Pathobiology of Rare Lung Diseases provides a detailed overview of the pathogenic mechanisms underlying this broad variety of conditions. This up-to-date reference focuses on the molecular mechanisms involved and provides insight into how this is changing the therapeutic landscape in rare lung diseases. The study of rare lung diseases often can inform the 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 and others have clearly defined pathways which allow a deep understanding and exploration of the molecular basis of disease. A considerable leap has been made in the last decade in rare lung disease research and this book comprehensively covers these scientific breakthroughs and inform the readers on the future potential strategies and direction of research for these conditions.