Abstract
There are more than 200 clinical forms of diffuse lung disease (DLD) with a wide variety of causes and associations. The physician faced with a suspected case of DLD must therefore have an organized and systematic approach to the diagnostic evaluation. One such approach is to divide these processes into five broad clinical categories. These are: primary disease related, environmental exposure related, drug induced, collagen-vascular associated, and the idiopathic interstitial pneumonias. The history and physical examination is of critical importance in identifying possible exposures to occupational and environmental substances known to cause DLD, documenting drug exposures, and eliciting symptoms and signs of collagen-vascular disease or other primary diseases. Radiographic, physiologic, and laboratory evaluation can often confirm the presumptive diagnosis. Importantly, a minority of patients will have no identifiable cause of DLD after careful clinical evaluation. These patients are considered to have idiopathic DLD, termed idiopathic interstitial pneumonias. The current ATS/ERS classification schema for the idiopathic interstitial pneumonias includes seven distinct subgroups associated with distinctive histopathological patterns of injury and repair: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia, acute interstitial pneumonia, cryptogenic organizing pneumonia, lymphocytic interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia.
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Collard, H.R., King, T.E. (2012). Diffuse Lung Disease: Classification and Evaluation. In: Baughman, R., du Bois, R. (eds) Diffuse Lung Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-9771-5_6
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DOI: https://doi.org/10.1007/978-1-4419-9771-5_6
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