Atlas of Diffuse Lung Diseases

A Multidisciplinary Approach

Editors Giorgia Dalpiaz & Alessandra Cancellieri

GIORGIA DALPIAZ Department of Radiology , Bellaria Hospital , Bologna , Italy

ALESSANDRA CANCELLIERI Department of Pathology , Maggiore Hospital , Bologna , Italy

ISBN 978-3-319-42750-8 ISBN 978-3-319-42752-2 (eBook) DOI 10.1007/978-3-319-42752-2

Library of Congress Control Number: 2016959558

© Springer International Publishing Switzerland 2017

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iii

Foreword For many clinicians and radiologists, particularly those with limited experience, the recognition and characterisation of diffuse

lung disease can be an uncomfortable challenge. Any aid that increases the confi dence and accuracy of HRCT interpretation is

thus very welcome. This handsomely produced guide to understanding diffuse interstitial lung disease and multidisciplinary

diagnosis, using practical divisions based on broad patterns, is an excellent addition to the literature. The underpinning of CT

interpretation by superb pathology illustrations is a real strength, and will remind readers that extracting the most out of HRCT

images requires a good grasp of the pathology of diffuse lung diseases.

Many radiologists have mental “thumbnail sketches” of diffuse lung diseases, that is to say they can compress what may be

a complex diffuse lung disease, with potentially variable appearances, into surprisingly succinct descriptions. For example, the

HRCT fi ndings in pulmonary veno-occlusive disease can be summarised as: “widespread ground glass, septal thickening, big

pulmonary artery and small effusions”. This approach allows a large bank of HRCT diagnoses to be stored in the otherwise

crowded hippocampus of a radiologist. The beauty of this book is the way it echoes this method which means that the thumbnail

sketches of common and rare diffuse lung diseases can be readily assimilated.

The authors are drawn from the foremost practitioners of multidisciplinary diagnosis of diffuse lung disease, and there is no

doubt that the enthusiastic and critical approach to HRCT by expert Italian clinicians, radiologists and pathologists over many

years has helped to establish the crucial imaging technique of HRCT in the modern practice of respiratory medicine.

David M. Hansell Professor of Thoracic Imaging

Royal Brompton Hospital

London , UK

Foreword

v

Foreword Since the fi rst evidences published in literature in the early 1980s demonstrating the usefulness of HRCT scans in evaluating lung

interstitium and the related lung diseases with excellent anatomical details, a tremendous progress has been made in the

knowledge of diffuse lung diseases (DLDs).

This remarkable book is structured as an atlas, but it is much more than that. It explores the DLDs through a reading key

centered on the correlation between radiological fi ndings and pathological appearance, without overlooking the most relevant

clinical aspects of the diseases and the updatings from literature.

The easiest approach to the complex topic of DLDs is to understand the reasons why a disease manifests certain characteristics

on HRCT scans and this work represents an excellent example of it. The authors brilliantly describe patterns and diseases in a

creative and original manner which facilitates the learning process by taking pictures not only from HRTC scans and pathological

specimens, but also from nature, art, and real life. Furthermore, differential diagnosis is complete, concise, and effectively

discussed in all the sections.

I would like to congratulate the authors on this outstanding volume which attests their great and continuous collaboration,

born in the daily practice. In an age in which the multidisciplinary approach to DLDs is becoming essential to an accurate

diagnosis, this atlas is timely perfect.

I highly recommend it to all the physicians who are involved in diagnosing and managing DLDs, as a stunning example of

how radiologists, pulmonologists, and pathologists can successfully cooperate to ensure the wellbeing of patients.

Anna Rita Larici Assistant Professor of Thoracic Imaging

Catholic University

Rome, Italy

Foreword

vii

Medical textbooks have evolved over the years, but most still serve as encyclopedic tomes, designed for reference use once a

diagnosis is being closely considered. But what written guidance exists for the physician seeking diagnostic answers in real

time, when the diagnosis is completely unknown?

