PFF HEALTH CARE PROFESSIONAL WEBINAR SERIES. Welcome!

Size: px
Start display at page:

Download "PFF HEALTH CARE PROFESSIONAL WEBINAR SERIES. Welcome!"

Transcription

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

Progress in Idiopathic Pulmonary Fibrosis

Progress in Idiopathic Pulmonary Fibrosis Progress in Idiopathic Pulmonary Fibrosis David A. Lynch, MB Disclosures Progress in Idiopathic Pulmonary Fibrosis David A Lynch, MB Consultant: t Research support: Perceptive Imaging Boehringer Ingelheim

More information

Diffuse Interstitial Lung Diseases: Is There Really Anything New?

Diffuse Interstitial Lung Diseases: Is There Really Anything New? : Is There Really Anything New? Sujal R. Desai, MBBS, MD ESTI SPEAKER SUNDAY Society of Thoracic Radiology San Antonio, Texas March 2014 Diffuse Interstitial Lung Disease The State of Play DILDs Is There

More information

Diagnostic challenges in IPF

Diagnostic challenges in IPF Medicine, Nursing and Health Sciences Diagnostic challenges in IPF Dr Ian Glaspole Central and Eastern Clinical School, Alfred Hospital and Monash University March 2015 Disclosures Consultancy fees from

More information

Unpaid scientific collaborator & advisor with Veracyte, Inc.

Unpaid scientific collaborator & advisor with Veracyte, Inc. Diagnosis and Classification of Idiopathic Interstitial Pneumonias: Role of Histopathology in the Golden Age of Consensus Jeffrey L. Myers, M.D. A. James French Professor of Diagnostic Pathology Vice Chair

More information

IPF: Epidemiologia e stato dell arte

IPF: Epidemiologia e stato dell arte IPF: Epidemiologia e stato dell arte Clinical Classification Diffuse parenchimal lung diseases Exposure-related: - occupational - environmental - medication Desquamative interstitial pneumonia Idiopathic

More information

UIP Possibile e Probabile

UIP Possibile e Probabile UIP Possibile e Probabile Sergio Harari U.O. di Pneumologia e UTIR Servizio di Emodinamica e Fisiopatologia Respiratoria Ospedale San Giuseppe - Milano Current definition of IPF IPF is a distinct type

More information

IPF - Inquadramento clinico

IPF - Inquadramento clinico IPF - Inquadramento clinico Sergio Harari Unità Operativa di Pneumologia UTIR Servizio di Fisiopat. Resp. e Emodinamica Polmonare Ospedale S. Giuseppe, Milano Clinical Classification Diffuse parenchimal

More information

Outline Definition of Terms: Lexicon. Traction Bronchiectasis

Outline Definition of Terms: Lexicon. Traction Bronchiectasis HRCT OF IDIOPATHIC INTERSTITIAL PNEUMONIAS Disclosures Genentech, Inc. Speakers Bureau Tadashi Allen, MD University of Minnesota Assistant Professor Diagnostic Radiology 10/29/2016 Outline Definition of

More information

An earlier and more confident diagnosis of idiopathic pulmonary fibrosis

An earlier and more confident diagnosis of idiopathic pulmonary fibrosis Eur Respir Rev 2012; 21: 124, 141 146 DOI: 10.1183/09059180.00000812 CopyrightßERS 2012 REVIEW: IPF An earlier and more confident diagnosis of idiopathic pulmonary fibrosis Roland M. du Bois ABSTRACT:

More information

Liebow and Carrington's original classification of IIP

Liebow and Carrington's original classification of IIP Liebow and Carrington's original classification of IIP-- 1969 Eric J. Stern MD University of Washington UIP Usual interstitial pneumonia DIP Desquamative interstitial pneumonia BIP Bronchiolitis obliterans

More information

A Review of Interstitial Lung Diseases. Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco

A Review of Interstitial Lung Diseases. Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco A Review of Interstitial Lung Diseases Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco Outline Overview of diagnosis in ILD Why it is important Definition/Classification

More information

A Review of Interstitial Lung Diseases

A Review of Interstitial Lung Diseases Outline A Review of Interstitial Lung Diseases Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco Overview of diagnosis in ILD Why it is important Definition/Classification

More information

NONE OVERVIEW FINANCIAL DISCLOSURES UPDATE ON IDIOPATHIC PULMONARY FIBROSIS/IPF (UIP) FOR PATHOLOGISTS. IPF = Idiopathic UIP Radiologic UIP Path UIP

NONE OVERVIEW FINANCIAL DISCLOSURES UPDATE ON IDIOPATHIC PULMONARY FIBROSIS/IPF (UIP) FOR PATHOLOGISTS. IPF = Idiopathic UIP Radiologic UIP Path UIP UPDATE ON IDIOPATHIC PULMONARY FIBROSIS/IPF () FOR PATHOLOGISTS Thomas V. Colby, M.D. Professor of Pathology (Emeritus) Mayo Clinic Arizona FINANCIAL DISCLOSURES NONE OVERVIEW IPF Radiologic Dx Pathologic

