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1 PFF HEALTH CARE PROFESSIONAL WEBINAR SERIES Welcome!

2 AGENDA TOPICS Welcome & Introduction Dr. Gregory Cosgrove, MD Chief Medical Officer Pulmonary Fibrosis Foundation PFF Resources Dolly Kervitsky, RCP, CCRC Vice President Patient Relations and Medical Affairs Presentation: Making an Accurate Diagnosis and How to Use the IPF Consensus Guidelines Dr. Fernando J. Martinez, MD, MS PFF Medical Advisory Board Member Executive Vice Chair of Medicine Weill Cornell Medical College Q & A Adjournment 2013 Pulmonary Fibrosis Foundation. All rights reserved. 2

3 PFF RESOURCES 2013 Pulmonary Fibrosis Foundation. All rights reserved. 3

4 RESOURCES

5 EDUCATIONAL + AWARENESS MATERIALS Breathe Bulletin Pulmonary Fibrosis Patient Information Guide * Support Group Leader Guide * Understanding PF brochures * Understanding PF posters * Physician notepads * Webinars + DVD s * * Available in multiple languages 2013 Pulmonary Fibrosis Foundation. All rights reserved. 5

6 ANNOUNCEMENTS Apply today! Application period closes August 8 th WASHINGTON D. C.

7 ANNOUNCEMENTS Apply today! Application period closes July 18 th

8 PFF HEALTH CARE PROFESSIONAL WEBINAR SERIES July 16, :30 p.m. 1:30 p.m. CDT Making an Accurate Diagnosis How to Use the IPF Consensus Guidelines Presented by Dr. Fernando J. Martinez, MD, MS PFF Medical Advisory Board Member Executive Vice Chair of Medicine Weill Cornell Medical College

9 Using guidelines to diagnose IPF Fernando J. Martinez, M.D., M.S

10 Disclosures Consultant for Able, Axon, Merck, BI, GSK, Ikaria Advisor for Amgen, Pfizer, Carden Jennings, Forest, GSK, Ikaria Speaker for BI, Nycomed, Stromedix Investigator for Forest, Gilead, Janssen, GSK, Nycomed, Stromedix CME contributor for CME Incite, NCME, NACE, Peer Voice, PIK, St. Johns, St. Mary, Inova, Up to Date

11 IPF confers a poor prognosis Parameter HR (95% CI) IPF diagnosis (5.5, 147) Age 0.99 (0.95, 1.03) Female sex 0.31 (0.13, 0.72) Smoker 0.30 (0.13, 0.72) Physio CRP 1.06 (1.01, 1.11) Onset Sx (yrs) 1.02 (0.93, 1.12) CTfib score > (0.29, 2.04) Flaherty et al. Eur Respir J. 2002;19:

12 Percent dead PANTHER- IPF: Prednisone, Azathioprine, N- acetylcysteine: A study That Evaluates Response in Idiopathic Pulmonary Fibrosis HR 9.26 (95% CI ) Triple therapy Matched placebo Weeks IPF Net; NEJM 2012; 366:

13 New therapeutic options are imminently on horizon Pirfenidone Nintedanib King TE Jr et al. NEJM 2014; 370: Richeldi L et al. NEJM 2014; 370:

14

15 Algorithm for Clinical Radiologic Classification and Diagnosis of IPF History, physical exam, CXR/HRCT, PFTs, 6 minute walk test, blood work/serological studies Not IIP Collagen vascular disease, environmental, drug-related, other causes Possible IIP HRCT Confident HRCT Dx of IPF with consistent clinical features Atypical clinical or HRCT features for IPF Features of another DPLD (eg, PLCH) Suspected other DPLD Surgical lung/vats Bx If non-diagnostic TBBX/BAL or additional tests UIP NSIP RB DIP DAD COP LIP Non-IIP Confirmed ATS/ETS. Am J Respir Crit Care Med. 2002;165:

16 Criteria for Diagnosing IPF in the Absence of Lung Biopsy Major criteria Exclusion of other known causes of ILD Abnormal pulmonary function tests that include evidence of restriction and impaired gas exchange Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scan Transbronchial lung biopsy or BAL without features to support an alternative diagnosis Minor criteria Age > 50 yr Insidious onset of otherwise unexplained dyspnea on exertion Duration of illness > 3 mo Bibasilar, inspiratory Velcro-like crackles All major criteria and at least 3 of the minor criteria must be present to increase the likelihood of an IPF diagnosis ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165: ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:

