Rare Diseases of the Respiratory System

Edited by Thomas Wagner, Marc Humbert, Marlies Wijsenbeek-Lourens, Michael Kreuter and Helge Hebestreit

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.

Contents list
1. How to identify rare diseases of the respiratory system
2. Differential diagnosis of reciprocal mimics of neoplastic and non-neoplastic pulmonary disorders; multidisciplinary approaches

Rare diseases of the lung interstitium
3. Interstitial lung diseases: an overview
4. Rare interstitial lung diseases of environmental origin
5. Amyloidosis and the lungs and airways
6. Diffuse cystic lung diseases including lymphangioleiomyomatosis

Rare diseases of the airways or alveoli
7. Bronchiolitis
8. Pulmonary alveolar proteinosis
9. Primary ciliary dyskinesia
10. Cystic fibrosis and other ion channel-related diseases
11. Bronchiectasis: from orphan disease to precision medicine
12. Alpha-1-antitrypsin deficiency and other rare forms of emphysema

Pulmonary vascular diseases
13. Pulmonary arterial hypertension
14. Chronic thromboembolic pulmonary hypertension
15. Pulmonary hypertension in orphan lung diseases
16. Hepatopulmonary syndrome: a liver-induced oxygenation defect

Rare lung diseases in systemic inflammatory disorders
17. Systemic inflammatory diseases with lung involvement 
18. ANCA-associated vasculitis and other pulmonary haemorrhage syndromes
19. Eosinophilic granulomatosis with polyangiitis
20. Idiopathic eosinophilic pneumonias
21. Sarcoidosis
22. Granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency

Other rare lung diseases
23. Thoracic endometriosis and catamenial pneumothorax
24. Chronic lung allograft dysfunction after lung transplantation
25. Malformations and idiopathic disorders of the trachea
26. Rare diseases of respiratory drive
27. Pleural mesothelioma
28. Mesothelioma and other rare tumours of the respiratory system

Print ISBN: 978-1-84984-166-5
eISBN: 978-1-84984-167-2
EPUB ISBN: 978-1-84984-168-9