Pulmonary Arterial Hypertension and Interstitial Lung Diseases: a Clinical Guide PDF
Preview Pulmonary Arterial Hypertension and Interstitial Lung Diseases: a Clinical Guide
Pulmonary Arterial Hypertension and Interstitial Lung Diseases Robert P. Baughman (cid:127) Roberto G. Carbone Giovanni Bottino Editors Pulmonary Arterial Hypertension and Interstitial Lung Diseases A Clinical Guide Editors Robert P. Baughman Roberto G. Carbone University of Cincinnati, Regional Hospital Department of Internal Medicine Department of Internal Medicine Cincinnati, OH Aosta, Italy Giovanni Bottino Regional Hospital Department of Internal Medicine Aosta, Italy ISBN: 978-1-58829-695-5 e-ISBN: 978-1-60327-074-8 DOI: 10.1007/978-1-60327-074-8 Library of Congress Control Number: 2008937495 © Humana Press, a part of Springer Science+Business Media, LLC 2009 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Humana Press, c/o Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013 USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper springer.com wife Elise Lower, in Memory of mother Attilia Innesti Carbone wife Elena Bottino Preface Interstitial lung disease (ILD) is a broad category of lung diseases that includes more than 150 disorders characterized by scarring or fibrosis of the lungs. Even among the many types of the disease, ILD’s progression can vary from person to person, and people respond differently to therapy. In the past, emphasis in treating ILDs has focused on the effect on gas exchange and loss of lung volume. This is a direct effect of the damage to the interstitium. However, an important indirect effect is on the pulmonary vasculature with resulting pulmonary hypertension. The asso- ciation between interstitial lung disease and pulmonary hypertension has long been recognized, it was often associated with hypoxia and fibrosis alone. Recent studies that demonstrate response to pulmonary vasodilators stresses the vascular compo- nent of this process. In this book, we examine the various interstitial lung diseases. We also examine the incidence and outcome of pulmonary hypertension in the vari- ous interstitial diseases. The book is divided into two main sections. The first discusses general issues. Drs. Carbone and Bottino introduce both ILD and associated pulmonary hyperten- sion in the first two chapters of the book. The next chapter is by Drs. Meyer and Raghu, who discuss the evaluation of idiopathic interstitial lung diseases. They point out that this includes not only idiopathic pulmonary fibrosis, but other condi- tions such as nonspecific interstitial pneumonitis and cryptogenic organizing pneu- monia. Drs. Moreira and Travis provide a detailed analysis of the pathology of the various ILDs. The pathologist often has the final say about what disease, although a comprehensive approach the clinician, radiologist, and pathologist gives a better definition of many cases. Finally, Drs. Carbone and Bottino summarize the evalua- tion of pulmonary hypertension. Although most of the information available is from patients with primary pulmonary hypertension, the observations can often be extended to patients with ILD. The other section of the book deals with specific categories of disease. Dr. Lynch and colleagues discuss bronchiolitis, an increasingly recognized problem leading to airway obstruction and restriction. The use of inspiratory and expiratory high-resolu- tion computed tomography scan has markedly enhanced the recognition of this proc- ess. Dr. Selman and his group then discuss hypersensitivity pneumonitis, a diffuse group of diseases bound together by common clinical and pathological features. vii viii Preface Drs. Brown and Strange discuss the collagen vascular diseases. Scleroderma has been one of the most widely studied lung diseases that can cause both interstitial lung process as well as pulmonary hypertension. In the past few years, large clinical trials have been published showing the benefits of some forms of therapy in these diseases. Dr. Martinez discusses the specific problem of pulmonary hypertension with idiopathic pulmonary fibrosis. Because idiopathic pulmonary fibrosis is asso- ciated with a high mortality, treatment for this complication may have major impact on the disease. Dr. Lee Newman examines the interstitial lung diseases associated with various occupational exposures. This divergent group can have a quite variable outcome. However, as a group it represents a major part of the differential diagnosis of all patients with interstitial lung diseases. Dr. Baughman and colleagues discuss sarcoidosis. This multi organ disease affects the lungs in more than 90% of cases. Although most patients do well, there is a group with persistent pulmonary disease. Up to half of these patients will have pulmonary hypertension. Drs. Baughman, Lower, and Engel provide an evaluation for the disease and treatment strategies for the disease and associated pulmonary hypertension. Finally, the editors would like to again to thank all the authors for their efforts in preparing this book. We would also like to thank Richard Lansing of Humana Press for his support. Contents Part I: General Principles 1 Interstitial Lung Disease: Introduction.................................................. 3 Roberto G. Carbone, Fabio Montanaro, and Giovanni Bottino 2 Pulmonary Hypertension in Interstitial Lung Disease ......................... 13 Roberto G. Carbone, Assaf Monselise, and Giovanni Bottino 3 Idiopathic Pulmonary Fibrosis and Associated Pulmonary Hypertension: Genetics, Pathobiology, Diagnosis, and Management ................................................................... 51 Keith C. Meyer and Ganesh Raghu 4 Lung Pathology ........................................................................................ 71 Andre L. Moreira and William D. Travis 5 Primary Pulmonary Hypertension ......................................................... 89 Roberto G. Carbone and Giovanni Bottino Part II: Specific Lung Disorders 6 Bronchiolar Disorders ............................................................................. 111 Joseph P. Lynch, III, Rajeev Saggar, Robert D. Suh, and Michael C. Fishbein 7 Hypersensitivity Pneumonitis ................................................................. 139 Moisés Selman, Guillermo Carrillo, Carmen Navarro, and Miguel Gaxiola 8 Connective Tissue Disease and Vasculitis-Associated Interstitial Lung Disease.......................................................................... 159 Alan N. Brown and Charlie Strange ix x Contents 9 Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis ................................................................................ 177 Fernando J. Martinez 10 Occupational Interstitial Lung Disease Update .................................. 195 Lee S. Newman 11 Sarcoidosis .............................................................................................. 213 Robert P. Baughman, Elyse E. Lower, and Peter Engel Index ................................................................................................................ 243 Contributors Robert P. Baughman, MD Professor of Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati Medical Center, Department of Medicine, Cincinnati, OH Giovanni Bottino, MD Professor of Medicine, Dept. of Internal Medicine, Docent of Respiratory Diseases, DIMI-University of Genoa, Genoa, Italy Alan N. Brown, MD Associate Professor of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC Roberto G. Carbone, MD, FCCP Department of Internal Medicine, Respiratory Unit, Regional Hospital, Aosta, Italy; Consultant Physician of DIMI University of Genoa, Genoa, Italy, for Interstitial Lung Disease, Consultant Physician of University of Turin, Turin, Italy, for Asbestosis correlated with Mesothelioma, Aosta, Italy Guillermo Carrillo, MD Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México Peter J. Engel, MD Ohio Heart and Vascular Center, Cincinnati, OH Michael C. Fishbein, MD Department of Pathology and Laboratory Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, CA Miguel Gaxiola, MD Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México Elyse E. Lower, MD Professor of Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati Medical Center, Department of Medicine, Cincinnati, OH xi
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