Transbronchial cryobiopsy in diffuse parenchymal lung disease

161 Pages·2019·18.458 MB·English

by  Venerino Poletti

Transbronchial
cryobiopsy in diffuse
parenchymal lung
disease
Venerino Poletti
Editor
123
Transbronchial cryobiopsy in diffuse
parenchymal lung disease
Venerino Poletti
Editor
Transbronchial
cryobiopsy in diffuse
parenchymal lung
disease
Editor
Venerino Poletti
Department of Diseases of the Thorax
Ospedale Morgagni-Pierantoni
Forlì
Italy
ISBN 978-3-030-14890-4 ISBN 978-3-030-14891-1 (eBook)
https://doi.org/10.1007/978-3-030-14891-1
© Springer Nature Switzerland AG 2019
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Preface
This book originates from postgraduate meetings on transbronchial lung cryo-
biopsy annually held in Ravenna (Italy) since 2014 (endorsed by the Italian
Association of Hospital Pulmonologists (Associazione Italiana Pneumologi
Ospedalieri, AIPO)), during which collaborations with experts around the
world on this specific topic were started or consolidated. It is also the fruit of
the experience acquired in the GB Morgagni Hospital in Forlì (Italy) in which
transbronchial cryobiopsy was introduced in clinical activities since 2010. In
this center, more than 1000 transbronchial lung biopsy procedures were car-
ried out so far. Finally during this period, I had the opportunity to be invited in
different centers around the world to present and discuss data on this new
technique and also to be appointed as Full Professor of Pulmonary Medicine
in the Aarhus University Hospital, Aarhus (Denmark). In this period, this pro-
cedure was introduced in the clinical practice also in that institute. Therefore,
suggestions provided by people I met during these meetings and the experi-
ence accumulated in Denmark contributed to build up the Forlì approach to
transbronchial cryobiopsy and are condensed in this book.
The objective of this book—after a concise clinical and radiologic over-
view of diffuse parenchymal lung diseases—is to provide technical informa-
tion on this new technique, illustrate the modalities by which lung samples
may be obtained using the machinery, and discuss the advantages and limits
of transbronchial cryobiopsy compared to surgical lung biopsy (performed in
intubated patients or in the “awaked” modality). The last part of the book is
dedicated to specific disorders approached also using information obtained
using this new bioptic method.
The book is meant to be practical, clinically oriented, and up-to-date.
Also, despite being it a multiauthored work, it was my intention to have a
uniform style, and at the end, it wasn’t a great effort to reach it since a large
part of the authors have been collaborating, sharing ideas and projects for a
long time, and the younger are or have been students of the older ones.
I hope that the positive feedbacks will far outweigh any negative ones.
However, if the objectives were not reached, the responsibility is only mine.
The book is dedicated to the memory of my parents Elisabetta and
Francesco, as well as to all students and pupils who gave and are giving me
enthusiasm and always new ideas.
Forlì, Italy Venerino Poletti
23 January 2019
v
Contents
Part I I ntroduction to Diffuse Parenchymal Lung Disease
1 Diffuse Parenchymal Lung Disease: A Clinical Overview . . . . . 3
Venerino Poletti
2 Radiologic-Pathologic Correlations in Diffuse
Parenchymal Lung Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Sara Piciucchi
3 Multidisciplinary Discussion in Diffuse Parenchymal
Lung Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
Silvia Puglisi, Jay H. Ryu, Sara Tomassetti,
and Venerino Poletti
Part II T ransbronchial Cryobiopsy
4 Cryobiopsy: Physics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
Sara Colella
5 Technique and Equipment in Transbronchial Cryobiopsy . . . . . 47
Sara Colella
6 Complications of Transbronchial Cryobiopsy . . . . . . . . . . . . . . . 59
Claudia Ravaglia
7 Histology for Transbronchial Lung Cryobiopsy Samples . . . . . . 67
Tamiko Takemura, Tomohisa Baba, Takashi Niwa,
and Takashi Ogura