The exceptional body of work presented by Dalpiaz and coworkers is just such a reference. The approach has tremendous

value for radiologists, pneumologists, and pathologists alike, emphasizing differential diagnosis based on key observations of

pattern and distribution of abnormalities identifi ed on HRCT. Further, the work is constructed to provide maximum benefi t to the

user through the application of a consistent format throughout including special annotations that serve to guide the reader in an

effi cient and highly user-friendly way.

Kevin O. Leslie, MD Professor and Consultant

Laboratory Medicine and Pathology

Mayo Clinic Arizona

Scottsdale, AZ, USA

Foreword Foreword

ix

As a clinician with a longstanding passion and a research interest for interstitial lung diseases, I still remember distinctly the day

when, back in 2006, I had for the fi rst time in my hands a new volume on the clinical features, pathology and HRCT patterns in

diffuse lung diseases. I was surprised to fi nd such a clear and rational description of the complex diagnostic algorithm behind

the recognition of the many and so different HRCT patterns for this group of patients. Even more so when I recognised the two

authors as well know Italian colleagues and friends! At that time, I was still working in Italy, more specifi cally in Modena, part of

a prolifi c Italian region, from which one of the authors was a native of. That book authored by Mario Maffessanti and Giorgia

Dalpiaz has been since then a faithful and useful companion of my clinical and research activity. Moreover, it has been a reliable

and friendly starting point for the younger colleagues interested in this challenging fi eld of our discipline.

When some weeks ago I received a nice message from Giorgia, anticipating the publication of a new Atlas of Diffuse Lung Diseases

(and kindly asking me for this preface), I became very excited to see if and how the previous excellent book, still sitting in my bookcase

(now in the United Kingdom) had been improved. Looking at the pre-print version of the atlas, three characteristics did catch my eye. First,

now the team is an all-female duo, both native from the same Emilia region: certainly one more reason to be proud of our strong, dedicated,

strong-willed and smart women, now that I have been working for the last 3 years abroad. Second, a colour-coded structure is even more

clear than in the previous book and now the BEST approach (please refer to the preface from the authors to know more about this), together

with the six colours strategy, is opening the mind while reading the text and is helping to have a comprehensive, but still detailed, view of

the diagnostic process. Lastly, the magic word “multidisciplinary” is on the front page, clearly anticipating the different and complementary

expertise of the two colleagues.

In fact, since 2006 a lot of change happened in this evolving and dynamic fi eld of respiratory medicine. We discovered that the

traditional combination of steroid and immunosuppressive therapy is associated with increased hospitalisation and mortality. This

new, groundbreaking discovery strongly argues now against the empiric use of this (and other) therapeutic combination in patients

with idiopathic pulmonary fi brosis. Similarly, the lack of consistent effi cacy of a simple regimen based on N -acetyl-cysteine and the

availability of effective and safe anti-fi brotic therapies has increased the urgency to make an accurate diagnosis in patient with

suspected idiopathic pulmonary fi brosis. Although the fi eld of diffuse lung diseases has benefi ted from updated clinical practice

guidelines that address multiple subtypes of interstitial lung diseases, nonetheless there remains signifi cant heterogeneity and

uncertainty in the practical application of existing classifi cation schema, with resulting heterogeneity in case classifi cation and

important implications for both clinical practice and future research. It is widely recognised that with clear divergence in the

management pathways of different disease subtypes, there is a need for a consistent and reproducible approach to diagnosis and

classifi cation that can be applied across diverse centres, ideally on a global scale.

Foreword Foreword

x

In this context, it is now clear to me that this Atlas of Diffuse Lung Diseases is the perfect companion of the fi rst book and

together will help to address the future challenges for clinicians dedicated to this important area of respiratory medicine. These

beautiful books will always occupy a privileged place in my bookshelf, wherever it will be.

