Monograph

The ERS Monograph is the quarterly book series from the ERS.

Each Monograph covers a specific area of respiratory medicine, providing in-depth reviews that give clinicians at all levels a concise, comprehensive guide to symptoms, diagnosis and treatment.

Rare Diseases of the Respiratory System

European Respiratory Society Monograph, Vol. 100. 2023
ISBN (electronic): 978-1-84984-167-2
DOI: 10.1183/2312508X.erm10023

Diagnosing rare diseases can be challenging, and treating these conditions is complex because of their often quite specific needs and treatment options. To address this, the European Respiratory Society (ERS) has published Rare Diseases of the Respiratory System. Structured into thematic sections, the book covers: the identification of rare diseases of the respiratory system and their differential diagnosis; rare diseases of the lung interstitium; rare diseases of the airways or alveoli; and rare pulmonary vascular diseases. The Guest Editors and authors belong to and/or support the vision and mission of the European Reference Network for Rare Diseases of the Respiratory System (ERN-LUNG), which offers expert support to both patients and professionals. As such, this comprehensive book will prove an excellent resource for healthcare professionals, researchers and students interested in rare diseases of the respiratory system.

This site only provides online access to the book. To order a print copy, visit ersbookshop.com

Preface Peter M.A. Calverley Eur Respir Monogr 2023; 100: vii
Guest Editors Eur Respir Monogr 2023; 100: viii-x
Introduction Thomas O.F. Wagner, Marc Humbert, Marlies Wijsenbeek, Michael Kreuter, Helge Hebestreit Eur Respir Monogr 2023; 100: xi-xiii
List of abbreviations Eur Respir Monogr 2023; 100: xiv
How to identify rare diseases of the respiratory system Helge Hebestreit, Florian Gahleitner, Simon Veldhoen, Matthias Griese Eur Respir Monogr 2023; 100: 1-9
Differential diagnosis of reciprocal mimics of neoplastic and non-neoplastic pulmonary disorders: multidisciplinary approaches Nicolas Girard Eur Respir Monogr 2023; 100: 10-22
Interstitial lung diseases: an overview Theodoros Karampitsakos, Marlies Wijsenbeek, Jose D. Herazo-Maya, Argyris Tzouvelekis, Michael Kreuter Eur Respir Monogr 2023; 100: 23-39
Rare interstitial lung diseases of environmental origin Carlos Robalo Cordeiro, Tiago Alfaro, Sara Freitas Eur Respir Monogr 2023; 100: 40-52
Amyloidosis and the lungs and airways Joshua A. Bomsztyk, Jennifer H. Pinney, Helen J. Lachmann Eur Respir Monogr 2023; 100: 53-68
Diffuse cystic lung diseases including lymphangioleiomyomatosis Davide Elia, Antonella Caminati, Lisa Tescaro, Roberto Cassandro, Sergio Harari Eur Respir Monogr 2023; 100: 69-84
Bronchiolitis Venerino Poletti, Claudia Ravaglia, Alessandra Dubini, Sissel Kronborg-White, Salvatore Cazzato, Sara Piciucchi Eur Respir Monogr 2023; 100: 85-102
Pulmonary alveolar proteinosis Evelyn Lynn, Omaima Omar, Ali Ataya, Elisabeth Bendstrup, Alessandro N. Franciosi, Cormac McCarthy Eur Respir Monogr 2023; 100: 103-117
Primary ciliary dyskinesia Petra Pennekamp, Johanna Raidt, Kai Wohlgemuth, Heike Olbrich, Heymut Omran Eur Respir Monogr 2023; 100: 118-134
Cystic fibrosis and other ion channel-related diseases Simon Y. Graeber, Marcus A. Mall Eur Respir Monogr 2023; 100: 135-149
Bronchiectasis: from orphan disease to precision medicine Hayoung Choi, James D. Chalmers Eur Respir Monogr 2023; 100: 150-164
a₁-Antitrypsin deficiency and other rare forms of emphysema Joanna Chorostowska-Wynimko, Sabina Janciauskiene, Magdalena Pelc, Pavel Strnad, David Parr Eur Respir Monogr 2023; 100: 165-179
Pulmonary arterial hypertension Sarah Cullivan, Sean Gaine Eur Respir Monogr 2023; 100: 180-191
Chronic thromboembolic pulmonary hypertension Marion Delcroix, Laurent Godinas, Rozenn Quarck, Catharina Belge, Bart Meyns, Geert Maleux, Tom Verbelen Eur Respir Monogr 2023; 100: 192-203
Pulmonary hypertension in orphan lung diseases David Montani, Mithum Kularatne, Etienne-Marie Jutant, Marc Humbert Eur Respir Monogr 2023; 100: 204-223
Hepatopulmonary syndrome: a liver-induced oxygenation defect Laurent Savale, Fabien Robert, Ly Tu, Marie-Caroline Certain, Audrey Baron, Audrey Coilly, Léa Duhaut, Marc Humbert, Christophe Guignabert, Olivier Sitbon Eur Respir Monogr 2023; 100: 224-236
Systemic inflammatory diseases with lung involvement Eirini Vasarmidi, Eleni Bibaki, Katerina Antoniou Eur Respir Monogr 2023; 100: 237-253
ANCA-associated vasculitis and other pulmonary haemorrhage syndromes Samuel Falde, Ulrich Specks Eur Respir Monogr 2023; 100: 254-266
Eosinophilic granulomatosis with polyangiitis Yann Nguyen, Loïc Guillevin Eur Respir Monogr 2023; 100: 267-280
Idiopathic eosinophilic pneumonias Vincent Cottin Eur Respir Monogr 2023; 100: 281-292
Sarcoidosis Francesco Bonella, W. Ennis James, Paolo Spagnolo Eur Respir Monogr 2023; 100: 293-309
Granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency Heba M. Bintalib, Siobhan O. Burns, John R. Hurst Eur Respir Monogr 2023; 100: 310-319
Thoracic endometriosis and catamenial pneumothorax Antonio Bobbio, Vincent de Pauw, Imane Lefqih, Antoine Sion, Marco Alifano Eur Respir Monogr 2023; 100: 320-330
Chronic lung allograft dysfunction after lung transplantation Berta Saez Gimenez, Merel Hellemons, Stijn E. Verleden, Jens Gottlieb, Geert M. Verleden Eur Respir Monogr 2023; 100: 331-342
Malformations and idiopathic disorders of the trachea Valentina Luzzi, Francesca Conway, Diletta Cozzi, Luca Ciani, Leonardo Giuntoli, Marco Trigiani, Sara Tomassetti Eur Respir Monogr 2023; 100: 343-356
Rare diseases of respiratory drive Katie Rose, Tamarin Foy, Christopher Grime, Ian P. Sinha Eur Respir Monogr 2023; 100: 357-366
Pleural mesothelioma Joachim G.J.V. Aerts, Jan P. van Meerbeeck Eur Respir Monogr 2023; 100: 367-380