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

Size: px
Start display at page:

Download "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"

Transcription

1 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)

2 BAL is not required as a diagnostic tool in patients with clinical features and HRCT appearances typical of IPF

3

4 EPIDEMIOLOGY OF IPF Incidence and Prevalence Prevalence of 20.2 cases per 100,000 for males and 13.2 cases per 100,000 for females Incidence has been estimated at 10.7 cases per 100,000 per year for males and 7.4 cases per 100,000 per year for females

5 POTENTIAL RISK FACTORS FOR IPF Cigarette Smoking Exposure to Commonly Prescribed Drugs Chronic Aspiration Environmental Factors Infectious Agents Genetic Predisposition to IPF. Familial IPF (FPF) is defined as at least two members of a primary biological family (parent, child, sibling) having clinical features of IPF that are confirmed histologically

6 Familial IPF Clinical findings, histopathology, and clinical course are indistinguishable between familial IPF and sporadic cases. Patients with familial IPF may be younger at diagnosis, but are otherwise indistinguishable from nonfamilial cases. IPF in families may be far more common than the 2% estimate that is currently reported, perhaps even 10-fold higher. The identification of the genetic basis of IPF in families may be the most effective method to identify the central pathogenetic features of IPF.

7 IDIOPATHIC PULMONARY FIBROSIS AND FAMILIAL PULMONARY FIBROSIS SURFACTANT PROTEIN C MUTATIONS AND LUNG FIBROSIS Interleukin-1 1 Receptor Antagonist and Tumor Necrosis Factor-α Complement Receptor 1 Surfactant Proteins A and B Angiotensin Converting Enzyme Transforming Growth Factor-1 Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis

8 Combined pulmonary fibrosis and emphysema (CPFE) A specific entity/syndrome? OR A subgroup of IPF with different prognosis? The diagnosis is based on findings of HRCT of the chest, which show either centrilobular emphysema or upper-zone bullous emphysema, associated in 90% of cases with very suggestive paraseptal emphysema and diffuse infiltrating fibrosing lung disease at the bases (subpleural reticular opacities, honeycomb images traction bronchiectasis)

9 Tobacco smoking Severe dyspnea CPFE Unexpected subnormal spirometry findings / preserved lung volumes Severely DLCO Hypoxemia at exercise Characteristic imaging features High probability of severe PAH PFTs may not be reliable for follow up of patients with CPFE

10 Cottin et al, Eur Respir J 2005 Lung volumes were normal in 42 out of 56 patients (75%) DLCO is markedly impaired The prevalence of pulmonary hypertension is particularly high in patients with CPFE Median survival was 6.1 yrs, a better survival than that reported in large studies of biopsy-proven UIP, but worse than emphysema alone

11 The prognosis of CPFE patients was better than that of IPF-alone patients The yearly decrease in VC and TLC of patients with CPFE is less than that of patients with IPF alone

12 CPFE 1) higher decrease in oxygen saturation during rest and exercise 2) a higher fibrosis HRCT scan score 3) a higher espap 4) lower median survival time IPF alone n=79 CPFE n=31 This dire prognosis seems to be at least partially associated with the development of severe pulmonary arterial hypertension

13 Natural History of the Clinical Progression of IPF The traditional view of IPF progression holds that a slow and steady decline in respiratory function ultimately leads to respiratory failure and death. Emerging evidence, however, has suggested that multiple injuries, or hits,, to the lung occur over a period of time, and these hits lead to acute exacerbations that result in periods of more rapid decline in lung function, which can ultimately result in death.

14 An n acute, clinically significant deterioration of unidentifiable cause in a patient with underlying IPF Only a subset of patients with IPF appears to develop acute exacerbations erbations

15 IPF is the most common form of IIP No therapy has been proven effective, and median survival from the time of diagnosis is approximately 3yrs Lung transplantation, which appears to improve survival in a subset of IPF patients, remains the only intervention. The natural history of IPF has been characterized as a steady, predictable decline in lung function over time. Some patients may experience a more precipitous course,, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF.