Luca Richeldi Professor of Respiratory Medicine

Chair of Interstitial Lung Disease

University of Southampton

Southampton, UK

Foreword

Dalpiaz & Cancellieri Preface

Atlas of Diffuse Lung Diseases xi

The BEST Points of the Atlas This preface is inspired by the Italian author Beppe Severgnini’s book Life is a Journey. In choosing BEST, we did not wish to give ourselves a pat on the back; in fact, it is the acronym for this atlas’s four guiding concepts:

• Brevity • Encouragement • Simplicity • Teaching

Beppe Severgnini is a columnist for Corriere della Sera (one of Italy’s leading daily newspapers) and a contributing opinion writer for The New York Times. He is the author of 16 books and teaches at the University of Milan’s School of Journalism. Severgnini was awarded the O.B.E. and is a Commendatore della Repubblica Italiana.

Briefness and precision are essential qualities in these busy times, and the importance of having quick guides is crucial. This atlas is designed as an easy-to-use reference guide that identifi es and illustrates the key patterns of diffuse lung diseases observed on high-resolution computed tomography (HRCT). This atlas documents in great detail each pattern’s signs with close Rad-Path correlations. This book also allows a rapid view of HRCT characteristics and appearances of the individual diseases grouped on the basis of their prevalent radiological pattern.

Brevity is the soul of wit. From the second act of Hamlet, this is one of the countless expressions coined by William Shakespeare that has entered into common usage. With appropriate conciseness, it extols the virtue of expressing ideas in as few words as possible.

We all need a little encouragement! One should not to be concerned or put off by the wide number of DLDs that exist; in this atlas the task is made much easier by the inclusion of many diagnostic tools as well as a variety of tips and tricks (examples of which can be found in the Case-Based Glossary).

We would also nudge you to deal with DLDs using a multidisciplinary approach. Multidisciplinary diagnosis is considered the gold standard: it can improve the accuracy of diagnosis, avoid unnecessary tests (e.g. lung biopsies), and optimize patient management.

Nudge: Improving Decisions about Health, Wealth, and Happiness. Richard H. Thaler Cass R. Sunstein, 2008 Yale University Press.

Simplicity is the ultimate sophistication (Steve Jobs). Jobs saw simplicity as the means for conquering complex realities. He later added: It takes a lot of hard work to make something simple, to truly understand the underlying challenges and come up with elegant solutions.

In this atlas, we have removed anything that was not essential and used a simple and visually pleasing layout.

We have employed an innovative format that we believe is effective for achieving optimal learning and memorization. In a way similar to color-code maps (geographic, political, railway, etc.), different colors are used so as to assist in the quick memorization of the six different patterns.

The six pattern colors are:

Purple (septal pattern) ••••••

Pink (fibrosing pattern)Green (nodular pattern) Sky blue (alveolar pattern) Red (cystic pattern) Dark grey (dark lung pattern)

In addition to the description of each pattern’s signs and a selected choice of signs in the Case-Based Glossary, we also provide some images that are easy to remember and recognize; some of these resemble everyday things (e.g., butterfl y sign, cheerio sign, etc.).

Brevity

Encouragement

Simplicity

Teaching

Preface Dalpiaz & Cancellieri

Symbols are used to allow quick identifi cation of important concepts

Synonyms commonly used in clinical practice

Tips and tricks

Caveat! Be careful! Remember!

Expansion of a concept just outlined

Bibliographic reference related to the topic in the specifi c context

Differential diagnosis

This new atlas is a companion volume to the book entitled Diffuse Lung Diseases: Clinical Features, Pathology, HRCT, Mario Maffessanti & Giorgia Dalpiaz (Eds), Springer 2006. Both volumes share the same layout, use of graphics, and multidisciplinary approach to DLDs, but this atlas contains a greater number of Rad-Path fi gures.

Giorgia Dalpiaz (radiologist) and Alessandra Cancellieri (pathologist) are close friends and a longstanding Rad-Path team. This atlas is the result of decades of professional collaboration in daily practice as well as for the preparation of joint lectures and talks.

This atlas is intended for a wide range of healthcare professionals, in particular radiologists, pulmonologists, and pathologists. A further feature of this book is its interdisciplinary content.

