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Biopsy is necessary for the diagnosis of IPF

Biopsy is necessary for the diagnosis of IPF. Nesrin Moğulkoç Pulmonary Medicine. A TS /E RS International Multidisciplinary Consensus Classification Of Idiopathic Interstitial Pneumonias General Principles and Recommendations. Co-chairs: William D. Travis, M.D.

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Biopsy is necessary for the diagnosis of IPF

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  1. Biopsy is necessary for the diagnosis of IPF NesrinMoğulkoç Pulmonary Medicine

  2. ATS/ERS International Multidisciplinary Consensus Classification Of Idiopathic Interstitial PneumoniasGeneral Principles and Recommendations Co-chairs: William D. Travis, M.D. Talmadge King, Jr. M.D. Am J Respir Crit Care Med 2002; 165: 277

  3. Diffuse Parenchymal Lung Disease Idiopathic interstitial pneumonias DPLD of known cause (e.g. drugs, dust exposure, collagen vascular disease) Granulomatous DPLD (e.g. sarcoidosis) Other forms of DPLD (e.g. LAM, HX, eosin. pneum.) Idiopathic Iinterstitial Pneumonia other than IPF Idiopathic pulmonary fibrosis (IPF) Respiratory bronchiolitis/ Interst. lung dis. (RBILD) Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Cryptogenic organising pneumonia (COP) Lymphocytic interstitial pneumonia (LIP) Nonspecific interstitial pneumonia (NSIP)

  4. Proportion of patients with IPF, NSIP, DIP/RBILD and COP among IIPs SeriesIPF NSIP DIP/RBILD COP Bjoraker et al. 62% 14% 10% 2% 1998 Nagai et al. 58% 28% - 14% 1998 Travis et al. 55% 29% 16% - 2000 Nicholsonet al. 47% 36% 17% - 2000

  5. %40-50 %47-64 <%2 <%2 %4-12 %10-17 %14-36 Diffuse Parenchymal Lung Disease Idiopathic interstitial pneumonias DPLD of known cause (e.g. drugs, dust exposure, collagen vascular disease) Granulomatous DPLD (e.g. sarcoidosis) Other forms of DPLD (e.g. LAM, HX, eosin. pneum. etc.) IIP other than idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis (IPF) Respiratory bronchiolitis/ Interst. lung dis. (RBILD) Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Cryptogenic organising pneumonia (COP) Lymphocytic interstitial pneumonia (LIP) Nonspecific interstitial pneumonia (NSIP)

  6. to provide a specific diagnosis Biopsy is necessary in IPF

  7. History, physical exam, clinical chemistry, PFT, Chest Xray Possible IIP Not IIP HRCT

  8. Pathology HRCT Lymphangitiscarcinomatosa Lymphoma Sarcoidosis Septal thickening UIP NSIP Irregular reticular / linear opacities Collagen vascular disease Asbestosis Cystic airspaces / honeycombing Hypersensitivity pneumonitis Lymphangioleiomyomatosis Langerhans’ cell histiocytosis Nodules Miliary TB Fungal infection Ground-glass attentuation RB/ILD DIP Consolidation LIP Alveolar proteinosis Chronic eosinophilic pneumonia BOOP/COP DAD/ARDS

  9. History, physical exam, clinical chemistry, PFT, Chest Xray Possible IIP Not IIP HRCT Typical of IPF (~50%) ∅ IPF (~50%) suggestive of Other ILD ? specific ILD

  10. HRCT Criteria of IPF • Reticular abnormality and/or traction bronchiectasis with basal and peripheral predominance • Honeycombing with basal and peripheral predominance • Atypical features are absent • Micronodules are not present • Peribronchovascular nodules are not present • Consolidation is not present • Ground glass attenuation, if present, is less extensive than reticular opacity • Mediastinal adenopathy, if present, is not extensive enough to be visible on chest X-ray Definite IPF: all 3 are met Probable IPF: 1 and 3 are met

  11. UIP: Progression of Fibrosis on HRCT Late: Extensive Honeycombing Early: Reticular Moderate: Subpleural Honeycombing

  12. Honeycombing

  13. Honeycombing (5%)

  14. Honeycombing (5%)

  15. Rating of κ scores Landis JR, Koch GG. 1977

  16. Kappa coefficients of agreement between 11 radiologists for HRCT diagnosis(Aziz et al. Thorax 2004)

  17. Accuracy of Clinical & Radiological Diagnosis of IPF • 59 patients with surgical biopsies • clinical diagnosis or radiological diagnosis • clinical diagnosis of IPF - 97% specific - 62% sensitive • HRCT diagnosis of IPF -90% specific -79% sensitive Raghu et al, 1999

  18. Accuracy of HRCT Diagnosis in IPF A confident diagnosis is made in only about two-thirds of patients with IPF

  19. History, physical exam, clinical chemistry, PFT, Chest Xray Not IIP Possible IIP HRCT Typical of IPF (~50%) suggestive of Other ILD ? ∅ IPF (~50%) specific ILD Ø IPF TBBx TBBx No TBBx diagnosis BAL BAL BAL Surgical lung biopsy (3 locations, min. 2cm3) UIP NSIP RB-ILD DIP DAD COP LIP ∅ IIP

  20. to assess disease activity Biopsy is necessary in IPF

  21. UIP: fibroblast foci

  22. Fibroblastic foci in UIP • Extent Of Fibroblastic foci Predict Mortality In Idiopathic Pulmonary FibrosisT.E. King Jr., AJRCCM 2001:164;1025-32. • The frequency of fibroblastic foci in usual interstitial pneumonia and their relationship to disease progressionNicholson AG, AJRCCM 2002; 166: 173-7. • Relationship between histopathologic features and course of IPF/UIPTitto L, Thorax 2006:61:1091-5. Site of initial injury that triggers fibrosing process?