16 These studies are primarily retrospective case series and provide discordant estimates of incidence and mortality rates. The risk of an exacerbation does not appear to be linked to the level l of PFTs derangement, although in one series, patients with lower FVC had more hospitalizations during subsequent follow-up.

17 Diagnostic Criteria Previous or concurrent diagnosis of IPF Unexplained worsening or development of dyspnea within 30 days HRCT with new bilateral ground-glass glass abnormality and/or consolidation superimposed on a background consistent with UIP pattern No evidence of pulmonary infection by endotracheal aspirate or BAL Exclusion of alternative causes,, including the following: Left heart failure Pulmonary embolism Identifiable cause of acute lung injury

18

19

20

21

22

23

24

25 POTENTIAL ETIOLOGY 1. Acute exacerbations of IPF represent a distinct, pathobiological manifestation of the primary disease process, characterized by idiopathic lung injury 2. Acute exacerbations of IPF may represent clinically occult but biologically distinct conditions that go undiagnosed (e.g., viral infection, aspiration) 3. Acute exacerbations of IPF may be the sequelae of an acute direct stress to the lung,, with a subsequent acceleration of the already abnormal fibroproliferative process intrinsic to IPF

26 Endotracheal aspirate or BAL investigation is recommended (infectious etiology??) Routine studies for bacterial organisms, opportunistic pathogens (e.g., Pneumocystis jiroveci), and common respiratory viruses. Although the epidemiology of viral respiratory infections varies globally, relevant viruses in North America would include influenza A and B, parainfluenza 1 4, respiratory syncytial virus A and B, human metapneumovirus, adenoviruses, rhinovirus, and coronaviruses. Nosocomial infection?

27

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

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

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

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

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

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

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

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

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

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

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

Combined Pulmonary Fibrosis and Emphysema - A Case Series

Combined Pulmonary Fibrosis and Emphysema - A Case Series IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) e-issn: 2279-0853, p-issn: 2279-0861.Volume 16, Issue 1 Ver. III (January. 2017), PP 15-19 www.iosrjournals.org Combined Pulmonary Fibrosis and Emphysema

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

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

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

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

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

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

Epidemiology and classification of smoking related interstitial lung diseases

Epidemiology and classification of smoking related interstitial lung diseases Epidemiology and classification of smoking related interstitial lung diseases Šterclová M. Department of Respiratory Diseases, Thomayer Hospital, Prague, Czech Republic Supported by an IGA Grant No G 1207

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

Lung Allograft Dysfunction

Lung Allograft Dysfunction Lung Allograft Dysfunction Carlos S. Restrepo M.D. Ameya Baxi M.D. Department of Radiology University of Texas Health San Antonio Disclaimer: We do not have any conflict of interest or financial gain to

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

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

World Journal of Radiology. Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis?

World Journal of Radiology. Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis? W J R World Journal of Radiology Submit a Manuscript: http://www.wjgnet.com/esps/ Help Desk: http://www.wjgnet.com/esps/helpdesk.aspx DOI: 10.4329/wjr.v7.i9.294 World J Radiol 2015 September 28; 7(9):

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

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

Combined Unclassifiable Interstitial Pneumonia and Emphysema: A Report of Two Cases

Combined Unclassifiable Interstitial Pneumonia and Emphysema: A Report of Two Cases CASE REPORT Combined Unclassifiable Interstitial Pneumonia and Emphysema: A Report of Two Cases Nobuhiko Nagata 1, Kentaro Watanabe 2, Michihiro Yoshimi 3, Hiroshi Okabayashi 4, Katsuo Sueishi 5, Kentaro

More information

Come gestire le comorbidità e il follow-up

Come gestire le comorbidità e il follow-up Come gestire le comorbidità e il follow-up Antonella Caminati U.O. di Pneumologia e Terapia Semi Intensiva Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare Osp. San Giuseppe - MultiMedica,

More information

We are IntechOpen, the first native scientific publisher of Open Access books. International authors and editors. Our authors are among the TOP 1%

We are IntechOpen, the first native scientific publisher of Open Access books. International authors and editors. Our authors are among the TOP 1% We are IntechOpen, the first native scientific publisher of Open Access books 3,350 108,000 1.7 M Open access books available International authors and editors Downloads Our authors are among the 151 Countries