Both of us affectionately dedicate this atlas to Prof. Mario Maffessanti, a very special and brilliant person.

We also wish to thank our families for their loving support.

Bologna, Italy Giorgia DalpiazBologna, Italy Alessandra Cancellieri

v

xii Atlas of Diffuse Lung Diseases

Symbols

About the Atlas

About the Editors

Target Readership

Dedication

Clinical Approach to Diffuse Lung Diseases PAGE 1

Classifi cation of DLDs DLDs Associated to Known Causes PAGE 2 Idiopathic DLDs

IPAF

Epidemiology and Prognosis

Clinical Evaluation Acute, subacute, chronic PAGE 4 History

Key symptoms and signs

Functional testing Spirometry PAGE 7 DLCO

Autoimmunity Laboratory fi ndings PAGE 9 Systemic symptoms and signs

The most frequent clinical settings IPF, NSIP, COP, sarcoidosis PAGE 11Bronchoalveolar lavage Procedure and normal values PAGE 13(BAL) Diagnostic role

TBNA, EBUS-TBNA Procedures and diagnostic role PAGE 18EUS-FNAEUS-B-FNATransbronchial Procedure PAGE 21biopsy (TBB) Diagnostic role

Transbronchial Procedure PAGE 23cryobiopsy Diagnostic role

Surgical lung biopsy Procedure PAGE 25in VATS Diagnostic role

The Multidisciplinary The role of MDA PAGE 26approach (MDA)

Thinking Through Pathology PAGE 27

Introduction and anatomy Secondary lobule PAGE 28Elementary lesions Defi ning lesions: neoplasm PAGE 31 Defi ning lesions: mixture Non-defi ning lesions: infl ammation Non-defi ning lesions: fi brosisHow to approach the Anatomic distribution PAGE 40diseases Patterns Ancillary histologic fi ndings

Contents Contents

Septal Pattern PAGE 49

Septal pattern Defi nition PAGE 50Septal signs Septal thickening PAGE 51 Subpleural thickening Peribronchovascular thickening Subset smooth PAGE 53and tableSubset nodular PAGE 54and table

Septal Diseases PAGE 55

Amyloidosis, interstitial Amyloidosis PAGE 56ECD Erdheim-Chester disease PAGE 58LC Lymphangitic carcinomatosis PAGE 60PE, interstitial Pulmonary edema, interstitial PAGE 62VOD Veno-occlusive disease PAGE 64

Fibrosing Pattern PAGE 67

Fibrosing pattern Defi nition PAGE 68Fibrosing key signs Honeycombing PAGE 69 Fibrosing reticulation and interface sign Traction bronchiectasis and bronchiolectasis Volume loss

Ancillary signs Ground-glass opacity PAGE 74 Lobular air trapping Disseminated pulmonary ossifi cation Table PAGE 77

Fibrosing Diseases PAGE 79

Asbestosis Asbestos-induced pneumoconiosis PAGE 80CVD Collagen vascular diseases – scleroderma PAGE 82Drug toxicity, chronic Methotrexate PAGE 84HP, chronic Hypersensitivity pneumonitis, chronic PAGE 86IPF Idiopathic pulmonary fi brosis PAGE 88NSIP Nonspecifi c interstitial pneumonia PAGE 90PPFE Pleuropulmonary fi broelastosis PAGE 92Sarcoidosis, chronic Sarcoidosis, chronic PAGE 94

xv

Nodular Pattern PAGE 97

Nodular pattern Defi nition PAGE 98Nodular signs Low-density (subsolid) nodules PAGE 99 High-density (solid) nodules

Cavitated nodules

Calcifi ed nodules

Nodules with halo sign

Subset lymphatic and table PAGE 104Subset random and table PAGE 105Subset centrilobular andtable PAGE 106Unusual distribution and table Galaxy sign PAGE 108 Cluster sign “Nodular” reversed halo sign