More information

Case Presentations in ILD. Harold R. Collard, MD Department of Medicine University of California San Francisco

Case Presentations in ILD. Harold R. Collard, MD Department of Medicine University of California San Francisco Case Presentations in ILD Harold R. Collard, MD Department of Medicine University of California San Francisco Outline Overview of diagnosis in ILD Definition/Classification High-resolution CT scan Multidisciplinary

More information

Difficulties Diagnosing Idiopathic Pulmonary Fibrosis

Difficulties Diagnosing Idiopathic Pulmonary Fibrosis 1. er Encuentro Entre Neumólogos y Radiólogos, Madrid, Spain, 2016, October 14th Difficulties Diagnosing Idiopathic Pulmonary Fibrosis Simon Walsh King s College Hospital Foundation Trust London, United

More information

5/9/2015. Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective. No, I am not a pulmonologist! Radiology

5/9/2015. Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective. No, I am not a pulmonologist! Radiology Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective No, I am not a pulmonologist! Radiology Pathology Clinical 1 Everyone needs a CT Confidence in diagnosis Definitive HRCT +

More information

The radiological differential diagnosis of the UIP pattern

The radiological differential diagnosis of the UIP pattern 5th International Conference on Idiopathic Pulmonary Fibrosis, Modena, 2015, June 12th The radiological differential diagnosis of the UIP pattern Simon Walsh King s College Hospital Foundation Trust London,

More information

Imaging: how to recognise idiopathic pulmonary fibrosis

Imaging: how to recognise idiopathic pulmonary fibrosis REVIEW IDIOPATHIC PULMONARY FIBROSIS Imaging: how to recognise idiopathic pulmonary fibrosis Anand Devaraj Affiliations: Dept of Radiology, St George s Hospital, London, UK. Correspondence: Anand Devaraj,

More information

International consensus statement on idiopathic pulmonary fibrosis

International consensus statement on idiopathic pulmonary fibrosis Eur Respir J 2001; 17: 163 167 Printed in UK all rights reserved Copyright #ERS Journals Ltd 2001 European Respiratory Journal ISSN 0903-1936 PERSPECTIVE International consensus statement on idiopathic

More information

Medical Policy An independent licensee of the Blue Cross Blue Shield Association

Medical Policy An independent licensee of the Blue Cross Blue Shield Association Idiopathic Pulmonary Fibrosis Page 1 of 10 Medical Policy An independent licensee of the Blue Cross Blue Shield Association Title: Idiopathic Pulmonary Fibrosis (Esbriet /pirfenidone, Ofev /nintedanib)

More information

4/17/2010 C ini n ca c l a Ev E a v l a ua u t a ion o n of o ILD U dat a e t e i n I LDs

4/17/2010 C ini n ca c l a Ev E a v l a ua u t a ion o n of o ILD U dat a e t e i n I LDs Update in ILDs Diagnosis 101: Clinical Evaluation April 17, 2010 Jay H. Ryu, MD Mayo Clinic, Rochester MN Clinical Evaluation of ILD Outline General aspects of ILDs Classification of ILDs Clinical evaluation

More information

11/10/2014. Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective. Radiology

11/10/2014. Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective. Radiology Multi-disciplinary Approach to Diffuse Lung Disease: The Imager s Perspective Radiology Pathology Clinical 1 Role of HRCT Diagnosis Fibrosis vs. inflammation Next step in management Response to treatment

More information

Guidelines for Diagnosis and Treatment of IPF

Guidelines for Diagnosis and Treatment of IPF Guidelines for Diagnosis and Treatment of IPF Katerina Antoniou, MD, PhD Lecturer in Thoracic Medicine ERS ILD Group Secretary Medical School, University of Crete Classification of Interstitial Lung Disease

More information

Financial disclosure COMMON DIAGNOSES IN HRCT. High Res Chest HRCT. HRCT Pre test. I have no financial relationships to disclose. Anatomy Nomenclature

Financial disclosure COMMON DIAGNOSES IN HRCT. High Res Chest HRCT. HRCT Pre test. I have no financial relationships to disclose. Anatomy Nomenclature Financial disclosure I have no financial relationships to disclose. Douglas Johnson D.O. Cardiothoracic Imaging Gaston Radiology COMMON DIAGNOSES IN HRCT High Res Chest Anatomy Nomenclature HRCT Sampling

More information

Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines

Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines Rebecca Keith, MD Assistant Professor, Division of Pulmonary and Critical Care Medicine National Jewish Health, Denver, CO Objectives

More information

Non-neoplastic Lung Disease II

Non-neoplastic Lung Disease II Pathobasic Non-neoplastic Lung Disease II Spasenija Savic Prince Pathology Program Systematic approach to surgical lung biopsies with ILD Examples (chronic ILD): Idiopathic interstitial pneumonias: UIP,