8 Pathologic Considerations in Transbronchial Cryobiopsy . . . . . 75
Alberto Cavazza, Maria Cecilia Mengoli, Alessandra Dubini,
Giulio Rossi, Rita Bianchi, and Thomas V. Colby
9 Immunohistochemistry and Molecular Biology
in Transbronchial Cryobiopsies . . . . . . . . . . . . . . . . . . . . . . . . . . . 81
Marco Chilosi, Lisa Marcolini, Anna Caliò,
and Venerino Poletti
vii
viii Contents
Part III Clinical Role of Transbronchial Cryobiopsy
10 Clinical Meaning of Transbronchial Cryobiopsy . . . . . . . . . . . . . 103
Silvia Puglisi, Claudia Ravaglia, Antonella Arcadu,
Sara Tomassetti, and Venerino Poletti
11 Idiopathic Pulmonary Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Venerino Poletti, Antonella Arcadu, Sissel Kronborg-White,
and Marco Chilosi
12 Chronic Hypersensitivity Pneumonitis . . . . . . . . . . . . . . . . . . . . . 117
Claudia Ravaglia and Venerino Poletti
13 Collagen Vascular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123
Martina Bonifazi, Francesca Barbisan, and Stefani Gasparini
14 Lymphangioleiomyomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133
Giulio Rossi, Mirca Valli, Alessandra Dubini,
and Paolo Spagnolo
15 Pleuroparenchymal Fibroelastosis . . . . . . . . . . . . . . . . . . . . . . . . 141
Maria Cecilia Mengoli, Thomas Colby, Alessandra Dubini,
Giulio Rossi, and Alberto Cavazza
16 Acute Lung Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149
Venerino Poletti, Giovanni Poletti, Christian Gurioli,
Carlo Gurioli, and Alessandra Dubini
17 Disseminated Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Venerino Poletti, Angelo Carloni, and Marco Chilosi
18 Miscellaneous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163
Venerino Poletti, Antonella Arcadu, Christian Gurioli,
Fabio Sultani, Linda Tagliaboschi, Carlo Gurioli,
and Giovanni Poletti
Part I
Introduction to Diffuse
Parenchymal Lung Disease
1
Diffuse Parenchymal Lung Disease:
A Clinical Overview
Venerino Poletti
1.1 Introduction The clinical profiles with which these diseases
manifest are variegated from very acute onset
Diffuse parenchymal lung disease (DPLD) is a with respiratory failure needing invasive respira-
generic term for a large group of disorders. A tory supports to mild chronic symptoms lasting
definition based on precise criteria to identify for more than 6 months (dry cough, dyspnea on
these disorders is not yet universally accepted. A effort) (Table 1.2). Diagnosis is a complex pro-
“morphologic definition” includes in this group cess beset with pitfalls—often representing the
all the disorders that are characterized by a patho- balancing of different uncertainties—that starts
logic accumulation/infiltration of extracellular having in mind a model based on pathophysi-
substances, fluids, or cells in the structures of ologic elements and other scientific knowledges.
the secondary pulmonary lobule [1]. Secondary Thereafter it requires acquisition of data deriving
pulmonary lobule is an anatomic and functional from a comprehensive clinical history, a careful
unit supplied by a cluster of three to five terminal physical examination, as well as clues provided
bronchioles, and it is usually separated from other by laboratory and pulmonary function tests.
secondary pulmonary lobules by connective tis- Imaging, mainly high-resolution CT has a piv-
sue septa. It is irregularly polyhedral in shape and otal role in the detection of these disorders and
approximately 1–2.5 on each side [2]. This accu- in the diagnostic workup providing information
mulation/infiltration is, by definition, not limited robust enough to draw up a differential diagnosis
to one single lobe. This definition is quite inclu- list and sometimes identifying pathognomonic
sive because almost all lung disorders may pres- features. More invasive procedures are deemed
ent with this morphologic background. However necessary only when these steps are inconclusive
in this huge group are included systemic disor- or do not allow a confident clinical-radiological
ders in which the lung is one of the main organs hypothesis.
