Pulmonary Hypertension and Interstitial Lung Disease

Pulmonary Hypertension and Interstitial Lung Disease

Robert P. Baughman Roberto G. Carbone Steven D. Nathan Editors Pulmonary Hypertension and Interstitial Lung Disease Second Edition

Editors Robert P. Baughman Department of Medicine University of Cincinnati Medical Center Cincinnati, OH, USA Roberto G. Carbone Regional Hospital Aosta and University of Genoa Genoa, Italy Steven D. Nathan Inova Fairfax Advanced Lung Disease and Transplant Program Inova Fairfax Hospital Falls Church, VA, USA ISBN 978-3-319-49916-1 DOI 10.1007/978-3-319-49918-5 ISBN 978-3-319-49918-5 (ebook) Library of Congress Control Number: 2017942775 Springer International Publishing AG 2009, 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Dr. Roberto Carbone wishes to dedicate this book to parents Attilia and Giuseppe Carbone.

Preface Several years ago, Roberto Carbone began working on a book which would deal exclusively regarding pulmonary hypertension in interstitial lung disease. He asked Drs. Baughman and Bottino to join in this effort as editors and we solicited chapters from experts around the world on this subject. This led to the first edition of Pulmonary Arterial Hypertension in Interstitial Lung Disease which was published in 2010. In this edition, Dr. Baughman and Carbone are joined by Dr. Steven Nathan as the third co-editor. Since that time, several studies have been published regarding the diagnosis and management of pulmonary hypertension in interstitial lung disease. The terminology has changed and pulmonary arterial hypertension is reserved for WHO group 1 patients. Pulmonary hypertension in interstitial lung disease is categorized as either group 3 (e.g. idiopathic pulmonary fibrosis) or group 5 (e.g. sarcoidosis). Thus, we have changed the title of our second edition to Pulmonary Hypertension in Interstitial Lung Disease. In addition to a new title and new co-editor, we have asked our contributors to provide a new version of their respective chapters, not just an update of the prior chapters. For many of the chapters, we sought new authors who have contributed to these respective areas. The book is divided into two main sections. The first section deals with general principles of diagnosis and management of pulmonary hypertension in interstitial lung disease (PH ILD). Dr. Carbone and colleagues start with a chapter detailing the radiographic imaging seen in various interstitial lung diseases, with insights into how the pattern on high resolution computer tomography (HRCT) scan may help determine the underlying interstitial lung disease. Since patients with significant pulmonary hypertension are poor candidates for surgical biopsy or even bronchoscopic biopsy, many of the PH ILD patients are diagnosed based on HRCT. Dr. Engel describes the findings on heart catheterization which characterize pulmonary hypertension. This includes separating precapillary pulmonary hypertension from pulmonary hypertension due to left ventricular dysfunction. Although pulmonary pathology may not be available as part of the initial assessment of PH ILD, studies of pathology from biopsies and explanted lungs have provided insights into the vii

viii Preface cause(s) of pulmonary hypertension associated with specific interstitial lung diseases. This is detailed in the chapter by Dr. Nunes and colleagues. The last two chapters of the general section focus on therapy. Dr. Nathan s group provide a review of the evidence supporting various medical managements for treating pulmonary hypertension in different interstitial lung diseases. In the past few years, some drugs have been shown not only to be ineffective for pulmonary hypertension associated with idiopathic pulmonary fibrosis but also to be potentially harmful. This is not a universal finding, since therapy for pulmonary hypertension with interstitial lung disease associated with scleroderma and sarcoidosis has had positive results. One potential treatment for PH ILD is transplant. Dr. Cordova and colleagues have reviewed this option in the last chapter in the general considerations section. The second half of the book deals with specific interstitial lung diseases. Dr. Wells group provide details regarding managing pulmonary hypertension in patients with idiopathic pulmonary fibrosis and other idiopathic interstitial lung diseases. Dr. Baughman and colleagues discuss the evaluation and treatment of sarcoidosis- associated pulmonary hypertension. Dr. Selman s group discuss pulmonary hypertension in patients with hypersensitivity pneumonitis. Dr. Highland and colleagues discuss pulmonary hypertension in interstitial lung disease associated with connective tissue disorders. Dr. Shlobin s group then address several other interstitial lung diseases associated with pulmonary hypertension. We believe this book will provide a framework for future studies in what is an important aspect of managing both interstitial lung disease and pulmonary hypertension. The editors wish to thank Stephanie Westendorf for all her help and support for this project. Cincinnati, OH, USA Genoa, Italy Falls Church, VA, USA Robert P. Baughman Roberto G. Carbone Steven D. Nathan

Acknowledgements Robert Baughman would like to dedicate this book to Elyse Lower, his spouse and most supportive collaborator. Steve Nathan would like to dedicate this book to his wife Romy and their two sons Jack and Max. ix