  23. Survival in IPF patients categorised by fibroblastic foci score in the lung biopsy

  24. to predict prognosis and to identify a more treatable process than originally suspected Biopsy is necessary in IPF

  25. IPF: worst case of an ILD 100 80 60 Other 40 NSIP 20 IPF 0 0 2 4 6 8 10 12 14 16 18 Years Bjoraker JA Am J Respir Crit CareMed.1998;157:199

  26. Byp=NSIP & HRCT=NSIP/Udtm 100 80 Byp=UIP &, HRCT=NSIP/Udtm Bx-Diagnosis: NSIP:n = 23, UIP:n = 73 HRCT-Diagnosis: NSIP: n = 44, UIP:n = 27 Udtm: n = 25 60 Cumulative proportion surviving 40 Byp=UIP & HRCT=UIP 20 0 0 2 4 8 10 6 Follow up Time (years) Flaherty et al., Thorax, 2003

  27. UIP pattern (histology vs HRCT) Flaherty et al Thorax 2003 (median survival in years) • HRCT UIP 2.08 • SLBx UIP 3.99 • SLBx of UIP and HRCT of other/NSIP 5.76 • HRCT of NSIP and SLBx of NSIP >9 • Pattern of UIP on HRCT or Bx = poorer prognosis in IPF

  28. to recognise purer cohorts of patients with regard investigation of cause and treatment strategies Biopsy is necessary in IPF

  29. ‘Importance of the lung biopsy for drug trials’ Stricter criteria for IPF, greater understanding of the ‘entity’ NSIP and problems with overlap, need for confident diagnosis of UIP/IPF for international drug trials

  30. Biopsy may be necessary in ILDto exclude neoplastic and infectious processes that occasionally mimic chronic, progressive interstitial disease

  31. Conditions mistaken for ILD • Infection • Cancer • Lymphoma • BAC • lymphangitis carcinomatosa

  32. Does the interactive diagnostic process improve the interobserver agreement? Idiopathic Interstitial Pneumonia What Is the Effect of a Multidisciplinary Approach to Diagnosis? Kevin R. Flaherty, Talmadge E. King, Jr., Ganesh Raghu, Joseph P. Lynch III, Thomas V. Colby,William D. Travis, Barry H. Gross, Ella A. Kazerooni, Galen B. Toews, Qi Long, Susan Murray,Vibha N. Lama, Steven E. Gay, and Fernando J. Martinez Am J Respir Crit Care Med Vol 170. pp 904–910, 2004

  33. Organisational Scheme (Review of 58 cases)

  34. Interobserver agreement at each diagnostic step

  35. Interobserver agreement at each diagnostic step

  36. Idiopathic Interstitial Pneumonia Do Community and Academic Physicians Agree on Diagnosis? Kevin R. Flaherty, Adin-Cristian Andrei, Talmadge E. King, Jr., Ganesh Raghu, Thomas V. Colby, Athol Wells, Nadir Bassily, Kevin Brown, Roland du Bois, Andrew Flint, Steven E. Gay, Barry H. Gross, Ella A. Kazerooni, Robert Knapp, Edmund Louvar, David Lynch, Andrew G. Nicholson, John Quick, Victor J. Thannickal, William D. Travis, James Vyskocil, Frazer A. Wadenstorer, Jeffrey Wilt, Galen B. Toews, Susan Murray, and Fernando J. Martinez AJRCCM 2007; 175: 1054 – 1060

  37. Final Diagnosis Agreement:Different among Community/Academic Physicians? • n = 39 pat. with ILD, retrospective review • Agreement in final diagnosis among 6 groups academic/community clinicians/radiologists/pathologists • Final agreement was better within academic centers (kappa = 0.55 to 0.71) than within community centers (kappa = 0.32 to 0.44) Flaherty KR, et al. AJRCCM 2007; 175: 1054

  38. Surgical Lung BiopsySpecial risk in IPF! • 60 pat with UIP (46 idiopathic, 14 associated with collagen/vasc dis) from Mayo Clinic 1986 - 1995 • 10/60 (=17%) died within 30 days after surgical biopsy 3/16 (19%) after VATS 7/44 (16%) after thoracotomy and biopsy • All 10 who died had IPF, 5 of these were biopsied for accelerated progress Utz et al, ERJ 2001; 17: 175

  39. Mortality and Risk Factors for Surgical Lung Biopsy in IIP • 200 pat. with IIP (140 IPF, 46 NSIP, 14 COP), retrospective study • 4.3% died within 30 days after surgical biopsy, no difference between VATS or OLB no difference between IPF and other IIPs • Biopsy at time of acute exacerbation: mortality 29% vs 3% • DLCO<50%: mortality 11% vs 1.4% Park JH et al, Eur J Cardiothorac Surg 2007

  40. Biopsy is necessary for the diagnosis of IPF

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