Nodular Diseases PAGE 113

EHE Epithelioid hemangioendothelioma PAGE 114FB Follicular bronchiolitis PAGE 116Hemorrhage Endometriosis PAGE 118HP, subacute Hypersensitivity pneumonitis, subacute PAGE 120HTL Hot tub lung PAGE 122Infection, miliary TB Tuberculosis, miliary PAGE 124LCH, early Langerhans cell histiocytosis, early PAGE 126LIP Lymphocytic interstitial pneumonia PAGE 128Metastases, hematogenous Metastases, hematogenous PAGE 130MPC “Metastatic” pulmonary calcifi cation PAGE 132PCH Pulmonary capillary hemangiomatosis PAGE 134RB-ILD Respiratory bronchiolitis-ILD PAGE 136Sarcoidosis Sarcoidosis PAGE 138Silicosis Silica-induced pneumoconiosis PAGE 144

xvi

Alveolar Pattern PAGE 145

Alveolar pattern Defi nition PAGE 146Alveolar key signs Ground-glass opacity PAGE 147 Consolidation

Ancillary signs Hypodense consolidation PAGE 149 Hyperdense consolidation

Cystic consolidation

Crazy paving

Head-cheese sign

Reversed halo sign (atoll sign)

Perilobular opacities

Lobular/sublobular opacities

Tree-in-bud sign, bronchiolar

Subset acute and table PAGE 160Subset chronic and table PAGE 161

Alveolar Diseases PAGE 163

Adenocarcinoma Adenocarcinoma PAGE 164AE-IPF Acute exacerbation of IPF PAGE 166AEP Acute eosinophilic pneumonia PAGE 168AIP Acute interstitial pneumonia PAGE 170ARDS Acute respiratory distress syndrome PAGE 172CEP Chronic eosinophilic pneumonia PAGE 174COP Cryptogenic organizing pneumonia PAGE 176DAH Diffuse alveolar hemorrhage in WG PAGE 178DIP Desquamative interstitial pneumonia PAGE 180Drug toxicity Amiodarone-induced lung disease PAGE 182FES Fat embolism syndrome PAGE 184Infection, acute (PJP) PJP PAGE 186Infection, chronic (TB) TB PAGE 188LP Lipoid pneumonia PAGE 192MALToma Mucosa-associated lymphatic tissue PAGE 194Metastases, aerogenous Metastases, aerogenous PAGE 196PAP Pulmonary alveolar proteinosis PAGE 198PE, alveolar Pulmonary edema, alveolar PAGE 200

xvii

Cystic Pattern PAGE 203

Cystic pattern Defi nition PAGE 204Cystic signs Thin-walled or not-walled cysts PAGE 205 Thick-walled cysts Cysts with irregular shape Cysts with regular shape

Bunch of grapes sign

Incidental lung cysts

Table of diseases PAGE 209

Cystic Diseases PAGE 211

BHD Birt–Hogg–Dubè PAGE 212Bronchiectasis, cystic Bronchiectasis, cystic PAGE 214CF Cystic fi brosis PAGE 216CPFE Combined pulmonary fi brosis and emphysema PAGE 218Emphysema, CL & PS Emphysema, centrilobular and paraseptal PAGE 220LAM Lymphangioleiomyomatosis PAGE 222LCH, advanced Langerhans cell histiocytosis, advanced PAGE 224LIP Lymphocytic interstitial pneumonia PAGE 226LTB papillomatosis Laryngotracheobronchial papillomatosis PAGE 228Metastases, cystic Metastases, cystic PAGE 230

Dark Lung Pattern PAGE 233

Dark lung pattern Defi nition PAGE 234Subset bronchiolar PAGE 235Subset vascular PAGE 237Tables PAGE 238

Dark Lung Diseases PAGE 239

CB Constrictive Bronchiolitis PAGE 240CPTE Chronic Pulmonary Thromboembolism PAGE 242DIPNECH DIPNECH PAGE 244Emphysema, panlobular Emphysema, panlobular PAGE 246SJS Swyer-James Syndrome PAGE 248

Case-Based Glossary with Tips & Tricks PAGE 251

Index PAGE 291