involved, idiopathic diseases limited to the lungs,
and diffuse parenchymal disorders of known
cause or well-known pathogenesis (Table 1.1). 1.2 Pathophysiology of DPLD
Due to the variety of entities included under the
V. Poletti (*) DPLD umbrella term, it is virtually impossible to
Department of Diseases of the Thorax, have a common pathogenetic scheme. The struc-
Ospedale Morgagni-Pierantoni, Forlì, Italy
tures injured are heterogeneous including bron-
Department of Respiratory Diseases and Allergy, chioles (terminal and respiratory), alveolar septa,
Aarhus University Hospital, Aarhus, Denmark
© Springer Nature Switzerland AG 2019 3
V. Poletti (ed.), Transbronchial cryobiopsy in diffuse parenchymal lung disease,
https://doi.org/10.1007/978-3-030-14891-1_1
4 V. Poletti
Table 1.1 Classification of diffuse parenchymal lung disorders
Systemic disorders Unknown cause Known cause/pathogenesis
Sarcoidosis IPF Drugs/radiation
Infections
CVDs NSIP Organic exposure (hypersensitivity
pneumonitis)
Neoplastic (metastases, lymphoproliferative disorders, COP Inorganic exposure (silicosis,
LAM, LCH, myeloid disorders) asbestosis)
Immunodeficiencies AIP Smoking related (RB-ILD, DIP, SRIF)
Telomeropathies (Dyskeratosis congenita, ...) LIP Pulmonary alveolar proteinosis
Inborn errors of metabolism (Niemann-Pick, Gaucher, PPFE Pulmonary alveolar microlithiasis
Hermansky-Pudlak, Fabry, Mucopolysaccharidoses)
Neurofibromatosis Chronic Mutations in genes coding for
eosinophilic surfactant proteins or in ABCA3
pneumonia transporter gene
Tuberous sclerosis
Hypereosinophilic syndrome Acute eosinophilic
pneumonia
IgG4-related disease
Infections
CVD collagen vascular disease, LAM lymphangioleiomyomatosis, LCH Langerhans cell histiocytosis, IPF idiopathic
pulmonary fibrosis, NSIP nonspecific interstitial pneumonia, COP cryptogenic organizing pneumonia, AIP acute inter-
stitial pneumonia, LIP lymphocytic interstitial pneumonia, PPFE pleuroparenchymal fibroelastosis, RB respiratory
bronchiolitis, DIP desquamative interstitial pneumonia, SRIF smoking-related interstitial fibrosis, ABCA3 ATP-binding
cassette subfamily A member 3
Table 1.2 Time course of disease onset interlobular septa, small pulmonary and bronchio-
Acute (in days to Subacute Chronic lar arteries, capillaries and veins, and lymphatics.
few weeks) (<3 months) (>3 months) These structures have also a different embryogenic
AIP HP IPF
origin and different regenerative machineries. The
Smoking-related
pathogenetic events in DPLD may be divided into
ILDs
five patterns. The neoplastic and infectious pro-
HP
AEP COP LCH cesses have pathogenetic events that are in com-
DAH/capillaritis NSIP LAM mon with all the other organs or systems. However
Drug-induced Drug-induced Drug-induced in the lungs some events taking place in the alveo-
ILDs ILDs ILDs
lar spaces (production of degradating enzymes in
Antisynthetase Sarcoidosis Sarcoidosis
lymphangioleiomyomatosis) may lead to charac-
syndrome
teristic cystic changes. Recently lymphangioleio-
AFOP Chronic
eosinophilic myomatosis has been reclassified as a low-grade
pneumonia sarcoma (belonging to the so-called PEComas)
Infections Infections Infections
and Langerhans cell histiocytosis and Erdheim-
Acute
Chester disease as inflammatory myeloid neo-
exacerbation of
IPF plasms with specific drug-targetable mutations
along the RAS-RAF mitogen-activated protein
AIP acute interstitial pneumonia, AEP acute eosinophilic
pneumonia, DAH diffuse alveolar hemorrhage, AFOP kinase (MEK) and extracellular signal-regulated
acute fibrinous organizing pneumonia, IPF idiopathic pul- kinase (ERK) signaling cascade [3–5].
monary fibrosis, HP hypersensitivity pneumonitis, COP
The other three pathophysiologic patterns left
cryptogenic organizing pneumonia, NSIP nonspecific
are granulomatous inflammation/fibrosis, inflam-
interstitial pneumonia, LCH Langerhans cell histiocyto-
sis, LAM lymphangioleiomyomatosis mation/fibrosis, and, finally, alveolar stem cell