6.1c) consists of numerous punc-tate densities essentially ranging in diameter from 1 mm (barely visible as an individual lesion) to 5 mm, although a few slightly larger nodular lesions can be interspersed. The key-findings on the X-ray are: ill-defined homogeneous opacity obscuring vessels; Silhouette sign: loss of lung/soft tissue interface; Air-bronchogram; Extention to the pleura or fissure, but not crossing it; No volume loss; Chronic diseases are indicated in red. Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases. This appearance is virtually diagnostic, although under very rare circumstances both pulmonary papillomatosis and paragonimiasis may mimic cystic bronchiectases. 7-10) tend to be well defined and can be seen in numerous interstitial lung diseases. Fig. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. Calcification occurs. pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. A nodular pattern (Fig. 7-14) is a phenomenon that occurs in the presence of severe lung fibrosis and distortion of lung architecture, in which the fibrous tissue produces traction on the bronchial walls, resulting in irregular bronchial dilation. 6.1a). It is often accompanied by other signs of interstitial lung disease, especially the patterns associated with reticular opacities and architectural distortion. They are most numerous at the base of the lungs. pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. Fairly well defined in silicosis and poorly in berylliosis. Exclusively these symptomatic cases are designated as respiratory bronchiolitis-interstitial lung disease. Although these disorders have frequently been referred to as interstitial lung diseases, many also involve the alveolar spaces. Localized fibrotic changes are often found in the chronic stage of a disease (e. g., tuberculosis and radiation pneumonitis). Nonspecific interstitial pneumonia (NSIP): CT appearance. Many diseases demonstrate more than one pattern (see Box 7-3). Prone scans are necessary to differentiate dependent atelectasis, a physiologic phenomenon that usually occurs posteriorly in the basal areas of the lungs, from true early interstitial lung disease. Peripheral septal lines lie perpendicular to the pleural surface (open arrow). Thick-walled cystic spaces can be seen subpleurally in the bases. The five classifications of patterns of diffuse parenchymal lung disease on HRCT are linear or reticular opacities, nodular opacities, cystic lesions, ground-glass opacification, and parenchymal consolidation (i.e., alveolar or airspace disease). Discrete and extremely sharply defined, less than 1 mm in diameter. Interstitial Lung Disease Jeffrey T. Chapman The term interstitial lung disease (ILD) refers to a broad category of lung diseases rather than a specific disease entity.1,2 It includes a variety of illnesses with diverse causes, treatments, and prognoses. However, they can usually be differentiated from honeycombing by their larger and less uniform size and by the presence of tiny meniscus-like fluid levels at the bottom of these cystic lesions. 6.16). Fig. Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. Box 7-3 Patterns of Opacities in Interstitial Lung Disease, Usual interstitial pneumonitis (idiopathic pulmonary fibrosis)*, Fibrosis associated with collagen vascular disease, Idiopathic pulmonary fibrosis (honeycombing), Congestive heart failure (interstitial edema). Pleural disease may take one of several forms (Box 7-5). When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. A honeycomb pattern is characterized by round or oval cystic lesions with a diameter up to 1 cm (Fig. 6.1a–f Patterns of interstitial lung disease. Central septal lines outline the secondary pulmonary lobule, which appears as a polyhedral structure. Miliary and larger with mid and upper lung fields predominance. A purely nodular pattern is found with the hematogenous spread of certain infections and tumors, but can also be encountered with other diseases (Table 6.1). Pneumoconiosis caused by radiopaque dusts (iron, tin, barium, antimony and rare-earth compounds) (Figs. * More sensitive in detection of adenopathy than radiography. Involvement of the interstitium within the lobule around the central artery and bronchiole or related to the interlobular septum may produce a fine reticular pattern within the lobule itself (Fig. Some types of autoimmune diseases, such as rheumatoid … Bronchial wall thickening, Thickening of the interlobular septa (Fig. Extrinsic allergic alveolitis (e.g., farmer’s lung, bird-fancier’s lung, mushroom-worker’s lung, bagassosis, and others) (Figs. Fig. Density of the tiny nodules depends on the atomic number of the inhaled element. 6.7). Intrauterine infection with high mortality rate. Kerley lines refer to septal lines that are thickened either by fluid accumulation, cellular infiltration, or connective tissue proliferation within the interlobular septa. Multiple small nodules are scattered throughout both lung fields. 6.2). Ill-defined nodules that are 6 mm to 1 cm in diameter may be associated with airspace consolidation around the peripheral bronchioles, particularly around the terminal bronchiole in the center of the secondary pulmonary lobule. Mycoplasma and viral pneumonias can present in their early stages as localized interstitial diseases of fine reticular appearance before the extension of the inflammation into the air spaces causes a consolidation. Truly localized interstitial lung disease is relatively rare and most often of an infectious etiology. It may present radiographically in a reticulonodular pattern, too, but this presentation is also found with many other disorders, including various acute abnormalities that can resolve completely with time. In the lower lobes, there are multiple, small, centrilobular ground-glass nodules. Adenopathy (Box 7-6), which is recognized on standard radiographs, is associated with silicosis and sarcoidosis, lymphangitic carcinomatosis, and lymphoma. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. f Bronchiectases evident as cystic lesions varying considerably in size and characteristically containing small air-fluid levels (arrows). Cystic abnormalities include honeycombing, traction bronchiectasis, lung cysts, and cavitary nodules. 7-8) within the secondary pulmonary lobule (i.e., centrilobular vessel) may occur in a number of interstitial lung diseases. Interstitial lung disease (ILD) consists of a large and heterogeneous group of rare pulmonary disorders, characterized by abnormalities involving the alveoli and airway. Calcification extremely rare. c Nodular pattern (silicosis). This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). Traction bronchiectasis (Fig. Table 6.3 summarizes all disorders that demonstrate radiographically a diffuse reticular or reticulonodular pattern characteristic of interstitial lung disease. Thickening of the axial interstitium (i.e., interstitium in a peribronchovascular location) (Fig. Fig. Fig. Respiratory bronchiolitis-interstitial lung disease affects active smokers with at least 30 pack years (number of cigarette packs per day multiplied by the number of years smoked) predominantly between the ages of 40 and 50 years. Honeycombing is the only dependable radiographic sign of interstitial fibrosis. Primarily lung disorders, and pulmonary function tests the pulmonary artery computed (. Preferences in the right side desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial disease. 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