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  • Insidious onset of progressive dyspnea and nonproductive chronic cough.

  • Tachypnea, small lung volumes, bibasilar dry rales; digital clubbing and right heart failure with advanced disease.

  • Chest radiographs with low lung volumes and patchy distribution of ground glass, reticular, nodular, reticulonodular, or cystic opacities.

  • Reduced lung volumes, pulmonary diffusing capacity, and 6-minute walk distance; hypoxemia with exercise.

Interstitial lung disease, or diffuse parenchymal lung disease, comprises a heterogeneous group of disorders that share common presentations (dyspnea), physical findings (late inspiratory crackles), and chest radiographs (septal thickening and reticulonodular changes).

The term “interstitial” is misleading since the pathologic process usually begins with injury to the alveolar epithelial or capillary endothelial cells (alveolitis). Persistent alveolitis may lead to obliteration of alveolar capillaries and reorganization of the lung parenchyma, accompanied by irreversible fibrosis. The process does not affect the airways proximal to the respiratory bronchioles. At least 180 disease entities may present as interstitial lung disease. Table 9–16 outlines a selected list of differential diagnoses of interstitial lung disease. In most patients, no specific cause can be identified. In the remainder, medications, a variety of organic and inorganic dusts, and connective tissue disease are the principal causes. The history—particularly the occupational and medication history—may provide evidence of a specific cause. The presence of diffuse parenchymal lung disease in the setting of an established connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis-dermatomyositis, Sjögren syndrome, and other overlap conditions, is suggestive of the cause. In some cases, lung disease precedes the more typical manifestations of the underlying connective tissue disease by months or years.

Table 9–16.Differential diagnosis of interstitial lung disease (listed alphabetically within category).

Known causes of interstitial lung disease are dealt with in their specific sections. The important idiopathic forms are discussed below.

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