Main description:

Pathobiology of Rare Lung Diseases provides an overview of the pathogenic mechanisms underlying this broad variety of conditions. This up-to-date reference focuses on the molecular mechanisms involved and provides insights into the changing therapeutic landscape for rare lung diseases. The study of rare lung diseases can inform our understanding of pathogenic mechanisms of more common disorders. Many of these conditions can be studied in detail, and an increasing wealth of knowledge exists regarding the pathogenesis of these conditions. Some are monogenic, while others have clearly defined pathways that allow for deep understanding and exploration of the molecular basis of disease.

As a considerable leap has been made in the last decade in rare lung disease research, this book comprehensively covers these scientific breakthroughs to inform readers on future potential, strategies and the direction of research for these conditions.

Contents:

Part 1. Genetic Causes of Lung Disease
1. Genetics of Fibrotic Lung Disease
2. Multigene Familial ILD
3. Monogenic

Part 2. Disorders of Surfactant Homeostasis
1. Primary pulmonary alveolar proteinosis
2. Secondary pulmonary alveolar proteinosis
3. Disorders of surfactant production

Part 3. Childhood Interstitial Lung Diseases
1. Alveolar capillary dysplasias
2. Pulmonary interstitial glycogenosis
3. Neuroendocrine hyperplasia of infancy
4. Lung growth abnormalities

Part 4. Disorders Associated with Autoimmunity and Inflammation
1. Rheumatoid arthritis associated lung disease
2. Systemic sclerosis associated lung disease
3. Myositis associated disorders
4. IgG4-related Lung Disease
5. Anti-glomerular basement disease
6. Pulmonary manifestations of vasculitis
7. Pulmonary amyloidosis
8. Cryptogenic organizing pneumonia
9. Hypersensitivity pneumonitis
10. Sarcoidosis

Part 5. Neoplastic Disorders
1. Lymphangioleiomyomatosis
2. Pulmonary Langerhans cell histiocytosis
3. Diffuse idiopathic pulmonary neuroendocrine hyperplasia (DPNECH)

Part 6. Smoking-associated ILD
1. Desquamative interstitial pneumonia
2. Respiratory-bronchiolitis ILD
3. Combined pulmonary fibrosis and emphysema

Part 7. Pulmonary Complications of Rare Diseases
1. Primary immunodeficiencies
2. Sickle cell disease
3. Congenital diaphragmatic hernia
4. Lymphatic anomalies
5. Yellow nail syndrome
6. Pulmonary arterial hypertension