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.