More information

Overview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy

Overview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy Overview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy Jeff Swigris, DO, MS Director, ILD Program National Jewish Health Disclosures Speaker - Boehringer Ingelheim and Genentech Objectives Describe

More information

Case 4 History. 58 yo man presented with prox IP joint swelling 2 months later pain and swelling in multiple joints Chest radiograph: bi-basilar

Case 4 History. 58 yo man presented with prox IP joint swelling 2 months later pain and swelling in multiple joints Chest radiograph: bi-basilar Case 4 History 58 yo man presented with prox IP joint swelling 2 months later pain and swelling in multiple joints Chest radiograph: bi-basilar basilar infiltrates suggestive of pulmonary fibrosis Open

More information

Interstitial Lung Disease (ILD)

Interstitial Lung Disease (ILD) Interstitial Lung Disease (ILD) ILD comprises more than 130 distinct disorders Characterized by cellular proliferation, cellular infiltration, and/or fibrosis of the lung parenchyma not due to infection

More information

Disclosures. Fibrotic lung diseases: Basic Principles, Common Problems, and Reporting. Relevant financial relationships: None. Off-label usage: None

Disclosures. Fibrotic lung diseases: Basic Principles, Common Problems, and Reporting. Relevant financial relationships: None. Off-label usage: None Fibrotic lung diseases: Basic Principles, Common Problems, and Reporting Brandon T. Larsen, MD, PhD Senior Associate Consultant Department of Laboratory Medicine and Pathology Mayo Clinic Arizona Arizona

More information

DIFFERENCES IN FIBROPROLIFERATIVE HEALING IN EXOGENEOUS AND IDIOPATHIC ILDs. ARE THERE ANY?

DIFFERENCES IN FIBROPROLIFERATIVE HEALING IN EXOGENEOUS AND IDIOPATHIC ILDs. ARE THERE ANY? NIHR Southampton Respiratory Biomedical Research Unit DIFFERENCES IN FIBROPROLIFERATIVE HEALING IN EXOGENEOUS AND IDIOPATHIC ILDs. ARE THERE ANY? Fibrosing Interstitial Lung Diseases CPFS/WASOG/AIPO/ERS

More information

CTD-related Lung Disease

CTD-related Lung Disease 13 th Cambridge Chest Meeting King s College, Cambridge April 2015 Imaging of CTD-related Lung Disease Dr Sujal R Desai King s College Hospital, London Disclosure Statement No Disclosures / Conflicts of

More information

Radiologic pathologic discordance in biopsy-proven usual interstitial pneumonia

Radiologic pathologic discordance in biopsy-proven usual interstitial pneumonia ERJ Express. Published on February 25, 2016 as doi: 10.1183/13993003.01680-2015 ORIGINAL ARTICLE IN PRESS CORRECTED PROOF Radiologic pathologic discordance in biopsy-proven usual interstitial pneumonia

More information

Pathologic Assessment of Interstitial Lung Disease

Pathologic Assessment of Interstitial Lung Disease Pathologic Assessment of Interstitial Lung Disease Dry and itchy? It could be eczema or fungal infection. We don t need to worry, the drugs aren t that dangerous. Kirk D. Jones, MD UCSF Dept. of Pathology

More information

DIAGNOSTIC NOTE TEMPLATE

DIAGNOSTIC NOTE TEMPLATE DIAGNOSTIC NOTE TEMPLATE SOAP NOTE TEMPLATE WHEN CONSIDERING A DIAGNOSIS OF IDIOPATHIC PULMONARY FIBROSIS (IPF) CHIEF COMPLAINT HISTORY OF PRESENT ILLNESS Consider IPF as possible diagnosis if any of the

More information

Conflicts of Interest. Advisory Board: Boehringer-Ingleheim, Genentech/Roche DSMB: Bristol-Myers Squibb, Fibrogen Clinical Endpoint Committee; Merck

Conflicts of Interest. Advisory Board: Boehringer-Ingleheim, Genentech/Roche DSMB: Bristol-Myers Squibb, Fibrogen Clinical Endpoint Committee; Merck Conflicts of Interest Advisory Board: Boehringer-Ingleheim, Genentech/Roche DSMB: Bristol-Myers Squibb, Fibrogen Clinical Endpoint Committee; Merck The Idiopathic Interstitial Pneumonias Idiopathic pulmonary

More information

INTERSTITIAL LUNG DISEASE. Radhika Reddy MD Pulmonary/Critical Care Long Beach VA Medical Center January 5, 2018

INTERSTITIAL LUNG DISEASE. Radhika Reddy MD Pulmonary/Critical Care Long Beach VA Medical Center January 5, 2018 INTERSTITIAL LUNG DISEASE Radhika Reddy MD Pulmonary/Critical Care Long Beach VA Medical Center January 5, 2018 Interstitial Lung Disease Interstitial Lung Disease Prevalence by Diagnosis: Idiopathic Interstitial

More information

Connective Tissue Disorder- Associated Interstitial Lung Disease (CTD-ILD) and Updates

Connective Tissue Disorder- Associated Interstitial Lung Disease (CTD-ILD) and Updates Connective Tissue Disorder- Associated Interstitial Lung Disease (CTD-ILD) and Updates Maria Elena Vega, M.D Assistant Professor of Medicine Lewis Katz School of Medicine at Temple University Nothing to

More information

Usual Interstitial pneumonia and Nonspecific Interstitial Pneumonia. Nitra and the Gangs.