More information

HYPERSENSITIVITY PNEUMONITIS

HYPERSENSITIVITY PNEUMONITIS HYPERSENSITIVITY PNEUMONITIS A preventable fibrosis MOSAVIR ANSARIE MB., FCCP INTERSTITIAL LUNG DISEASES A heterogeneous group of non infectious, non malignant diffuse parenchymal disorders of the lower

More information

an inflammation of the bronchial tubes

an inflammation of the bronchial tubes BRONCHITIS DEFINITION Bronchitis is an inflammation of the bronchial tubes (or bronchi), which are the air passages that extend from the trachea into the small airways and alveoli. Triggers may be infectious

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

Management of Co morbidities in Idiopathic Pulmonary Fibrosis. Disclosures

Management of Co morbidities in Idiopathic Pulmonary Fibrosis. Disclosures Management of Co morbidities in Idiopathic Pulmonary Fibrosis Joyce S. Lee, MD MAS Director, Interstitial Lung Disease Clinic University of California, San Francisco Disclosures Intermune, advisory board

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

UIP OR NOT UIP PATTERN: THAT IS NOT THE ONLY QUESTION!

UIP OR NOT UIP PATTERN: THAT IS NOT THE ONLY QUESTION! UIP OR NOT UIP PATTERN: THAT IS NOT THE ONLY QUESTION! STÉPHANE JOUNEAU 11 JULY 2014 Respiratory Medicine Department, Pontchaillou Hospital, Rennes, France CASE OVERVIEW This case highlights how a usual

More information

Initial presentation of idiopathic pulmonary fibrosis as an acute exacerbation

Initial presentation of idiopathic pulmonary fibrosis as an acute exacerbation Respiratory Medicine CME (2008) 1, 43 47 respiratory MEDICINE CME CASE REPORT Initial presentation of idiopathic pulmonary fibrosis as an acute exacerbation Krishna M. Sundar a,b,, Dixie L. Harris a a

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

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

Emphysema association in a prospective series with patients suffering from Idiopathic Pulmonary Fibrosis.

Emphysema association in a prospective series with patients suffering from Idiopathic Pulmonary Fibrosis. Emphysema association in a prospective series with patients suffering from Idiopathic Pulmonary Fibrosis. David Jiménez-Restrepo, Mª Luisa Domingo Montañana, Claudia Fernandez Ruiz, Andrea Martínez Deltoro*,

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

Combined pulmonary fibrosis and emphysema; prevalence and follow up among health-care personnel

Combined pulmonary fibrosis and emphysema; prevalence and follow up among health-care personnel Combined pulmonary fibrosis and emphysema; prevalence and follow up among health-care personnel Poster No.: C-0698 Congress: ECR 2013 Type: Scientific Exhibit Authors: K. Chae, G. Jin, S. Chon, Y. Lee;

More information

ARTICLE IN PRESS. Ahuva Grubstein a, Daniele Bendayan b, Ithak Schactman c, Maya Cohen a, David Shitrit b, Mordechai R. Kramer b,

ARTICLE IN PRESS. Ahuva Grubstein a, Daniele Bendayan b, Ithak Schactman c, Maya Cohen a, David Shitrit b, Mordechai R. Kramer b, Respiratory Medicine (2005) 99, 948 954 Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the literature

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

Acute exacerbation of idiopathic pulmonary fibrosis: a proposal

Acute exacerbation of idiopathic pulmonary fibrosis: a proposal Curr Respir Care Rep (2013) 2:233 240 DOI 10.1007/s13665-013-0065-x INTERSTITIAL LUNG DISEASE (G TINO, SECTION EDITOR) Acute exacerbation of idiopathic pulmonary fibrosis: a proposal Kerri A. Johannson

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

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

The role of Pulmonary function Testing In Interstitial lung disease in infants. [ ipft in child ]

The role of Pulmonary function Testing In Interstitial lung disease in infants. [ ipft in child ] The role of Pulmonary function Testing In Interstitial lung disease in infants [ ipft in child ] Introduction Managing infants with diffuse lung disease (DLD) suspected to have interstitial lung disease