17 Updated Consensus Statement for Diagnosis of IPF The diagnosis of IPF requires: Exclusion of other known causes of interstitial lung disease Presence of UIP pattern on HRCT (in patients without surgical biopsy) A HRCT pattern of definite/possible UIP with a Surgical lung biopsy showing Definite/Probable UIP The Major and Minor Criteria proposed in the 2000 ATS/ERS Consensus Statement were Eliminated Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

18 Classification of Diffuse Parenchymal Pulmonary Disorders Diffuse parenchymal lung disease (DPLD) DPLD of known cause Idiopathic InterstitiaI pneumonia Granulomatous DPLD Other DPLD Idiopathic UIP = IPF Non-UIP IIP Is it idiopathic? DIP RBILD AIP COP (BOOP) NSIP LIP American Thoracic Society. Am J Respir Crit Care Med. 2002;165:

19 Classification of Diffuse Parenchymal Pulmonary Disorders Diffuse parenchymal lung disease (DPLD) DPLD of known cause Idiopathic InterstitiaI pneumonia Granulomatous DPLD Other DPLD Idiopathic UIP = IPF Non-UIP IIP Or of known cause? DIP AIP RBILD COP (BOOP) NSIP LIP American Thoracic Society. Am J Respir Crit Care Med. 2002;165:

20 Updated Consensus Statement for Diagnosis of IPF The diagnosis of IPF requires: Exclusion of other known causes of interstitial lung disease Presence of UIP pattern on HRCT (in patients without surgical biopsy) A HRCT pattern of definite/possible UIP with a Surgical lung biopsy showing Definite/Probable UIP The Major and Minor Criteria proposed in the 2000 ATS/ERS Consensus Statement were Eliminated Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

21 Survival UIP Associated With Collagen Vascular Disease (CVD) Is Associated With Improved Prognosis and is not considered idiopathic pulmonary fibrosis Collagen vascular disease P = Idiopathic Years Flaherty et al; AJRCCM 2003; 167: Park et al; AJRCCM 2007; 175:

22 RA-UIP may have outcome similar to IPF RA-UIP versus non RAUIP, p 0.015; RA-UIP versus CVD-NSIP, p 0.043; RA-UIP versus I-NSIP, not significant; RA-UIP versus IPF/UIP, not significant. RA-UIP Park et al; AJRCCM 2007; 175:

23 UCTD is Common in patients with suspected IIP 101 pts with clinical/hrct/slb material UCTD present in 14/45 (31%) NSIP and 7/56 (13%) UIP, p=0.02 Corte et al., Eur Resp J Sep 2011 epub

24 Significance of UCTD uncertain HR (95% CI) p value UCTD 1.07 (0.54, 2.10) 0.85 Survival Age 1.01 (0.99, 1.05) 0.24 Female 0.48 (0.25, 0.90) 0.02 Raynaud s 0.65 (0.26, 1.64) 0.36 Any Serology 0.68 (0.37, 1.26) 0.22 CPI 1.04 (1.02, 1.06) < p = NS UIP biopsy 3.71 (2.00, 6.89) < Treated with steroids +/- cytotoxic agent Corte et al., Eur Resp J Sep 2011 epub

25 Frequency of CHP in patients previously diagnosed with IPF Original cohort 2000 ATS/ERS criteria 2011 ATS/ERS/JRS /ALAT criteria Final diagnoses 305 with ILD 60 IPF* 245 without HRCT UIP 46 IPF 14 non- UIP 26 IPF 20 CHP* * - 41 HRCT diagnosis * - 9 BCT; 7 IgG/SLB 1 IgG/BAL; 3 SLB Morell et al., Lancet Respir Med 2013; 1:

26 Updated Consensus Statement for Diagnosis of IPF The diagnosis of IPF requires: Exclusion of other known causes of interstitial lung disease Presence of UIP pattern on HRCT (in patients without surgical biopsy) A HRCT pattern of definite/possible UIP with a Surgical lung biopsy showing Definite/Probable UIP The Major and Minor Criteria proposed in the 2000 ATS/ERS Consensus Statement were Eliminated Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

