特发性肺纤维化IPF

1、2022-7-212022-7-222022-7-232022-7-242022-7-252022-7-262022-7-272022-7-28DPLDKnown causese.g. drugs, CT dsPost-RT, occupation,HPIdiopathicInterstitialpneumoniasGranulomatouse.g. sarcoidosisOther formse.g. LAM, HXIPFOther than IPFDIPRB-ILDAIPCOPLIPNSIP2022-7-292022-7-2102022-7-2112022-7-2122022-7-2132

2、022-7-2142022-7-21550%YearsRespiratoryFunction/Symptoms1234FVCTraditional View of UIP/IPF ProgressionFVC = forced vital capacity2022-7-21650%YearsRespiratoryFunction/Symptoms123Acute exacerbationStep Theory of UIP/IPF ProgressionFVC042022-7-2172022-7-2182022-7-219Estimated 83,000 CurrentPatients in

3、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: CHEST 2002, November

4、2-7, 2002; San Diego, California.PrevalenceIncidence2022-7-2202022-7-2210.01.00 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 UIP (n = 51)Flaherty K

5、R, et al. Am J Respir Crit Care Med. 2001;164:1722-1727.2022-7-222ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2000;161:646-664.2022-7-2232022-7-2242022-7-2252022-7-2262022-7-2272022-7-2282022-7-2292022-7-2302022-7-2312022-7-2322022-7-2332022-7-234InjuryEpithelial cellsSlide courtesy of Pau

6、l Noble, MDCapillary2022-7-235Epithelial cellsCollagenMyofibroblastCell deathGrowth factors and other products of epithelial cell InjurySlide courtesy of Paul Noble, MDCapillary2022-7-2362022-7-2372022-7-2382022-7-2392022-7-2402022-7-2412022-7-242Courtesy of Kevin O. Leslie, MD.a. Peripheral accentu

7、ation of the diseaseb. Transition into uninvolved lungc. Low power pathologyd. High power image of fibroblastic focus Courtesy of Kevin O. Leslie, MD.2022-7-243ff = fibroblastic fociCourtesy of Fernando J. Martinez, MD.2022-7-2442022-7-2452022-7-246NSIP FibroticUIPCourtesy of Kevin O. Leslie, MD.202

8、2-7-247A 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/Phase2022-7-2482022-7-2492022-7-250Clinical History Physical Laboratory PFTsPrima

9、ry care physiciansPulmonologistsRadiologistsPathologistsMultidimensional and multidisciplinaryRadiology Chest X-ray HRCTPathology Surgical lung biopsy2022-7-2511970sInterstitial Lung DiseasesSarcoidosisHypersensitivity PneumonitisA heterogeneous group that included a number of diseases2002Asbestosis

10、LAMetcUIP/IPFDIPRB-ILDNSIPCOPLIPCellularFibroticAdapted from ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.AIP2022-7-2522022-7-2532022-7-254ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2002;165:277-304.ATS/ERS Consensus Statement. Am J Resp Crit Care Med. 2000;161:6

11、46-664. 2022-7-2552022-7-2562022-7-257Chang 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 morbidity and mortality than open lung biopsy - BAL and TBBx

12、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 procedure for patients with interstitial or focal lung di

13、sease- Diagnosis obtained in 61/62 patients- 72.5 % discharged home within 8 hours- 22.5% discharged home within 23 hours2022-7-2582022-7-2592022-7-2603)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：2022-7-261A normal chest x-ray does not exclude IPF ATS/ERS Consensus Statement. Am J Resp

14、ir Crit Care Med. 2000;161:646-664.2022-7-2622022-7-2632022-7-264ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.Prone scans are often best for showing early abnormalities2022-7-265Courtesy of David A. Lynch, MD.2022-7-266UIP: HoneycombingCourtesy of W. Richard Webb, MD.2022

15、-7-267UIP: Minimal HoneycombingCourtesy of W. Richard Webb, MD.Courtesy of W. Richard Webb, MD.UIP: Irregular Reticular Opacities2022-7-268UIP: Traction BronchiectasisCourtesy of W. Richard Webb, MD.2022-7-269UIP: Minimal Traction BronchiectasisCourtesy of W. Richard Webb, MD.2022-7-270Subpleural Pr

16、edominanceCourtesy of W. Richard Webb, MD.2022-7-271UIP in a 56-year-old man. Thin-section :ground-glass attenuation and localized intralobular reticular opacities (Red arrows). Mild honeycombing (black arrows) also is present2022-7-2722022-7-2732022-7-2742022-7-2752022-7-2762022-7-2772022-7-2782022

