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
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Dedicated to the late Drs. Charles Carrington and William Thurlbeck Andrew Churg Dedicated to my family Nestor L Müller
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
Contents Preface iv chapter 1 General Approach to Interstitial Lung Disease: Clinical and Pathologic Considerations........ 1 chapter 2 Imaging in Interstitial Lung Disease...4 chapter 3 Biopsy Choices and Handling in Interstitial Lung Disease.............................. 9 chapter 4 Acute Interstitial Pneumonia.................................................. 15 chapter 5 Bronchiolitis Obliterans Organizing Pneumonia...25 chapter 6 Usual Interstitial Pneumonia.................................................. 38 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.................................................. 95 chapter 13 Sarcoid................................................................. 111 chapter 14 Granulomatous Interstitial Lung Disease in Common Variable Immunodeficiency.......... 125 chapter 15 Eosinophilic Pneumonias...127 v
vi contents chapter 16 Pulmonary Alveolar Proteinosis...138 chapter 17 Lymphangioleiomyomatosis...145 chapter 18 Drug Reactions Producing Interstitial Lung Disease................................ 154 chapter 19 Lymphoid and Hematopoietic Processes Producing a Pattern of Interstitial Lung Disease...168 chapter 20 Bronchiolitis............................................................. 181 chapter 21 Interstitial Lung Disease in Patients with Collagen Vascular Diseases...195 chapter 22 Pneumoconioses Producing a Pattern of Interstitial Lung Disease...200 chapter 23 Miscellaneous Forms of Interstitial Lung Disease.................................. 213 chapter 24 Mimics of Interstitial Lung Disease...228 Index 240