Usual Interstitial pneumonia and Nonspecific Interstitial Pneumonia. Nitra and the Gangs. Usual Interstitial pneumonia and Nonspecific Interstitial Pneumonia Nitra and the Gangs. บทน ำและบทท ๓, ๑๐, ๑๒, ๑๓, ๑๔, ๑๕, ๑๗ Usual Interstitial Pneumonia (UIP) Most common & basic pathologic pattern

More information

Summary: Key Learning Points, Clinical Strategies, and Future Directions

Summary: Key Learning Points, Clinical Strategies, and Future Directions Summary: Key Learning Points, Clinical Strategies, and Future Directions Introduction Idiopathic pulmonary fibrosis (IPF), a peripheral lobular fibrosis of unknown cause, is a chronic, progressive lung

More information

Idiopathic Pulmonary Fibrosis: The Importance of Qualitative and Quantitative Phenotyping

Idiopathic Pulmonary Fibrosis: The Importance of Qualitative and Quantitative Phenotyping Idiopathic Pulmonary Fibrosis: The Importance of Qualitative and Quantitative Phenotyping K. R. Flaherty Division of Pulmonary and Critical Care Medicine, University of Michigan Health System, Ann Arbor,

More information

Differential diagnosis

Differential diagnosis Differential diagnosis Idiopathic pulmonary fibrosis (IPF) is part of a large family of idiopathic interstitial pneumonias (IIP), one of four subgroups of interstitial lung disease (ILD). Differential

More information

Strategies for Updated Treatment Options for IPF

Strategies for Updated Treatment Options for IPF Strategies for Updated Treatment Options for IPF Paul W. Noble, MD Vera and Paul Guerin Family Distinguished Chair in Pulmonary Medicine Professor and Chair Department of Medicine Director, Women's Guild

More information

IPF AND OTHER FIBROSING LUNG DISEASE: WHAT DRUGS MIGHT WORK AND ON WHOM DO THEY W ORK?

IPF AND OTHER FIBROSING LUNG DISEASE: WHAT DRUGS MIGHT WORK AND ON WHOM DO THEY W ORK? IPF AND OTHER FIBROSING LUNG DISEASE: WHAT DRUGS MIGHT WORK AND ON WHOM DO THEY W ORK? KEVIN K. BROWN, MD PROFESSOR AND VICE CHAIRMAN, DEPARTMENT OF MEDICINE NATIONAL JEWISH HEALTH DENVER, CO Kevin K.

More information

Current diagnostic recommendations for ILD: The multidisciplinary meeting TSANZSRS ASM

Current diagnostic recommendations for ILD: The multidisciplinary meeting TSANZSRS ASM Medicine, Nursing and Health Sciences Current diagnostic recommendations for ILD: The multidisciplinary meeting Dr Ian Glaspole Central and Eastern Clinical School, Alfred Hospital and Monash University

More information

Criteria for confident HRCT diagnosis of usual interstitial pneumonia (UIP)

Criteria for confident HRCT diagnosis of usual interstitial pneumonia (UIP) Criteria for confident HRCT diagnosis of usual interstitial pneumonia (UIP) Assem El Essawy (1) & Amr A. Nassef (٢) Abstract Identification of interstitial pneumonia (IP) was mainly based on histological

More information

INTERSTITIAL LUNG DISEASES: FOCUS ON IDIOPATHIC PULMONARY FIBROSIS (IPF)

INTERSTITIAL LUNG DISEASES: FOCUS ON IDIOPATHIC PULMONARY FIBROSIS (IPF) INTERSTITIAL LUNG DISEASES: FOCUS ON IDIOPATHIC PULMONARY FIBROSIS (IPF) Marilyn K. Glassberg Csete, M.D. Professor of Medicine, Surgery, and Pediatrics Director, Interstitial and Rare Lung Disease Program

More information

Update on Therapies for Idiopathic Pulmonary Fibrosis. Outline

Update on Therapies for Idiopathic Pulmonary Fibrosis. Outline Update on Therapies for Idiopathic Pulmonary Fibrosis Paul Wolters Associate Professor University of California, San Francisco Outline Classification of Interstitial lung disease Clinical classification

More information

Manish Powari Regional Training Day 10/12/2014

Manish Powari Regional Training Day 10/12/2014 Manish Powari Regional Training Day 10/12/2014 Large number of different types of Interstitial Lung Disease (ILD). Most are very rare Most patients present with one of a smaller number of commoner diseases

