obtaining a lung biopsy- High diagnostic accuracy- Less morbidity and mortality than open lung biopsy - BAL and TBBx limited to excluding other IPF mimickers Ideal biopsy- Two or more surgical wedge biopsies with areas of normal lung- Samp

13、les should measure 3*5 cm in length and 2*3 cm in depth Outpatient thoracoscopic lung biopsy can be a safe and effective procedure for patients with interstitial or focal lung disease- Diagnosis obtained in 61/62 patients- 72.5 % discharged home within 8 hours- 22.5% discharged home within 23 hours2

14、021-12-18582021-12-18592021-12-1860胸部胸部x x线片线片3)3)常规胸部常规胸部x x线片或胸部高分辨率线片或胸部高分辨率CT CT (HRCT) (HRCT) 异常：异常：2021-12-1861A normal chest x-ray does not exclude IPF ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.2021-12-18622021-12-18632021-12-1864ATS/ERS Consensus Statement. Am

15、J Respir Crit Care Med. 2000;161:646-664.Prone scans are often best for showing early abnormalities2021-12-1865Courtesy of David A. Lynch, MD.2021-12-1866UIP: HoneycombingCourtesy of W. Richard Webb, MD.2021-12-1867UIP: Minimal HoneycombingCourtesy of W. Richard Webb, MD.Courtesy of W. Richard Webb,

16、 MD.UIP: Irregular Reticular Opacities2021-12-1868UIP: Traction BronchiectasisCourtesy of W. Richard Webb, MD.2021-12-1869UIP: Minimal Traction BronchiectasisCourtesy of W. Richard Webb, MD.2021-12-1870Subpleural PredominanceCourtesy of W. Richard Webb, MD.2021-12-1871UIP in a 56-year-old man. Thin-

17、section :ground-glass attenuation and localized intralobular reticular opacities (Red arrows). Mild honeycombing (black arrows) also is present2021-12-18722021-12-18732021-12-18742021-12-18752021-12-18762021-12-18772021-12-18782021-12-1879Pathology of IPF:Peripheral Accentuation of DiseaseSlide cour

18、tesy of Kevin Leslie, MD2021-12-1880Slide courtesy of Kevin Leslie, MD2021-12-1881Slide courtesy of Kevin Leslie, MD2021-12-1882Slide courtesy of Kevin Leslie, MD2021-12-18832021-12-1884Other idiopathic interstitial pneumonias NSIP AIP DIP COP RB-ILDATS/ERS Consensus Statement. Am J Respir Crit Care

19、 Med. 2002;165:277-304.ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.2021-12-18852021-12-18862021-12-18872021-12-18882021-12-1889 AIPAIP患者患者HRCTHRCT示磨玻璃影及示磨玻璃影及实变影呈地图实变影呈地图样分布样分布2021-12-1890AIP in a 48-year-old woman. Thin-section CT scan of the right lung obtained 2 cm be

20、low the level of the tracheal carinademonstrates diffuse ground-glass attenuation and intralobular reticular opacities (arrows). 2021-12-1891Acute interstitial pneumonia in a 43-year-old woman. Thin-section CT scan obtained in the right lung 20 days after onset of symptoms. extensive areas of ground

21、-glass attenuation and intralobular reticular opacities. Traction bronchiectasis extends to the level of the segmental bronchi (solid arrowsAlso note a small pleural effusion (open arrows) in the major fissure. 2021-12-1892Acute interstitial pneumonia in a 64-year-old woman. Thin-section CT scan obt

22、ained in the right lung at the level of the tracheal carina 7 days after the onset of symptomspatchy airspace consolidation and traction bronchiectasis (small arrows). Subsegmental bronchi (large arrows) and arteries are distorted, indicating architectural distortion. 2021-12-18932021-12-18942021-12

23、-18952021-12-18962021-12-1897 CTCT片多呈磨玻璃样影片多呈磨玻璃样影 约约1/31/3患者磨玻璃影为仅有的异常征象患者磨玻璃影为仅有的异常征象, , 可合并牵可合并牵拉性支气管扩张拉性支气管扩张, , 蜂窝肺或肺实变较少见蜂窝肺或肺实变较少见 约约1/31/3患者的片难以与患者的片难以与IPFIPF鉴别鉴别2021-12-1898病理病理 病理可见不同程度间质性炎症或纤维化病理可见不同程度间质性炎症或纤维化 病理不具备病理不具备UIPUIP、DIPDIP、COPCOP或或LIPLIP特征特征 镜下镜下纤维化程度比较均一纤维化程度比较均一, , 无成纤维细胞灶无成

24、纤维细胞灶 细胞浸润以淋巴细胞为主细胞浸润以淋巴细胞为主, , 少量肥大细胞少量肥大细胞, , 但肺但肺泡隔淋巴细胞不如泡隔淋巴细胞不如LIPLIP那样密集显著。那样密集显著。2021-12-1899 Non-specific Interstitial PneumoniaFibrotic Non-specific Interstitial PneumoniaCellularImages courtesy of Kevin O. Leslie, MD2021-12-18100NSIP in a 69-year-old woman. Thin-section CT scandemonstrates

