Abstract
Interstitial lung diseases (ILDs) are a set of heterogeneous lung diseases characterized by inflammation and, in some cases, fibrosis of the space between alveoli. These lung conditions lead to dyspnea, cough, abnormalities in gas exchange, restrictive physiology (characterized by decreased lung volumes), hypoxemia, and, if progressive, respiratory failure. In some cases, ILD can be caused by systemic disease or environmental exposures. The ability to treat or cure these ILDs varies based on the subtype, and in many cases lung transplantation remains the only curative therapy. There is a growing body of evidence that both common and rare genetic variants are important to the development and to the clinical manifestation of many of the ILDs. This chapter will review the current understanding genetic risk and ILD.
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