More information

Daria Manos RSNA 2016 RC 401. https://medicine.dal.ca/departments/depar tment-sites/radiology/contact/faculty/dariamanos.html

Daria Manos RSNA 2016 RC 401. https://medicine.dal.ca/departments/depar tment-sites/radiology/contact/faculty/dariamanos.html Daria Manos RSNA 2016 RC 401 https://medicine.dal.ca/departments/depar tment-sites/radiology/contact/faculty/dariamanos.html STEP1: Is this fibrotic lung disease? STEP 2: Is this a UIP pattern? If yes:

More information

INVITED REVIEW SERIES: PULMONARY FIBROSIS SERIES EDITORS: MARTIN KOLB AND GERARD COX

INVITED REVIEW SERIES: PULMONARY FIBROSIS SERIES EDITORS: MARTIN KOLB AND GERARD COX INVITED REVIEW SERIES: PULMONARY FIBROSIS SERIES EDITORS: MARTIN KOLB AND GERARD COX Diagnosing fibrotic lung disease: When is high-resolution computed tomography sufficient to make a diagnosis of idiopathic

More information

Disclosures. Traditional Paradigm. Overview 4/17/2010. I have relationships with the following organizations and companies:

Disclosures. Traditional Paradigm. Overview 4/17/2010. I have relationships with the following organizations and companies: Disclosures Pharmacological Therapy for ILD What to Use and How to Use It Harold R Collard MD Interstitial Lung Disease Program University of California San Francisco (UCSF) I have relationships with the

More information

T he diagnostic evaluation of a patient with

T he diagnostic evaluation of a patient with 546 REVIEW SERIES Challenges in pulmonary fibrosis? 1: Use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias Michael B Gotway, Michelle M

More information

Challenges in the Diagnosis of Interstitial Lung Disease

Challenges in the Diagnosis of Interstitial Lung Disease Challenges in the Diagnosis of Interstitial Lung Disease Kirk D. Jones, MD UCSF Dept. of Pathology kirk.jones@ucsf.edu Overview New Classification of IIP Prior classification Modifications for new classification

More information

Challenges in the Diagnosis of Interstitial Lung Disease

Challenges in the Diagnosis of Interstitial Lung Disease Challenges in the Diagnosis of Interstitial Lung Disease Kirk D. Jones, MD UCSF Dept. of Pathology kirk.jones@ucsf.edu Overview New Classification of IIP Prior classification Modifications for new classification

More information

Idiopathic Pulmonary of Care

Idiopathic Pulmonary of Care Chapter 6.1 Living Medical etextbook A Digital Tool at the Point of Care From Projects In Knowledge Pulmonology Idiopathic Pulmonary Fibrosis @Point of Care IPF Case Study: Typical Presentation, Role of

More information

Wim Wuyts. Treatment of idiopathic interstitial pneumonias. March 12 th Interstitial lung diseases state of the art.

Wim Wuyts. Treatment of idiopathic interstitial pneumonias. March 12 th Interstitial lung diseases state of the art. nterstitial ungdiseases euven Department of pneumology Unit for interstitial lung diseases University Hospitals Leuven March 12 th 2015 Interstitial lung diseases state of the art Treatment of idiopathic

More information

Challenges in Pulmonary and Critical Care: 2018

Challenges in Pulmonary and Critical Care: 2018 Challenges in Pulmonary and Critical Care: 2018 Interstitial Lung Disease: Evolving Our Understanding of a Deadly Disease 1 Faculty Kevin Flaherty, MD, MS Professor in Pulmonary and Critical Care Medicine

More information

A case of a patient with IPF treated with nintedanib. Prof. Kreuter and Prof. Heussel

A case of a patient with IPF treated with nintedanib. Prof. Kreuter and Prof. Heussel A case of a patient with IPF treated with nintedanib Prof. Kreuter and Prof. Heussel Case Overview This case describes the history of a patient with IPF who, at the time of diagnosis, had symptoms typical

More information

IDIOPATHIC PULMONARY FIBROSIS Guidelines for Diagnosis and Management

IDIOPATHIC PULMONARY FIBROSIS Guidelines for Diagnosis and Management IDIOPATHIC PULMONARY FIBROSIS Guidelines for Diagnosis and Management An ATS Pocket Publication This publication was produced in collaboration with Boehringer Ingelheim Pharmaceuticals, Inc. GUIDELINES

More information

Diagnosing ILD. What is important in 2016? Chris Grainge

Diagnosing ILD. What is important in 2016? Chris Grainge Diagnosing ILD What is important in 2016? Chris Grainge Senior Staff Specialist Respiratory Medicine John Hunter Hospital Conjoint A/Prof University of Newcastle Conflict of interest I have acted as a