25、 areas of air-space consolidation (straight arrows) with a predominantly peribronchovascular distribution. Focal areas of ground-glass attenuation (curved arrows) also are present. 2021-12-18101shows bilateral areas of ground-glass attenuation (arrows) with a predominantly central distribution. The

26、reviewers believed that these findings were more compatible with alveolar proteinosis(蛋蛋白沉积白沉积)than with NSIP. Transverse high-resolution CT image obtained at the level of the lingular bronchus in a 33-year-old woman with NSIP2021-12-181022021-12-18103隐源性机化性肺炎隐源性机化性肺炎(COP)(COP) COPCOP由由DavisonDaviso

27、n等等 19831983年提出。年提出。 19851985年，年，EplerEpler等称本病为阻塞性细支气管炎伴机等称本病为阻塞性细支气管炎伴机化性肺炎化性肺炎(BOOP)(BOOP)。后这一称谓曾获得普遍接受。后这一称谓曾获得普遍接受。但多数学者认为但多数学者认为COPCOP更符合本病的特点更符合本病的特点2021-12-181042021-12-181052021-12-181062021-12-181072021-12-181082021-12-18109 CTCT片显示有实变片显示有实变, , 位于位于胸膜下和支气管周围胸膜下和支气管周围, , 下肺下肺野最常见野最常见 实变区常见实

28、变区常见空支气管征及轻度柱状支气管扩张空支气管征及轻度柱状支气管扩张图图 患者支气管血管束周围可见的小结节影患者支气管血管束周围可见的小结节影, , 须与肺泡须与肺泡癌、淋巴瘤、结节病、嗜酸性粒细胞性肺炎等鉴别癌、淋巴瘤、结节病、嗜酸性粒细胞性肺炎等鉴别 如呈支气管周围及胸膜下片状实变如呈支气管周围及胸膜下片状实变, , 经数周抗生素经数周抗生素治疗阴影反增多者治疗阴影反增多者, , 应高度疑应高度疑COPCOP2021-12-18110shows poorly defined arcadelike(拱廊样拱廊样) and polygonal(多角形的多角形的) opacities (peri

29、lobular pattern) in the left lower lobe in both subpleural and central regions of the lung. The opacities resemble thickened interlobular septa.Transverse thin-section CT scan at the level of dome of the right hemidiaphragm in a 50-year-old woman with cryptogenic organizing pneumonia2021-12-18111Few

30、 abnormally thickened interlobular septa (arrowheads) are seen anteriorly in both upper lobes. These linear opacities are thinner and more sharply demarcatedTransverse thin-section CT scan through upper lobes in a 49-year-old woman with cryptogenic organizing pneumonia2021-12-18112There is extensive

31、 airspace consolidation posteriorly(后面的后面的) in the right lower lobe.Transverse thin-section CT scan at the level of lung base in a 51-year-old woman with cryptogenic organizing pneumoniaPerilobular opacities (arrows) are seen abutting(邻接的邻接的) the pleural surface in the left lower lobe2021-12-18113Th

32、e perilobular opacities (arrows) in right lower lobe are centrally located and surrounded by aerated lung parenchyma.Transverse thin-section CT scan through lower lobes in a 44-year-old man with cryptogenic organizing pneumonia2021-12-18114Perilobular opacity (arrow) not immediately obvious is locat

33、ed adjacent to a focus of airspace consolidation in the left lower lobe. There are few bandlike opacities (arrowheads) in the right lower lobe. Transverse thin-section CT scan at the level of lung base in a 61-year-old man with cryptogenic organizing pneumonia2021-12-18115There is perilobular opacit

34、y (arrowhead) and dilatation and distortion of airways (arrow) indicating presence of interstitial fibrosis. Transverse thin-section CT scan through lower lobes in a 37-year-old woman with cryptogenic organizing pneumonia2021-12-18116BOOP in an 81-year-old woman. Thin-section CT scan obtained 1 cm b

35、elow the level of the tracheal carinapatchy bilateral air-space consolidation (open arrows)areas of ground-glass attenuation (curved arrows)Ill-defined nodular(小结的小结的) areas of consolidation (solid straight arrows) also are present. 2021-12-181172021-12-18118 是间质性肺病合并呼吸细支气管损害。是间质性肺病合并呼吸细支气管损害。 呼吸细支气

36、管炎呼吸细支气管炎RBRB是吸烟者的病变是吸烟者的病变, , 其特征为其特征为、级呼吸细支气管腔内充满大量含色素的巨噬细胞级呼吸细支气管腔内充满大量含色素的巨噬细胞 单纯单纯RBRB很少引起症状很少引起症状, , 但当它成为间质性肺病时但当它成为间质性肺病时, , 可有明显症状和影像学改变可有明显症状和影像学改变 多小叶中心性肺气肿。含色素的巨噬细胞充填肺泡多小叶中心性肺气肿。含色素的巨噬细胞充填肺泡, , 故有人认为故有人认为RBILDRBILD是是DIPDIP的早期阶段的早期阶段2021-12-181192021-12-181202021-12-181212021-12-181222021