17、-7-279Pathology of IPF:Peripheral Accentuation of DiseaseSlide courtesy of Kevin Leslie, MD2022-7-280Slide courtesy of Kevin Leslie, MD2022-7-281Slide courtesy of Kevin Leslie, MD2022-7-282Slide courtesy of Kevin Leslie, MD2022-7-2832022-7-284Other idiopathic interstitial pneumonias NSIP AIP DIP COP

18、 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;161:646-664.2022-7-2852022-7-2862022-7-2872022-7-2882022-7-2892022-7-290AIP in a 48-year-old woman. Thin-section CT scan of the right lung obtained 2 cm below

19、the level of the tracheal carinademonstrates diffuse ground-glass attenuation and intralobular reticular opacities (arrows). 2022-7-291Acute 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-glass

20、 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. 2022-7-292Acute interstitial pneumonia in a 64-year-old woman. Thin-section CT scan obtained in

21、 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. 2022-7-2932022-7-2942022-7-2952022-7-29

22、62022-7-2972022-7-2982022-7-299 Non-specific Interstitial PneumoniaFibrotic Non-specific Interstitial PneumoniaCellularImages courtesy of Kevin O. Leslie, MD2022-7-2100NSIP in a 69-year-old woman. Thin-section CT scandemonstrates areas of air-space consolidation (straight arrows) with a predominantl

23、y peribronchovascular distribution. Focal areas of ground-glass attenuation (curved arrows) also are present. 2022-7-2101shows bilateral areas of ground-glass attenuation (arrows) with a predominantly central distribution. The reviewers believed that these findings were more compatible with alveolar

24、 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 NSIP2022-7-21022022-7-21032022-7-21042022-7-21052022-7-21062022-7-21072022-7-21082022-7-21092022-7-2110shows poorly defined arcadelike(拱廊样拱廊样) and poly

25、gonal(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-section CT scan at the level of dome of the right hemidiaphragm in a 50-year-old woman with cryptogenic organ

26、izing pneumonia2022-7-2111Few 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 pneumonia20

27、22-7-2112There 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 cryptogenic organizing pneumoniaPerilobular opacities (arrows) are seen abutting(邻接的邻接的) the pleural surface in the lef

28、t lower lobe2022-7-2113The 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 pneumonia2022-7-2114Perilobular opacity (arrow) not immed

29、iately 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-section CT scan at the level of lung base in a 61-year-old man with cryptogenic organizing pneumonia2022-7-2115There

30、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 a 37-year-old woman with cryptogenic organizing pneumonia2022-7-2116patchy bilateral air-space consolidation (open

31、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. 2022-7-21172022-7-21182022-7-21192022-7-21202022-7-21212022-7-21222022-7-21232022-7-21242022-7-21252022-7-21262022-7-21272022-7-21282022-7-2129Thin-sec

32、tion CT scan obtained at the level of the dome of the right hemidiaphragm demonstrates diffuse, bilateral areas of ground-glass attenuation (arrows). 2022-7-21302022-7-21312022-7-2132shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows)

33、. Sjgren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. Photomicrograph (original magnification, x20; H-E stain)2022-7-21332022-7-21342022-7-21352022-7-2136 centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts (curv

34、ed 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 2022-7-21372022-7-21382022-7-2139diffusely distributed areas with airspace consolidation and ground-glass

35、 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 lobe2022-7-2140patchy ground-glass opacity with interlobular septal lines a

36、nd centrilobular nodules and branching lines (arrows). Isoniazid-induced pneumonitis in a 45-year-old woman. Transverse thin-section CT scan2022-7-21412022-7-2142ground-glass attenuation and irregular linear hyperattenuating areas in the subpleural areas of both lower lung zones, as well as traction

37、 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 vein2022-7-2143patchy areas of ground-glass attenuation and consolidat

38、ion 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 vein2022-7-2144NL = normal lungff = fibroblastic foci Copyright 2004-2005 The Franc

39、e Foundation2022-7-21452022-7-21462022-7-21472022-7-21482022-7-21492022-7-2150N Engl J Med 1997; 336:1224-1234 2022-7-2151Pl = pleuraBV = bronchovascular bundles Copyright 2004-2005 The France Foundation2022-7-21522022-7-21532022-7-21542022-7-21552022-7-21562022-7-21572022-7-2158cysts of somewhat un

40、iform size2022-7-21592022-7-21602022-7-21612022-7-2162shows fine reticulonodular pattern in the upper lobes, with relative sparing of the lower lobes. Langerhans cell histiocytosis. (a) Transverse thin-section CT image2022-7-2163shows marked dilatation of the main pulmonary artery (arrowheads) relat

41、ive to the ascending aorta (a), indicating severe pulmonary hypertension. Langerhans cell histiocytosis. ( (b) Transverse contrast-enhanced CT image2022-7-2164shows 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