More information

Idiopathic Pulmonary Fibrosis Treatable and Not Idiopathic

Idiopathic Pulmonary Fibrosis Treatable and Not Idiopathic Idiopathic Pulmonary Fibrosis Treatable and Not Idiopathic Brett Ley, MD University of California San Francisco CTS 1/26/18 Disclosures Speaker s bureau honorarium from Genentech (makers of pirfenidone)

More information

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival Turk Thorac J 205; 6:4-20 DOI: 0.552/ttd.205.4584 ORIGINAL INVESTIGATION Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival Subramanian Natarajan,

More information

NAVIGATING the NEW ERA in IPF: Idiopathic Pulmonary Fibrosis

NAVIGATING the NEW ERA in IPF: Idiopathic Pulmonary Fibrosis NAVIGATING the NEW ERA in IPF: Idiopathic Pulmonary Fibrosis Mark J Rumbak, MD Division Director Pulmonary, Critical Care and Sleep Medicine Morsani College of Medicine University of South Florida, Tampa

More information

Pulmonary manifestations of CTDs Diagnosis, differential diagnosis and treatment

Pulmonary manifestations of CTDs Diagnosis, differential diagnosis and treatment Prague, June 2014 Pulmonary manifestations of CTDs Diagnosis, differential diagnosis and treatment Katerina M. Antoniou, MD, PhD As. Professor in Thoracic Medicine ERS ILD Group Secretary Medical School,

More information

Radiologic Approach to Smoking Related Interstitial Lung Disease

Radiologic Approach to Smoking Related Interstitial Lung Disease Radiologic Approach to Smoking Related Interstitial Lung Disease Poster No.: C-1854 Congress: ECR 2013 Type: Educational Exhibit Authors: K.-N. Lee, J.-Y. Han, E.-J. Kang, J. Kang; Busan/KR Keywords: Toxicity,

More information

Idiopathic interstitial pneumonias (IIPs) are a group of

Idiopathic interstitial pneumonias (IIPs) are a group of SYMPOSIA C. Isabela S. Silva, MD, PhD and Nestor L. Müller, MD, PhD Abstract: The idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unknown etiology characterized

More information

New Horizons The Future of IPF and ILD

New Horizons The Future of IPF and ILD New Horizons The Future of IPF and ILD Talmadge E. King, Jr., M.D. Julius R. Krevans Distinguished Professorship in Internal Medicine Chair, Department of Medicine University of California San Francisco

More information

IPF : Dalla Diagnosi alla Terapia

IPF : Dalla Diagnosi alla Terapia IV Forum di Pneumologia Interventistica Bologna 5-6 Giugno 2015 IPF : Dalla Diagnosi alla Terapia Sergio Harari U.O. di Pneumologia e UTIR Servizio di Emodinamica e Fisiopatologia Respiratoria Ospedale

More information

Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis

Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis Original Article Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis Xia Li 1, Chang Chen 2, Jinfu Xu 1, Jinming Liu 1, Xianghua

More information

Bronchoscopic lung cryobiopsy increases diagnostic confidence. in the multidisciplinary diagnosis of idiopathic pulmonary

Bronchoscopic lung cryobiopsy increases diagnostic confidence. in the multidisciplinary diagnosis of idiopathic pulmonary Bronchoscopic lung cryobiopsy increases diagnostic confidence in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis. Sara Tomassetti, Athol U Wells, Ulrich Costabel, Alberto Cavazza, Thomas

More information

New Therapies and Trials in IPF

New Therapies and Trials in IPF Conflict of interest disclosure I have the following real or perceived conflicts of interest that relate to this presentation: New Therapies and Trials in IPF Talmadge E. King, Jr., M.D. Julius R. Krevans

More information

Katerina M. Antoniou, MD, PhD As. Professor in Thoracic Medicine ERS ILD Group Secretary Medical School, University of Crete Prague, June 2014

Katerina M. Antoniou, MD, PhD As. Professor in Thoracic Medicine ERS ILD Group Secretary Medical School, University of Crete Prague, June 2014 Hypersensitivity pneumonitis: Causes, clinical course, diagnosis and differential diagnosis, treatment Katerina M. Antoniou, MD, PhD As. Professor in Thoracic Medicine ERS ILD Group Secretary Medical School,

More information

Presente e futuro della terapia della fibrosi polmonare idiopatica

Presente e futuro della terapia della fibrosi polmonare idiopatica Presente e futuro della terapia della fibrosi polmonare idiopatica Antonella Caminati U.O. di Pneumologia e Terapia Semi Intensiva Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare Osp.