27 HRCT Criteria for UIP Pattern UIP Pattern (All 4 Features) Possible UIP (All 3 Features) Inconsistent with UIP (any) Subpleural basal predominance Reticular abnormality Honeycombing with/without traction bronchiectasis Absence of features listed as inconsistent with UIP (column three) Subpleural, basal predominance Reticular abnormality Absence of features listed as inconsistent with UIP (column three) Upper or mid-lung predominance Peribronchovascular predominance Extensive ground glass abnormality (extent > reticular abnormality) Profuse micronodules (bilateral, predominantly upper lobe) Discrete cysts (multiple, bilateral, away from areas of honeycombing) Diffuse mosaic attenuation/air-trapping (bilateral, in three or more lobes) Consolidation in bronchopulmonary segment(s)/lobe(s) Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

28 Diagnostic criteria for IPF HRCT pattern SLB (when performed) IPF Diagnosis? UIP Probable UIP Inconsistent with UIP UIP Probable UIP Possible UIP Nonclassifiable fibrosis Not UIP UIP Probable UIP Possible UIP Noclassifiable fibrosis Not UIP UIP Probable UIP Possible UIP Nonclassifiable fibrosis Not UIP Yes No Yes Probable No Possible No Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

29 UIP: Honeycombing Flaherty et al. Thorax. 2003; 58:

30 Usual Interstitial Pneumonitis Subpleural and Basal Predominance

31 Accuracy of diagnosis of UIP Study Correctness of first choice diagnosis Correctness of confident first choice Mathieson 89% 95% Lee 88% 100% Swensen 89% 100% Hunninghake 85% 96%

32 Diagnostic criteria for IPF HRCT pattern SLB (when performed) IPF Diagnosis? UIP Possible UIP Inconsistent with UIP UIP Probable UIP Possible UIP Nonclassifiable fibrosis Not UIP UIP Probable UIP Possible UIP Noclassifiable fibrosis Not UIP UIP Probable UIP Possible UIP Nonclassifiable fibrosis Not UIP Yes No Yes Probable No Possible No Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

33 Histological correlates in the ARTEMIS IPF study shed insight on HRCT findings Study cohort HRCT and SLB available HRCT diagnoses SLB definite or probable UIP (PPV) 1087 subjects screened for trial 315 with HRCT and SLB 772 UIP (n=84) Possible UIP (n=108) 81 (96.4%) 108 (97.2%) Inconsistent with UIP (n=98) 12 (18.3%) Raghu G, et al Lancet Respir Med 2014; 2:

34 Accuracy of diagnosis of UIP Study Correctness of first choice diagnosis Correctness of confident first choice % of UIP cases with confident diagnosis Mathieson 89% 95% 72% Lee 88% 100% 71% Swensen 89% 100% 67% Hunninghake 85% 96% 48%

35 Usual Interstitial Pneumonia

36 BUT

37 The presence of UIP (Concordant or Discordant) Confers a Poor Prognosis Statistically equal Flaherty, et al AJRCCM :

38

39 Multidisciplinary approach to diagnosis acknowledged as a major advance Category CRP Dx Radiologic/ pathologic pattern Chronic fibrosing IP Smoking related IPF Acute/subacute IP IPF insip RBILD DIP COP AIP UIP NSIP RB DIP OP DAD Travis et al., Am J Respir Crit Care Med 2013; 188:

40 The Clinical Radiographic and Pathologic Diagnosis of IIP: Clinical Gold Standard Clinician Radiologist Pathologist Multidisciplinary communication is essential to an accurate diagnosis Raghu et al., Am J Respir Crit Care Med 2011; 183:788-24

41 Agreement (k) Step Clinico/Radiological/Pathological Evaluation of 79 Consecutive IIP Patients Assessment Method Information Provided 1 Individual HRCT 2 Individual 3 Group 4 Group 5 Consensus HRCT, clinical data HRCT, clinical data HRCT, clinical data, SLB HRCT, clinical data, SLB Step Flaherty KR, et al. Am J Respir Crit Care Med. 2004;170:

