特发性肺纤维化IPF

1、2022-3-1712022-3-1722022-3-1732022-3-1742022-3-1752022-3-1762022-3-1772022-3-178DPLDKnown causese.g. drugs, CT dsPost-RT, occupation,HPIdiopathicInterstitialpneumoniasGranulomatouse.g. sarcoidosisOther formse.g. LAM, HXIPFOther than IPFDIPRB-ILDAIPCOPLIPNSIP2022-3-1792022-3-17102022-3-17112022-3-171

2、22022-3-17132022-3-17142022-3-171550%YearsRespiratoryFunction/Symptoms1234FVCTraditional View of UIP/IPF ProgressionFVC = forced vital capacity2022-3-171650%YearsRespiratoryFunction/Symptoms123Acute exacerbationStep Theory of UIP/IPF ProgressionFVC042022-3-17172022-3-17182022-3-1719Estimated 83,000

3、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, Incidence, and Economic Costs of Idiopathic Pulmonary Fibrosis. Paper presented at: CH

4、EST 2002, November 2-7, 2002; San Diego, California.PrevalenceIncidence2022-3-17202022-3-17210.01.00 1 2 3 4 5 6 7 8 9Cumulative Proportion SurvivingSurvival Curve for IIP Patients Grouped by Histologic ClassificationP 0.0003NSIP (n = 30)Discordant UIP (n = 28)Concordant U

5、IP (n = 51)Flaherty KR, et al. Am J Respir Crit Care Med. 2001;164:1722-1727.2022-3-1722ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2000;161:646-664.2022-3-17232022-3-17242022-3-17252022-3-17262022-3-17272022-3-17282022-3-17292022-3-17302022-3-17312022-3-17322022-3-17332022-3-1734InjuryEpi

6、thelial cellsSlide courtesy of Paul Noble, MDCapillary2022-3-1735Epithelial cellsCollagenMyofibroblastCell deathGrowth factors and other products of epithelial cell InjurySlide courtesy of Paul Noble, MDCapillary2022-3-17362022-3-17372022-3-17382022-3-17392022-3-17402022-3-17412022-3-1742Courtesy of

7、 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.2022-3-1743ff = fibroblastic fociCourtesy of Fernando J. Martinez, MD.2022-3-17442022-3-17452022-3-1746NSIP

8、FibroticUIPCourtesy of Kevin O. Leslie, MD.2022-3-1747A Potential Natural History of the Pathogenesis of NSIP UIPBeginningNSIPEnd StageUIPFlaherty KR, et al. Am J Respir Crit Care Med. 2001;164:1722-1727. Slide courtesy of Robert Strieter, MDIntermediateNSIP-FStage/Phase2022-3-17482022-3-17492022-3-

9、1750Clinical History Physical Laboratory PFTsPrimary care physiciansPulmonologistsRadiologistsPathologistsMultidimensional and multidisciplinaryRadiology Chest X-ray HRCTPathology Surgical lung biopsy2022-3-17511970sInterstitial Lung DiseasesSarcoidosisHypersensitivity PneumonitisA heterogeneous gro

10、up that included a number of diseases2002AsbestosisLAMetcUIP/IPFDIPRB-ILDNSIPCOPLIPCellularFibroticAdapted from ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.AIP2022-3-17522022-3-17532022-3-1754ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.ATS/ERS Co

11、nsensus Statement. Am J Resp Crit Care Med. 2000;161:646-664. 2022-3-17552022-3-17562022-3-1757Chang AC, et al. Ann Thorac Surg. 2002.74;1942-1946.Rena O, et al. Eur J Cardiothorac Surg. 1999;16:624-627. VATS is the preferred procedure for obtaining a lung biopsy- High diagnostic accuracy- Less morb

12、idity 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- Samples should measure 3*5 cm in length and 2*3 cm in depth Outpatient thoracoscopic lung biopsy can be a safe and effective

13、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 hours2022-3-17582022-3-17592022-3-17603)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：2022-3-1761A normal chest x-ra

14、y does not exclude IPF ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.2022-3-17622022-3-17632022-3-1764ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.Prone scans are often best for showing early abnormalities2022-3-1765Courtesy of David A. Lynch, M

15、D.2022-3-1766UIP: HoneycombingCourtesy of W. Richard Webb, MD.2022-3-1767UIP: Minimal HoneycombingCourtesy of W. Richard Webb, MD.Courtesy of W. Richard Webb, MD.UIP: Irregular Reticular Opacities2022-3-1768UIP: Traction BronchiectasisCourtesy of W. Richard Webb, MD.2022-3-1769UIP: Minimal Traction

16、BronchiectasisCourtesy of W. Richard Webb, MD.2022-3-1770Subpleural PredominanceCourtesy of W. Richard Webb, MD.2022-3-1771UIP in a 56-year-old man. Thin-section :ground-glass attenuation and localized intralobular reticular opacities (Red arrows). Mild honeycombing (black arrows) also is present202

17、2-3-17722022-3-17732022-3-17742022-3-17752022-3-17762022-3-17772022-3-17782022-3-1779Pathology of IPF:Peripheral Accentuation of DiseaseSlide courtesy of Kevin Leslie, MD2022-3-1780Slide courtesy of Kevin Leslie, MD2022-3-1781Slide courtesy of Kevin Leslie, MD2022-3-1782Slide courtesy of Kevin Lesli

18、e, MD2022-3-17832022-3-1784Other idiopathic interstitial pneumonias NSIP AIP DIP COP RB-ILDATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.2022-3-17852022-3-17862022-3-17872022-3-17882022-3-17892022-3-1