More information

9/12/18. Emerging Challenges in Primary Care: Navigating the Maze of Interstitial Lung Disease: Improving Outcomes through Early Diagnosis

9/12/18. Emerging Challenges in Primary Care: Navigating the Maze of Interstitial Lung Disease: Improving Outcomes through Early Diagnosis Emerging Challenges in Primary Care: 2018 Navigating the Maze of Interstitial Lung Disease: Improving Outcomes through Early Diagnosis Faculty Kevin Flaherty, MD, MS Professor in Pulmonary and Critical

More information

Case 1 : Question. 1.1 What is the intralobular distribution? 1. Centrilobular 2. Perilymphatic 3. Random

Case 1 : Question. 1.1 What is the intralobular distribution? 1. Centrilobular 2. Perilymphatic 3. Random Interesting case Case 1 Case 1 : Question 1.1 What is the intralobular distribution? 1. Centrilobular 2. Perilymphatic 3. Random Case 1: Answer 1.1 What is the intralobular distribution? 1. Centrilobular

More information

Disclosures. Clinical Approach: Evaluating CTD-ILD for the pulmonologist. ILD in CTD. connective tissue disease or collagen vascular disease

Disclosures. Clinical Approach: Evaluating CTD-ILD for the pulmonologist. ILD in CTD. connective tissue disease or collagen vascular disease Disclosures Clinical Approach: Evaluating CTD-ILD for the pulmonologist Industry relationships: Actelion, atyr Pharma, Boehringer-Ingelheim, Genentech- Roche, Gilead Aryeh Fischer, MD Associate Professor

More information

UPDATE ON INTERSTITIAL LUNG DISEASE. Thomas V. Colby, M.D.

UPDATE ON INTERSTITIAL LUNG DISEASE. Thomas V. Colby, M.D. UPDATE ON INTERSTITIAL LUNG DISEASE Thomas V. Colby, M.D. FINANCIAL DISCLOSURES NONE Topics Discussed 1. Idiopathic pulmonary fibrosis (IPF) 2. Idiopathic interstitial pneumonias (IIPs) 3. Interstitial

More information

Lines and crackles. Making sense of ILD

Lines and crackles. Making sense of ILD Lines and crackles Making sense of ILD Case JM 65 year old male Gradual shortness of breath, going on over a year Some dry cough Ex-smoker, quit 10 years ago Crackles in the bases CXR presented Sent to

More information

Cryptogenic Organizing Pneumonia Diagnosis Approach Based on a Clinical-Radiologic-Pathologic Consensus

Cryptogenic Organizing Pneumonia Diagnosis Approach Based on a Clinical-Radiologic-Pathologic Consensus Cryptogenic Organizing Pneumonia Diagnosis Approach Based on a Clinical-Radiologic-Pathologic Consensus Poster No.: C-1622 Congress: ECR 2012 Type: Scientific Exhibit Authors: C. Cordero Lares, E. Zorita

More information

Integration and Evaluation of Clinical Decision Support Systems for Diagnosis Idopathics Pulmonary Fibrosis (IPF)

Integration and Evaluation of Clinical Decision Support Systems for Diagnosis Idopathics Pulmonary Fibrosis (IPF) Original Article Healthc Inform Res. 2010 December;16(4):260-272. pissn 2093-3681 eissn 2093-369X Integration and Evaluation of Clinical Decision Support Systems for Diagnosis Idopathics Pulmonary Fibrosis

More information

Hypersensitivity Pneumonitis Common Diagnostic and Treatment Dilemmas

Hypersensitivity Pneumonitis Common Diagnostic and Treatment Dilemmas Hypersensitivity Pneumonitis Common Diagnostic and Treatment Dilemmas Rishi Raj MD Director, Interstitial Lung Diseases Program Clinical Professor of Pulmonary and Critical Care Medicine Stanford University

More information

Annual Rheumatology & Therapeutics Review for Organizations & Societies

Annual Rheumatology & Therapeutics Review for Organizations & Societies Annual Rheumatology & Therapeutics Review for Organizations & Societies A Rheumatologist s Approach to Interstitial Lung Disease Outline ILD classification and patterns in CTD The clinical landscape and

More information

UNRAVELING THE ETIOLOGY OF FAMILIAL INTERSTITIAL PNEUMONIA: GENETIC INVESTIGATIONS OF A COMPLEX DISEASE. Anastasia Leigh Wise

UNRAVELING THE ETIOLOGY OF FAMILIAL INTERSTITIAL PNEUMONIA: GENETIC INVESTIGATIONS OF A COMPLEX DISEASE. Anastasia Leigh Wise UNRAVELING THE ETIOLOGY OF FAMILIAL INTERSTITIAL PNEUMONIA: GENETIC INVESTIGATIONS OF A COMPLEX DISEASE by Anastasia Leigh Wise University Program in Genetics and Genomics & Integrated Toxicology and Environmental

More information

Case Report Clinical Management of Acute Interstitial Pneumonia: ACaseReport

Case Report Clinical Management of Acute Interstitial Pneumonia: ACaseReport Case Reports in Pulmonology Volume 2012, Article ID 678249, 4 pages doi:10.1155/2012/678249 Case Report Clinical Management of Acute Interstitial Pneumonia: ACaseReport Yang Xia, 1, 2 Zhenyu Liang, 1 Zhenzhen

