Nestor L. Müller, MD, PhD Professor Emeritus of Radiology University of British Columbia Vancouver, BC, Canada

Transcription

1 Atlas of Interstitial Lung Disease Pathology Pathology with High Resolution CT Correlations First Edition Andrew Churg, MD Professor of Pathology University of British Columbia Pathologist, Vancouver General Hospital Vancouver, BC, Canada Nestor L. Müller, MD, PhD Professor Emeritus of Radiology University of British Columbia Vancouver, BC, Canada

2 Acquisitions Editor: Ryan Shaw Product Manager: Kate Marshall Vendor Manager: Senior Manufacturing Coordinator: Beth Welsh Marketing Manager: Alexander Burns Designer: Teresa Mallon Production Service: 2014 by LIPPINCOTT WILLIAMS & WILKINS, a WOLTERS KLUWER business Two Commerce Square 2001 Market Street Philadelphia, PA USA LWW.com All rights reserved. This book is protected by copyright. No part of this book may be reproduced in any form by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. Printed in China Library of Congress Cataloging-in-Publication Data Churg, Andrew, author. Atlas of interstitial lung disease pathology : pathology with high resolution CT correlations / authored by Andrew Churg, Nestor L. Müller. p. ; cm. Includes bibliographical references and index. ISBN (alk. paper) I. Müller, Nestor Luiz, author. II. Title. [DNLM: 1. Lung Diseases, Interstitial pathology Atlases. 2. Tomography, X-Ray Computed Atlases. WF 17] RC734.T dc Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of the information in a particular situation remains the professional responsibility of the practitioner. The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new or infrequently employed drug. Some drugs and medical devices presented in the publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice. To purchase additional copies of this book, call our customer service department at (800) or fax orders to (301) International customers should call (301) Visit Lippincott Williams & Wilkins on the Internet: at LWW.com. Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to 6 pm, EST

3 Dedicated to the late Drs. Charles Carrington and William Thurlbeck Andrew Churg Dedicated to my family Nestor L Müller

4 Preface Interstitial lung disease (ILD) is an extremely confusing topic, and this problem extends from clinicians to radiologists to pathologists. Confusion arises in part because of the sheer number of ILDs; clinicians can name more than 150 separate entities. From the point of view of the pathologist there are many fewer diagnosable patterns, but this phenomenon immediately raises the question of how to make those patterns correspond to clinically defined diseases, particularly so because, at first glance, there appears to be considerable morphologic overlap among these various conditions. A further source of confusion is that much of what is called interstitial lung disease is really characterized by processes that take place largely in the airspaces bronchiolitis obliterans organizing pneumonia (BOOP) is a good example or those that affect primarily small airways; for example, constrictive bronchiolitis. However, we believe that the biggest problem for pathologists trying to deal with ILD is that the non-lung specialist will see relatively few such cases in a year, and turning to standard textbooks provides only limited help because textbooks by their very nature can supply only a few illustrations of any particular condition. This Atlas is intended to address this problem by providing a large number of illustrations to give the practicing pathologist a feel for the morphologic spectrum of any given ILD and also to illustrate the various differential diagnoses of any particular condition, something that textbooks often do not provide. For this reason we have included some uncommon variants of relatively common ILD; for example, fibrosis in chronic eosinophilic pneumonia (CEP) and in BOOP, interstitial spread of Langerhans cell histiocytosis (LCH), and progression of desquamative interstitial pneumonia (DIP) to a picture of fibrotic nonspecific interstitial pneumonia (NSIP). We have also included some material on imaging in every chapter, because non-neoplastic lung disease in general and ILD in particular is very difficult to diagnose without clinical and especially radiologic information. Conversely, we hope that radiologists will find this volume to be helpful in understanding the pathologic changes behind the radiologic appearances. But this book is not intended as a general detailed text on clinical features, imaging, pathogenesis, treatment, and so on of ILD, and we have also purposely kept references to an absolute minimum. Rather the book is meant as a quick reference whereby one can look at a set of pictures and get a reasonable idea of whether and how well a particular case shows the diagnostic features of a particular disease. Andrew Churg Nestor L Müller iv

5 Contents Preface iv chapter 1 General Approach to Interstitial Lung Disease: Clinical and Pathologic Considerations chapter 2 Imaging in Interstitial Lung Disease...4 chapter 3 Biopsy Choices and Handling in Interstitial Lung Disease chapter 4 Acute Interstitial Pneumonia chapter 5 Bronchiolitis Obliterans Organizing Pneumonia...25 chapter 6 Usual Interstitial Pneumonia chapter 7 Nonspecific Interstitial Pneumonia...55 chapter 8 respiratory Bronchiolitis with Interstitial Lung Disease and Desquamative Interstitial Pneumonia...66 chapter 9 Combined Fibrosis with Emphysema...76 chapter 10 Langerhans Cell Histiocytosis (Eosinophilic Granuloma of Lung)...82 chapter 11 Introduction to Granulomatous Forms of Interstitial Lung Disease...94 chapter 12 Hypersensitivity Pneumonitis chapter 13 Sarcoid chapter 14 Granulomatous Interstitial Lung Disease in Common Variable Immunodeficiency chapter 15 Eosinophilic Pneumonias v

6 vi contents chapter 16 Pulmonary Alveolar Proteinosis chapter 17 Lymphangioleiomyomatosis chapter 18 Drug Reactions Producing Interstitial Lung Disease chapter 19 Lymphoid and Hematopoietic Processes Producing a Pattern of Interstitial Lung Disease chapter 20 Bronchiolitis chapter 21 Interstitial Lung Disease in Patients with Collagen Vascular Diseases chapter 22 Pneumoconioses Producing a Pattern of Interstitial Lung Disease chapter 23 Miscellaneous Forms of Interstitial Lung Disease chapter 24 Mimics of Interstitial Lung Disease Index 240