37、-12-181232021-12-181242021-12-181252021-12-181262021-12-18127 X X线胸片对诊断线胸片对诊断DIPDIP不够敏感不够敏感, 3, 3一一22%22%显示正常显示正常 CTCT片显示片显示全部均有磨玻璃影全部均有磨玻璃影, , 位于下肺野占位于下肺野占73%, 73%, 周边占周边占59%, 59%, 弥漫分布占弥漫分布占18%18%。59%59%可见线状或网状可见线状或网状影影, , 下肺居多下肺居多, , 少于少于1/31/3有局限性蜂窝状变有局限性蜂窝状变2021-12-181282021-12-18129DIP in a 42

38、-year-old man.Thin-section CT scan obtained at the level of the dome of the right hemidiaphragm demonstrates diffuse, bilateral areas of ground-glass attenuation (arrows). 2021-12-181302021-12-181312021-12-18132shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surroun

39、ding alveolar septa (arrows). Sjgren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. Photomicrograph (original magnification, x20; H-E stain)2021-12-181332021-12-181342021-12-181352021-12-18136 centrilobular nodules and branching linear structures (straight arrow) in the righ

40、t lung. Many thin-walled cysts (curved arrows) were seen in both lungs. Sjgren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the carina 2021-12-181372021-12-181382021-12-18139diffusely distributed areas wi

41、th airspace consolidation and ground-glass attenuation. Note thickened bronchovascular bundles (arrowheads) and interlobular septal thickening (arrows). Drug-induced eosinophilic pneumonia in a 56-year-old man. Transverse thin-section CT scan through the right upper lobe2021-12-18140patchy ground-gl

42、ass opacity with interlobular septal lines and centrilobular nodules and branching lines (arrows). Isoniazid-induced pneumonitis in a 45-year-old woman. Transverse thin-section CT scan2021-12-181412021-12-18142ground-glass attenuation and irregular linear hyperattenuating areas in the subpleural are

43、as of both lower lung zones, as well as traction bronchiectasis (arrows). Systemic lupus erythematosus and nonspecific interstitial pneumonia (fibrosing pattern) in a 67-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein2021-12-18143patch

44、y areas of ground-glass attenuation and consolidation with a subpleural or peribronchovascular distribution in both lungs. Rheumatoid arthritis and BOOP in a 68-year-old man. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein2021-12-18144NL = normal lung

45、ff = fibroblastic foci Copyright 2004-2005 The France Foundation2021-12-181452021-12-181462021-12-181472021-12-181482021-12-181492021-12-18150N Engl J Med 1997; 336:1224-1234 2021-12-18151Pl = pleuraBV = bronchovascular bundles Copyright 2004-2005 The France Foundation2021-12-181522021-12-181532021-

46、12-181542021-12-181552021-12-181562021-12-181572021-12-18158cysts of somewhat uniform size2021-12-181592021-12-181602021-12-181612021-12-18162shows fine reticulonodular pattern in the upper lobes, with relative sparing of the lower lobes. Langerhans cell histiocytosis. (a) Transverse thin-section CT

47、 image2021-12-18163shows marked dilatation of the main pulmonary artery (arrowheads) relative to the ascending aorta (a), indicating severe pulmonary hypertension. Langerhans cell histiocytosis. ( (b) Transverse contrast-enhanced CT image2021-12-18164shows Langerhans cell histiocytosis nodule involv

48、ing and partly obliterating(使消失使消失) a pulmonary artery (arrowheads). (Original magnification, x20.)Langerhans cell histiocytosis. (c) Photomicrograph of biopsy specimen in another patient2021-12-18165demonstrates extensive areas of airspace consolidation (large arrows) and ground-glass attenuation (

49、small arrows) involving mainly the peripheral lung region. Chronic eosinophilic pneumonia in a 64-year-old woman. Transverse thin-section CT scan (1.5-mm collimation) at the level of the tracheal carina2021-12-18166diffusely distributed thickening of interlobular septa (large arrows) and areas of gr

50、ound-glass attenuation. Also note peripheral thickened bronchovascular bundles (small arrows). Acute eosinophilic pneumonia in a 28-year-old man. Transverse thin-section CT scan through the apical segments of right upper lobe 2021-12-181672021-12-181682021-12-181692021-12-181702021-12-181712021-12-181722021-12-181732021-12-181742021-12-18175 双肺磨玻璃样改变双肺磨玻璃样改变, ,双肺中下野斑片样阴影双肺中下野斑片样阴影, ,边缘边缘模糊模糊, ,其内隐约见小结节影其内隐约见小结节影 图图2 2 双肺双肺磨玻璃样磨玻璃样改变改变, ,肺野内弥漫性斑片状影肺野内弥漫性斑片状影, ,边缘清边缘清,