More information

I n 2002 the American Thoracic Society (ATS) and

I n 2002 the American Thoracic Society (ATS) and 1008 REVIEW SERIES Challenges in pulmonary fibrosis? 5: The NSIP/UIP debate Roland du Bois, Talmadge E King Jr... Among the idiopathic interstitial s, the two entities idiopathic pulmonary fibrosis (IPF)

More information

ESBRIET (pirfenidone) oral capsule and oral tablet OFEV (nintedanib) oral capsule

ESBRIET (pirfenidone) oral capsule and oral tablet OFEV (nintedanib) oral capsule OFEV (nintedanib) oral capsule Coverage for services, procedures, medical devices and drugs are dependent upon benefit eligibility as outlined in the member's specific benefit plan. This Pharmacy Coverage

More information

Bronchoalveolar Lavage and Histopathologic Diagnosis Based on Biopsy

Bronchoalveolar Lavage and Histopathologic Diagnosis Based on Biopsy Idiopathic Pulmonary Fibrosis Bronchoalveolar Lavage and Histopathologic Diagnosis Based on Biopsy JMAJ 46(11): 469 474, 2003 Yukihiko SUGIYAMA Professor, Division of Pulmonary Medicine, Department of

More information

TBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease (other than

TBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease (other than TBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease (other than PAP) BAL is not required as a diagnostic tool in patients

More information

CT in Idiopathic Pulmonary Fibrosis: Diagnosis and Beyond

CT in Idiopathic Pulmonary Fibrosis: Diagnosis and Beyond Cardiopulmonary Imaging Review Gruden CT of Idiopathic Pulmonary Fibrosis Cardiopulmonary Imaging Review James F. Gruden 1 Gruden JF FOCUS ON: Keywords: CT, diagnosis, high-resolution CT, idiopathic pulmonary

More information

ERS 2016 Congress Highlights Interstitial Lung Disease (ILD)

ERS 2016 Congress Highlights Interstitial Lung Disease (ILD) ERS 216 Congress Highlights Interstitial Lung Disease (ILD) London, UK September 3 rd 7 th 216 The 26 th European Respiratory Society International Congress, (ERS) the largest respiratory meeting in the

More information

Patient with FVC>90% predicted. Demosthenes Bouros, Vasilios Tzilas University of Athens

Patient with FVC>90% predicted. Demosthenes Bouros, Vasilios Tzilas University of Athens Patient with FVC>90% predicted Demosthenes Bouros, Vasilios Tzilas University of Athens CASE OVERVIEW A 63-year-old, male patient with progressive exertional dyspnoea lasting for 2 years and dry cough

More information

Careful histopathological evaluation has shown the traditionally clinical diagnosis of

Careful histopathological evaluation has shown the traditionally clinical diagnosis of Demystifying Idiopathic Interstitial Pneumonia Harold R. Collard, MD; Talmadge E. King, Jr, MD REVIEW ARTICLE Careful histopathological evaluation has shown the traditionally clinical diagnosis of idiopathic

More information

Radiological features of idiopathic interstitial pneumonia: a pictorial review

Radiological features of idiopathic interstitial pneumonia: a pictorial review Radiological features of idiopathic interstitial pneumonia: a pictorial review Poster No.: C-2012 Congress: ECR 2013 Type: Educational Exhibit Authors: M. Piccoli, F. Roccasalva, S. Palmucci, G. Cappello,

More information

Controversies in Clinical Trials. Pirfenidone for Idiopathic Pulmonary Fibrosis (IPF)

Controversies in Clinical Trials. Pirfenidone for Idiopathic Pulmonary Fibrosis (IPF) Controversies in Clinical Trials Pirfenidone for Idiopathic Pulmonary Fibrosis (IPF) Controversies to be highlighted by IPF Post-hoc analyses Story Primary end point selection Changing prespecified endpoints

More information

Hypersensitivity Pneumonitis: Epidemiology and Classification

Hypersensitivity Pneumonitis: Epidemiology and Classification Hypersensitivity Pneumonitis: Epidemiology and Classification Ulrich Costabel, MD University of Duisburg-Essen, Ruhrlandklinik Department of Pneumology/Allergy Objectives Definitions, Etiology Epidemiology

More information

I have no relevant conflicts of interest to disclose

I have no relevant conflicts of interest to disclose I have no relevant conflicts of interest to disclose Diffuse parenchymal lung disease (DPLD) and its associations Secondary lobular anatomy DPLD History, clinical findings, temporal evolution, and exposures

More information

In medicine, the term overlap is very common, to the extent that there is even a

In medicine, the term overlap is very common, to the extent that there is even a Case Report Payam Mehrian (MD) 1 Ali Cheraghvandi (MD) 2 Atousa Droudnia (PhD) 3 Firouzeh Talischi (MD) 4 Saeid Fallah Tafti (MD) *5 Shahram Kahkouee (MD) 6 Hamidreza Jamaati (MD) 7 1. Pediatric Respiratory

More information