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

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

Chapter. Diffusion capacity and BMPR2 mutations in pulmonary arterial hypertension

Chapter. Diffusion capacity and BMPR2 mutations in pulmonary arterial hypertension Chapter 7 Diffusion capacity and BMPR2 mutations in pulmonary arterial hypertension P. Trip B. Girerd H.J. Bogaard F.S. de Man A. Boonstra G. Garcia M. Humbert D. Montani A. Vonk Noordegraaf Eur Respir

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

SCLERODERMA LUNG DISEASE: WHAT THE PATIENT SHOULD KNOW

SCLERODERMA LUNG DISEASE: WHAT THE PATIENT SHOULD KNOW SCLERODERMA LUNG DISEASE: WHAT THE PATIENT SHOULD KNOW Lung disease can be a serious complication of scleroderma. The two most common types of lung disease in patients with scleroderma are interstitial

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

The Egyptian Journal of Hospital Medicine (July 2017) Vol.68 (2), Page

The Egyptian Journal of Hospital Medicine (July 2017) Vol.68 (2), Page The Egyptian Journal of Hospital Medicine (July 2017) Vol.68 (2), Page 1135-1140 Role of High Resolution Computed Tomography in Diagnosis of Interstitial Lung Diseases in Patients with Collagen Diseases

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

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

Acute and Chronic Lung Disease

Acute and Chronic Lung Disease KATHOLIEKE UNIVERSITEIT LEUVEN Faculty of Medicine Acute and Chronic Lung Disease W De Wever, JA Verschakelen Department of Radiology, University Hospitals Leuven, Belgium Clinical utility of HRCT To detect

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

Patient with IPF and no honeycombing on HRCT. Case 1 Demosthenes Bouros, Vasilios Tzilas University of Athens

Patient with IPF and no honeycombing on HRCT. Case 1 Demosthenes Bouros, Vasilios Tzilas University of Athens Patient with IPF and no honeycombing on HRCT Case 1 Demosthenes Bouros, Vasilios Tzilas University of Athens CASE OVERVIEW A 76-year-old male patient presented with progressive exertional dyspnoea refractory

More information

11/19/2012. The spectrum of pulmonary diseases in HIV-infected persons is broad.

11/19/2012. The spectrum of pulmonary diseases in HIV-infected persons is broad. The spectrum of pulmonary diseases in HIV-infected persons is broad. HIV-associated Opportunistic infections Neoplasms Miscellaneous conditions Non HIV-associated Antiretroviral therapy (ART)-associated

More information

Role of Pirfenidone in Idiopathic Pulmonary Fibrosis - A Longitudinal Cohort Study

Role of Pirfenidone in Idiopathic Pulmonary Fibrosis - A Longitudinal Cohort Study 36 Journal of The Association of Physicians of India Vol. 64 May 2016 Original Article Role of Pirfenidone in Idiopathic Pulmonary Fibrosis - A Longitudinal Cohort Study KP Suraj 1, Neethu K Kumar 2, E

More information

Shigeki Chiba, 1 Hiromitsu Ohta, 1 Kyoko Abe, 1 Shu Hisata, 1 Shinya Ohkouchi, 1 Yasushi Hoshikawa, 2 Takashi Kondo, 2 and Masahito Ebina 1

Shigeki Chiba, 1 Hiromitsu Ohta, 1 Kyoko Abe, 1 Shu Hisata, 1 Shinya Ohkouchi, 1 Yasushi Hoshikawa, 2 Takashi Kondo, 2 and Masahito Ebina 1 Pulmonary Medicine Volume 2012, Article ID 492960, 6 pages doi:10.1155/2012/492960 Research Article The Diagnostic Value of the Interstitial Biomarkers KL-6 and SP-D for the Degree of Fibrosis in Combined

More information

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease Disclosure Objectives Pulmonary veno-occlusive disease Tilman Humpl The Hospital for Sick Children University of Toronto, Canada Advisor/Research Grants Actelion Pfizer Historical aspects Epidemiology/Genetics

