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  • Interstitial lung diseases (ILDs) are a heterogeneous group of conditions that involve the alveolar walls and perialveolar tissue. The ILDs are nonmalignant and are not caused by any defined infectious agents. Approximately 180 known individual diseases are characterized by interstitial lung involvement, either as primary disease or as a significant part of a multiorgan process, as occurs in collagen vascular diseases. The initial response is inflammation in the air spaces and alveolar walls. If the disease is chronic, inflammation spreads to adjacent portions of the interstitium and vasculature and eventually produces interstitial fibrosis. Scarring and distortion of lung tissue leads to significant derangement of gas exchange and ventilatory function. One approach to classification is to separate ILDs into two groups based on 1) known causes or 2) unknown causes. Each group can be further subdivided based on the presence or absence of granulomas.
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