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Diffuse parenchymal lung diseases include a large number (>200) of heterogeneous conditions that affect the lung parenchyma with varying degrees of inflammation and fibrosis. While remodeling of the interstitial space, the region between the epithelium and endothelium, tends to be the dominant site of involvement for most of the interstitial lung diseases (ILDs), it is important to recognize the prominent role of the alveolar epithelium and endothelial cells (including both airways and vessels) in the pathogenesis of these interstitial lung disorders.

Despite the diverse array of conditions, most patients ultimately diagnosed with an ILD will come to medical attention with reports of progressive exertional dyspnea or a persistent dry cough. However, because some ILDs are part of multisystem disorders, some patients will be identified based on non-respiratory symptomatology (e.g., skin thickening in the setting of systemic sclerosis, Chap. 353) or physical examination findings (e.g., ulnar deviation of the fingers in the setting of rheumatoid arthritis [RA], Chap. 351). Additionally, ILDs can also be identified incidentally based on the results of abnormal pulmonary function tests, chest x-rays (CXRs), computed tomography (CT) studies of both the chest and abdomen (which can both visualize, at least a portion, of the lung parenchyma), and positron emission tomography (PET) scans. It is important to remember that ILDs can be associated with high rates of morbidity and mortality, and although prognosis depends on both disease extent and specificity, this fact makes these important disorders to recognize in a timely manner.

Owing to a variety of clinical presentations, as well as overlapping imaging and histopathologic findings (Table 287-1), ILDs can be difficult to diagnose. A generally accepted central tenet of ILD diagnosis is that the combined weight of clinical data, laboratory studies, pulmonary function testing, imaging findings, and histopathology (if obtained) are jointly required to make a confident diagnosis. No single piece of data confers a diagnosis alone. For example, a lung biopsy demonstrating a usual interstitial pneumonia (UIP) pattern is helpful in diagnosing a patient with idiopathic pulmonary fibrosis (IPF) but can also be present in some connective tissue diseases (CTDs) (e.g., RA-associated ILD, Chap. 351). In light of this challenge, most ILD centers recommend a multidisciplinary approach to the diagnosis (and in some cases the management) of ILDs. An example of a multidisciplinary approach might include a conference attended by pulmonologists, rheumatologists, radiologists, and pathologists where all of the data generated on a patient can be discussed and reviewed jointly by those with unique sets of expertise in the care of patients with ILD.

TABLE 287-1Common Interstitial Lung Disease Findings

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