More information

Idiopathic pulmonary fibrosis (IPF) is a

Idiopathic pulmonary fibrosis (IPF) is a Eur Respir J 2011; 38: 176 183 DOI: 10.1183/09031936.00114010 CopyrightßERS 2011 Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema S.L. Schmidt*,

More information

Asymmetry in acute exacerbation of idiopathic pulmonary fibrosis

Asymmetry in acute exacerbation of idiopathic pulmonary fibrosis ORIGINAL ARTICLE IDIOPATHIC PULMONARY FIBROSIS Asymmetry in acute exacerbation of idiopathic pulmonary fibrosis Akihiko Sokai 1, Kiminobu Tanizawa 2, Tomohiro Handa 1, Takeshi Kubo 3, Seishu Hashimoto

More information

OFEV MEDIA BACKGROUNDER

OFEV MEDIA BACKGROUNDER OFEV MEDIA BACKGROUNDER 1 What is OFEV (nintedanib*)? 2 How does OFEV (nintedanib*) work? 3 Data overview 4 OFEV (nintedanib*) approval status 1 What is OFEV (nintedanib*)? OFEV (nintedanib*) is a small

More information

Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone

Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone Review Article Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone Huijin Lin 1, Shanping Jiang 1,2 1 Department of Respiratory Medicine, Sun

More information

Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis

Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis Respiratory Medicine (2009) 103, 1209e1215 available at www.sciencedirect.com journal homepage: www.elsevier.com/locate/rmed Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary

More information

Chapter 10 Respiratory System J00-J99. Presented by: Jesicca Andrews

Chapter 10 Respiratory System J00-J99. Presented by: Jesicca Andrews Chapter 10 Respiratory System J00-J99 Presented by: Jesicca Andrews 1 Respiratory System 2 Respiratory Infections A respiratory infection cannot be assumed from a laboratory report alone; physician concurrence

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

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

Monday 10 September Interstitial lung disease 15:10 15:35. The uncommon interstitial lung diseases (ILD)

Monday 10 September Interstitial lung disease 15:10 15:35. The uncommon interstitial lung diseases (ILD) Interstitial lung disease 15:10 15:35 The uncommon interstitial lung diseases (ILD) Dr Grant Griffiths, Cwm Taf University Health Board, Cardiff Be familiar with the Diagnostic criteria for idiopathic

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

Smoking-related Interstitial Lung Diseases: High-Resolution CT Findings

Smoking-related Interstitial Lung Diseases: High-Resolution CT Findings Smoking-related Interstitial Lung Diseases: High-Resolution CT Findings Poster No.: C-2358 Congress: ECR 2013 Type: Educational Exhibit Authors: V. Cuartero Revilla, M. Nogueras Carrasco, P. Olmedilla

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

Imaging Small Airways Diseases: Not Just Air trapping. Eric J. Stern MD University of Washington

Imaging Small Airways Diseases: Not Just Air trapping. Eric J. Stern MD University of Washington Imaging Small Airways Diseases: Not Just Air trapping Eric J. Stern MD University of Washington What we are discussing SAD classification SAD imaging with MDCT emphasis What is a small airway? Airway with

More information

Smoking-related interstitial lung disease

Smoking-related interstitial lung disease Smoking-related interstitial lung disease Sergio Harari U.O. di Pneumologia UTIR Servizio di Fisiopatologia Respiratoria e Emodinamica Polmonare Ospedale S. Giuseppe MultiMedica Milano Milano, 7 Ottobre

More information

Case 1: Question. 1.1 What is the main pattern of this HRCT? 1. Intralobular line 2. Groundglass opacity 3. Perilymphatic nodule

Case 1: Question. 1.1 What is the main pattern of this HRCT? 1. Intralobular line 2. Groundglass opacity 3. Perilymphatic nodule HRCT WORK SHOP Case 1 Case 1: Question 1.1 What is the main pattern of this HRCT? 1. Intralobular line 2. Groundglass opacity 3. Perilymphatic nodule Case 1: Question 1.2 What is the diagnosis? 1. Hypersensitivity