42 Clinico/Radiological/Pathological Evaluation: The new gold standard Talmadge King Ganesh Raghu Kevin Brown Athol Wells Ron DuBois Victor Thannickal James Vyskocil Frazier Wadenstorer Jeffrey Wilt William Travis Thomas Colby Andrew Flint Andrew Nicholson N. Basily Robert Knapp Suspected IIP Clinicians HRCT review without history Radiologists review without history Stage 1 Clinicians & Radiologists review with clinical history Stage 2 Clinicians and Radiologists discuss Clinical & radiologic findings Stage 3 Clinicians & Radiologists review with pathologists impression Stage 4 Final consensus diagnoses Stage 5 Ella Kazerooni David Lynch Jeffrey Quick Edmund Louvar Flaherty et al., Am J Respir Crit Care Med. 2007; 175:

43 Final Dx Kappa Academic radiologists compared with community-based radiologists Academic Radiologist Kappa 0.55 Community Radiologist Kappa 0.32 Academic 1 Academic 2 Community Community Flaherty et al., Am J Respir Crit Care Med. 2007; 175:

44 Proportion with IPF diagnosis Clinico/Radiological/Pathological Evaluation: Radiologists Community Academic Stage1 Stage 2 Stage 3 Stage 4 Stage 5 Flaherty et al., Am J Respir Crit Care Med. 2007; 175:

45 Final Dx Kappa - Pathologists Academic Pathologists Kappa 0.57 Community Pathologists Kappa 0.41 Acad 1 Acad 2 Acad 3 Acad 4 Comm Comm Flaherty et al., Am J Respir Crit Care Med. 2007; 175:

46 Final Dx Kappa - Clinicians Academic Clinicians Kappa 0.71 Community Clinicians Kappa 0.44 Acad 1 Acad 2 Acad 3 Acad 4 Acad 5 Acad 6 Comm Comm Comm Flaherty et al., Am J Respir Crit Care Med. 2007; 175:

47

48 Increased age is discriminant for UIP Score Probability = [(0.084 * age * HRCT of Interstitial IPF Score 3.31)/5.856] (truncate negative values as zero; positive values greater than 1 as 1) Score PPV Specificity Sensitivity NPV Na > Fell et al., Am J Respir Crit Care Med 2010; 181:832-7

49 Significance of BAL lymphocytosis in IPF 101 patients with suspected IPF on HRCT 74 met 2002 ATS/ERS criteria for IPF BAL lymphocytosis > 30% in 6/74 (8%) 3 insip 3 EAA Ohshimo et al, AJRCCM 2009: 179:

50 So we have several additional options expand use of molecular markers In lung: In blood: Selman et al, AJRCCM 2005: 173: TBBx and SLB fibroblast cultures from patients with: No IIP NSIP UIP Exhibit similar phenotype Hogaboam (personal communication) Rosas et al; PLos Med 2008; 5: e93

51

52 Q & A 2013 Pulmonary Fibrosis Foundation. All rights reserved. 5 2

53 QUESTIONS 1. What are the best ways to assist IPF patients with self-management? 2. What is out in the medical field to assist with the effects of this disease? 3. What are the best questions for patients to ask their physicians and health care providers? 4. On first presentation, how to judge if the Interstitial Pneumonia is active or infective if patient is not producing sputum? Should BAL be performed after use of antibiotics or after use of steroids/immunosuppressants? 5. Does changing the primary place where you live make any sense for a patient? 6. Since IPF is a condition that affects the global community, how do we achieve consensus across the globe? 2013 Pulmonary Fibrosis Foundation. All rights reserved. 5 3

54 NEXT UP IN PFF DISEASE EDUCATION WEBINAR SERIES Patient & Caregiver Focused Webinar Tools for Living Better with PF: Pulmonary Rehabilitation and Support Groups Wednesday July 23 rd 12:00 p.m. to 1:00 p.m. CDT presented by: Chris Schumann, MS, RCEP, CES and Susan Jacobs, RN, MS This webinar will also include an interview with PFF Ambassador Doug Jones who will share the story of his journey with IPF Pulmonary Fibrosis Foundation. All rights reserved. 5 4

55 Thank you. Sponsored with the Generous Support of

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