19、790AIP in a 48-year-old woman. Thin-section CT scan of the right lung obtained 2 cm below the level of the tracheal carinademonstrates diffuse ground-glass attenuation and intralobular reticular opacities (arrows). 2022-3-1791Acute interstitial pneumonia in a 43-year-old woman. Thin-section CT scan

20、obtained in the right lung 20 days after onset of symptoms. extensive areas of ground-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. 2022

21、-3-1792Acute interstitial pneumonia in a 64-year-old woman. Thin-section CT scan obtained 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

22、 are distorted, indicating architectural distortion. 2022-3-17932022-3-17942022-3-17952022-3-17962022-3-17972022-3-17982022-3-1799 Non-specific Interstitial PneumoniaFibrotic Non-specific Interstitial PneumoniaCellularImages courtesy of Kevin O. Leslie, MD2022-3-17100NSIP in a 69-year-old woman. Thi

23、n-section CT scandemonstrates areas of air-space consolidation (straight arrows) with a predominantly peribronchovascular distribution. Focal areas of ground-glass attenuation (curved arrows) also are present. 2022-3-17101shows bilateral areas of ground-glass attenuation (arrows) with a predominantl

24、y central distribution. The 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 NSIP2022-3-171022022-3-171032022-3-171042022-3-171

25、052022-3-171062022-3-171072022-3-171082022-3-171092022-3-17110shows poorly defined arcadelike(拱廊样拱廊样) and polygonal(多角形的多角形的) opacities (perilobular pattern) in the left lower lobe in both subpleural and central regions of the lung. The opacities resemble thickened interlobular septa.Transverse thin

26、-section CT scan at the level of dome of the right hemidiaphragm in a 50-year-old woman with cryptogenic organizing pneumonia2022-3-17111Few abnormally thickened interlobular septa (arrowheads) are seen anteriorly in both upper lobes. These linear opacities are thinner and more sharply demarcatedTra

27、nsverse thin-section CT scan through upper lobes in a 49-year-old woman with cryptogenic organizing pneumonia2022-3-17112There is extensive 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 cryptog

28、enic organizing pneumoniaPerilobular opacities (arrows) are seen abutting(邻接的邻接的) the pleural surface in the left lower lobe2022-3-17113The perilobular opacities (arrows) in right lower lobe are centrally located and surrounded by aerated lung parenchyma.Transverse thin-section CT scan through lower

29、 lobes in a 44-year-old man with cryptogenic organizing pneumonia2022-3-17114Perilobular opacity (arrow) not immediately obvious is located 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-sec

30、tion CT scan at the level of lung base in a 61-year-old man with cryptogenic organizing pneumonia2022-3-17115There is perilobular opacity (arrowhead) and dilatation and distortion of airways (arrow) indicating presence of interstitial fibrosis. Transverse thin-section CT scan through lower lobes in

31、a 37-year-old woman with cryptogenic organizing pneumonia2022-3-17116patchy 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. 2022-3-171172022-3-171182022-3-1711

32、92022-3-171202022-3-171212022-3-171222022-3-171232022-3-171242022-3-171252022-3-171262022-3-171272022-3-171282022-3-17129Thin-section CT scan obtained at the level of the dome of the right hemidiaphragm demonstrates diffuse, bilateral areas of ground-glass attenuation (arrows). 2022-3-171302022-3-17

33、1312022-3-17132shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows). Sjgren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. Photomicrograph (original magnification, x20; H-E stain)2022-3-171332022-3-171342022-3-

34、171352022-3-17136 centrilobular nodules and branching linear structures (straight arrow) in the right 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 obtai

35、ned at the level of the carina 2022-3-171372022-3-171382022-3-17139diffusely distributed areas with airspace consolidation and ground-glass attenuation. Note thickened bronchovascular bundles (arrowheads) and interlobular septal thickening (arrows). Drug-induced eosinophilic pneumonia in a 56-year-o

36、ld man. Transverse thin-section CT scan through the right upper lobe2022-3-17140patchy ground-glass 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 scan2022-3-171412022-3-1

37、7142ground-glass attenuation and irregular linear hyperattenuating areas in the subpleural areas 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-

38、mm collimation) CT scan obtained at the level of the inferior pulmonary vein2022-3-17143patchy 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 collimati

39、on) CT scan obtained at the level of the inferior pulmonary vein2022-3-17144NL = normal lungff = fibroblastic foci Copyright 2004-2005 The France Foundation2022-3-171452022-3-171462022-3-171472022-3-171482022-3-171492022-3-17150N Engl J Med 1997; 336:1224-1234 2022-3-17151Pl = pleuraBV = bronchovasc

40、ular bundles Copyright 2004-2005 The France Foundation2022-3-171522022-3-171532022-3-171542022-3-171552022-3-171562022-3-171572022-3-17158cysts of somewhat uniform size2022-3-171592022-3-171602022-3-171612022-3-17162shows fine reticulonodular pattern in the upper lobes, with relative sparing of the

41、lower lobes. Langerhans cell histiocytosis. (a) Transverse thin-section CT image2022-3-17163shows marked dilatation of the main pulmonary artery (arrowheads) relative to the ascending aorta (a), indicating severe pulmonary hypertension. Langerhans cell histiocytosis. ( (b) Transverse contrast-enhanc

42、ed CT image2022-3-17164shows Langerhans cell histiocytosis nodule involving 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 patient2022-3-17165demonstrates extensive areas of airspace consolidation (large arrows) and ground-glass attenuation (small arrows) involving mainly the per