More information

Key words: CT scanners; interstitial lung diseases; polymyositis-dermatomyositis; x-ray

Key words: CT scanners; interstitial lung diseases; polymyositis-dermatomyositis; x-ray Nonspecific Interstitial Pneumonia Associated With Polymyositis and Dermatomyositis* Serial High-Resolution CT Findings and Functional Correlation Hiroaki Arakawa, MD; Hidehiro Yamada, MD; Yasuyuki Kurihara,

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

Tracy Ward Highly Specialist Respiratory Nurse Rotherham NHS Foundation Trust

Tracy Ward Highly Specialist Respiratory Nurse Rotherham NHS Foundation Trust Interstitial Lung Disease (ILD) Tracy Ward Highly Specialist Respiratory Nurse Rotherham NHS Foundation Trust The views expressed in this presentation are those of the speaker and are not necessarily those

More information

Management of idiopathic pulmonary fibrosis: selected case reports

Management of idiopathic pulmonary fibrosis: selected case reports CASE-BASED REVIEW IDIOPATHIC PULMONARY FIBROSIS Management of idiopathic pulmonary fibrosis: selected case reports Michael Kreuter 1, Peter Kardos 2 and Victor Hoffstein 3 Affiliations: 1 Dept of Pneumology

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

How To Assess Severity and Prognosis

How To Assess Severity and Prognosis How To Assess Severity and Prognosis Gregory Tino, M.D. Chief, Department of Medicine Penn Presbyterian Medical Center Associate Professor of Medicine Perelman School of Medicine at the University of Pennsylvania

More information

Acute Exacerbation of Usual Interstitial Pneumonia After Resection of Lung Cancer

Acute Exacerbation of Usual Interstitial Pneumonia After Resection of Lung Cancer Acute Exacerbation of Usual Interstitial Pneumonia After Resection of Lung Cancer Hiroaki Sugiura, MD, Atsuya Takeda, MD, PhD, Toshiko Hoshi, MD, PhD, Yoshinori Kawabata, MD, Koichi Sayama, MD, PhD, Masahiro

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

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

A CASE OF UNFORTUNATE

A CASE OF UNFORTUNATE A CASE OF UNFORTUNATE TAPER Hélène Schoemans, MD University Hospitals Leuven, Belgium EBMT Complications and Quality of Life Working Party Educational Course Warsaw, October 24 th 2014 Company name No

More information

Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia

Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia Original article: Clinical research SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES 2013; 30; 103-112 Mattioli 1885 Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia

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

How to identify interstitial pneumonias.

How to identify interstitial pneumonias. How to identify interstitial pneumonias. Poster No.: C-0804 Congress: ECR 2014 Type: Educational Exhibit Authors: S. claret loaiza, M. C. Cañete Moslero, R. Carreño Gonzalez, C. de la Torre; Malaga/ES

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

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

Experience with the Compassionate Use Program of nintedanib for the treatment of Idiopathic Pulmonary Fibrosis in Argentina

Experience with the Compassionate Use Program of nintedanib for the treatment of Idiopathic Pulmonary Fibrosis in Argentina ORIGINAL 131 RAMR 2017;2:131-135 ISSN 1852-236X Correspondence Gabriela Tabaj gabrielatabaj@gmail.com Received: 11.15.2016 Accepted: 02.03.2017 Experience with the Compassionate Use Program of nintedanib

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

Respiratory Pathology. Kristine Krafts, M.D.

Respiratory Pathology. Kristine Krafts, M.D. Respiratory Pathology Kristine Krafts, M.D. Normal lung: alveolar spaces Respiratory Pathology Outline Acute respiratory distress syndrome Obstructive lung diseases Restrictive lung diseases Vascular

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

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

ACUTE RESPIRATORY DISTRESS SYNDROME

ACUTE RESPIRATORY DISTRESS SYNDROME ACUTE RESPIRATORY DISTRESS SYNDROME Angel Coz MD, FCCP, DCE Assistant Professor of Medicine UCSF Fresno November 4, 2017 No disclosures OBJECTIVES Identify current trends and risk factors of ARDS Describe

More information