Contents 1 Radiographic Imaging in Interstitial Lung Disease and Pulmonary Hypertension... 1 Roberto G. Carbone, Assaf Monselise, and Giovanni Bottino 2 Invasive Techniques for Diagnosis of PH... 29 Peter J. Engel 3 Pathology of Vascular Changes in Interstitial Lung Diseases... 45 Hilario Nunes, Peter Dorfmüller, Yurdagul Uzunhan, Dominique Valeyre, Jean-François Bernaudin, and Marianne Kambouchner 4 Treatment of Pulmonary Hypertension in Interstitial Lung Disease... 67 Christopher S. King and Steven D. Nathan 5 Lung Transplantation in Interstitial Lung Disease... 85 Cynthia Kim, Francis Cordova, and Yoshiya Toyoda 6 Pulmonary Hypertension in Idiopathic Interstitial Pneumonias... 103 Simon Bax, Athol Wells, Laura Price, and John Wort 7 Sarcoidosis-Associated Pulmonary Hypertension: Diagnosis and Treatment... 129 Robert P. Baughman and Elyse E. Lower 8 Hypersensitivity Pneumonitis... 145 Moisés Selman, Ivette Buendía-Roldán, Carmen Navarro, and Miguel Gaxiola xi

xii Contents 9 Interstitial Lung Disease-Associated Pulmonary Hypertension in the Connective Tissue Disorders... 165 Debabrata Bandyopadhyay, Tanmay S. Panchabhai, and Kristin B. Highland 10 Pulmonary Hypertension in Rare Parenchymal Lung Diseases... 181 Oksana A. Shlobin and Steven D. Nathan Index... 201

Contributors Debabrata Bandyopadhyay, MD, MRCP Department of Thoracic Medicine, Geisinger Medical Center, Danville, PA, USA Robert P. Baughman, MD Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA Simon Bax, BSc, MBBS, MRCP Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK Jean-François Bernaudin Université Paris 13, Sorbonne Paris Cité, Bobigny, France Histologie et Cytologie, Université Pierre et Marie Curie, Sorbonne Universités Paris, Paris, France Giovanni Bottino, MD Department of Respiratory Medicine, University of Genoa, Genoa, Italy Ivette Buendía-Roldán, MSc Instituto Nacional de Enfermedades Respiratorias, Dr. Ismael Cosío Villegas, Tialpan, México DF, Mexico Roberto G. Carbone, MD, FCCP Regional Hospital Aosta and University of Genoa, Genoa, Italy Francis Cordova, MD Department of Thoracic Medicine and Surgery, Temple University School of Medicine, Philadelphia, PA, USA Peter Dorfmüller Service d Anatomie pathologique et INSERM UMRS 999, LabEx LERMIT, Hôpital Marie Lannelongue, Le Plessis-Robinson, France Peter J. Engel, MD Ohio Heart and Vascular Center, The Christ Hospital, Cincinnati, OH, USA Miguel Gaxiola, MD Instituto Nacional de Enfermedades Respiratorias, Dr. Ismael Cosío Villegas, Tialpan, México DF, Mexico xiii

xiv Contributors Kristin B. Highland, MD, MSCR Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH, USA Cynthia Kim, MD Department of Medicine, Division of Pulmonary and Critical Care, David Geffen School of Medicine at University of California, Los Angeles, CA, USA Marianne Kambouchner Assistance Publique Hôpitaux de Paris, Service d Anatomie et Cytologie pathologiques, Hôpital Avicenne, Bobigny, France Christopher S. King, MD Inova Fairfax Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA Elyse E. Lower, MD Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA Assaf Monselise, MD Department of Internal Medicine, University of Tel Aviv, Tel Aviv, Israel Steven D. Nathan, MD Inova Fairfax Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA Carmen Navarro, MD Instituto Nacional de Enfermedades Respiratorias, Dr. Ismael Cosío Villegas, Tlalpan, México DF, Mexico Hilario Nunes, MD, PhD Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, France Université Paris 13, Sorbonne Paris Cité, Bobigny, France Tanmay S. Panchabhai, MD Norton Thoracic Institute, St Joseph s Medical Center, Phoenix, AZ, USA Laura Price, BSc, MBChB, MRCP, PhD Pulmonary Hypertension Service, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK Moises Selman, MD Instituto Nacional de Enfermedades Respiratorias, Dr. Ismael Cosío Villegas, Tlalpan, México DF, Mexico Oksana A. Shlobin, MD Inova Fairfax Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA Yoshiya Toyoda, MD, PhD Department of Thoracic Medicine and Surgery, Temple University School of Medicine, Philadelphia, PA, USA Yurdagul Uzunhan Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, France Université Paris 13, Sorbonne Paris Cité, Bobigny, France

Contributors xv Dominique Valeyre Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, France Université Paris 13, Sorbonne Paris Cité, Bobigny, France Athol Wells, MBChB, MD, FRACP, FRCP, FRCR Interstitial Lung Disease Unit, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK John Wort, MA, MBBS, PhD, FRCP, FFICM Pulmonary Hypertension Service, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK