Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. In interstitial disease it is produced when the fine reticulogranular pattern has progressed to such an extent that the overall density of the involved lung is increased, but the individual interstitial lesion is no longer recognizable. These diseases represent specific clinicopathologic entities characterized by varying degrees of lung parenchymal inflammation and fibrosis. 6.1a). 7-6) occurs in many diseases, such as lymphangitic spread of carcinoma, pulmonary fibrosis, and sarcoidosis. Cystic bronchiectases may produce a radiographic picture similar to honeycombing. The nodular pattern (Fig. It parallels the pleura and is a few millimeters thick (see Chapter 8). 6.14 and 6.15). Fig. The majority of interstitial lung diseases involve both lungs, or stated differently, the interstitial disease is usually diffuse, although some areas may be more affected and others more or less spared. Fig. c Nodular pattern (silicosis). Cylindrical bronchiectases present as tubular opacities with parallel walls of 1 mm or slightly larger thickness. Occasionally, lines that are 2.5 cm long and that outline more than one lobule can be identified, particularly in the periphery of the lung. The chest radiograph, however, is often nonspecific. Although rare, they are classified with similar clinical, radiological, physiologic, or pathologic signs. The thickening may be smooth or nodular. Large masses of fibrous tissue seen centrally in the upper lobes are associated with traction bronchiectasis (arrow). Box 7-8 High-Resolution Computed Tomography Findings for Linear Opacities, Thickening of bronchovascular bundles (axial), Interlobular septal thickening (septal lines). In interstitial disease it is produced when the fine reticulogranular pattern has progressed to such an extent that the overall density of the involved lung is increased, but the individual interstitial lesion is no longer recognizable. Plate-like (discoid) atelectases and localized fibrotic strands can be differentiated from Kerley lines by their lack of a characteristic anatomic location and by the great variation in length and width of these densities. Central septal lines outline the secondary pulmonary lobule, which appears as a polyhedral structure. The fibrotic disorders are characterized by marked restriction, and small lungs invariably are seen in idiopathic pulmonary fibrosis and related disorders. 3. 6.9 Sarcoidosis. HIGH-RESOLUTION COMPUTED TOMOGRAPHY FEATURES OF INTERSTITIAL LUNG DISEASE. 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. Irregular linear opacities (Fig. In this review we present the key findings in the most common interstitial lung diseases. 6.5 Miliary tuberculosis. Nonspecific interstitial pneumonia (NSIP): CT appearance. Ground-glass attenuation is a term used almost exclusively with CT. Development of high-resolution computed tomography (HRCT) has resulted in markedly improved accuracy in diagnosing interstitial lung disease. The reticular pattern consists of a network of linear densities (Fig. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. Acute Kerley lines are frequently associated with prominent interlobar fissures caused by subpleural edema. Interstitial Lung Disease (ILD) Interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs. Notice the dilated bronchus in the right upper lobe, Chronic Obstructive Pulmonary Disease and Asthma, Thoracic Radiology: Imaging Methods, Radiographic Signs, and Diagnosis of Chest Disease. Thick-walled cysts are seen in the both lung bases. Ill-defined nodules up to 1 cm in diameter are identified in both lungs. Often less evident in apices and 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). 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. Extrinsic allergic alveolitis (e.g., farmer’s lung, bird-fancier’s lung, mushroom-worker’s lung, bagassosis, and others) (Figs. 6.16). 6.1e). In the lower lobes, there are multiple, small, centrilobular ground-glass nodules. Honeycombing is produced pathologically by the dissolution of alveolar walls with the formation of randomly distributed airspaces that are lined by fibrous tissue. Notice the dilated bronchus in the right upper lobe (thin arrow) and the subpleural honeycomb cysts (thick arrow). The term interstitial lung diseases (ILD) comprises a diverse group of diseases that lead to inflammation and fibrosis of the alveoli, distal airways, and septal interstitium of the lungs. This opacification obliterates the pulmonary vasculature. Interstitial pulmonary fibrosis has many causes. Despite the large number, approximately 15 to 20 constitute 90% of such disease states, and these are the entities that are discussed in this chapter. f Bronchiectases evident as cystic lesions varying considerably in size and characteristically containing small air-fluid levels (arrows). Multiple poorly defined nodules are seen bilaterally. This usually extends from the centrilobular vessel peripherally to join a thickened septum. Notice the dilated esophagus (e). A graphic or morphometric classification is a better approach and is enumerated in Box 7-3. Cylindrical bronchiectases present as tubular opacities with parallel walls of 1 mm or slightly larger thickness. Chapter 56: Interstitial Lung Diseases. 6.14 Farmer’s lung. 6.7). Smoking cessation often improves symptoms; the disease also … Large masses of fibrous tissue may occur, usually in the central or axial interstitium (Fig. Except for pulmonary vascular diseases, signs of pulmonary arterial hypertension are rarely identified. Peripheral septal lines lie perpendicular to the pleural surface (open arrow). Finally, fibrotic scars may be the sequelae of virtually any disease capable of damaging the lung parenchyma severely enough. bronchiolitis–associated interstitial lung disease (RB-ILD); desquama-tive interstitial pneumonia (DIP); lymphoid interstitial pneumonia (LIP); and acute interstitial pneumonia (AIP). Truly localized interstitial lung disease is relatively rare and most often of an infectious etiology. Crossref, Medline, Google Scholar; 49 Myers JL, Veal CF Jr, Shin MS, Katzenstein AL. They may be located in the axial interstitium along the peribronchovascular bundles, in the interlobular septa in a subpleural location adjacent to fissures, and in the central portion of the secondary pulmonary lobule. Poorly defined, confluent nodules are seen bilaterally, but only shown for the right side. Lymphatic obstruction appears to be a major factor in the development of Kerley lines associated with malignancies (e.g., lymphangitic carcinomatosis, bronchogenic carcinoma, and lymphoma), since at least histologically, ipsilateral hilar involvement with tumor is almost invariably present under such conditions. Miliary and larger (up to 3 cm). Miliary and slightly larger (up to 5 mm). In bronchiectasis, the bronchi show evidence of bronchial wall thickening, but they also are dilated and larger than adjacent pulmonary artery branches. Both congenital and acquired bronchiectases can be mistaken radiographically for localized interstitial lung disease (Fig. Interstitial lung disease (ILD) refers to a broad category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis of the lungs. 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). Fig. 6.1e). Only the right lower lung field is shown. 6.1d) is caused by a hazy increase in lung density that is not associated with obscuration of underlying vascular markings. f Bronchiectases evident as cystic lesions varying considerably in size and characteristically containing small air-fluid levels (arrows). Multiple nodules ranging from a few millimeters up to 10 cm. Interstitial lung disease (ILD) consists of a large and heterogeneous group of rare pulmonary disorders, characterized by abnormalities involving the alveoli and airway. 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. Interstitial nodules in sarcoidosis. Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic significance. These disorders are grouped together because of similarities in their clinical presentations, plain chest radiographic appearance, and… Honeycombing typically is in the peripheral portions of the lungs subpleurally, particularly in idiopathic pulmonary fibrosis. This results in the appearance of large ring shadows. Multiple, small (1 to 3 mm) nodules are distributed diffusely throughout the lungs. Traction bronchiectasis (Fig. They have been called parenchymal bands and long lines. However, histiocytosis X and sarcoidosis in the early stages are usually associated with normal lung volumes, but lymphangioleiomyomatosis produces air trapping with large lung volumes. 6.19). 6.4 Metastases from breast carcinoma. Bronchiectases are often associated with loss of volume and crowding of the lung markings in the affected area together with compensatory overinflation of the spared lung (Fig. Keywords Diffuse parenchymal lung disease, Interstitial lung disease, Lung disease, Lung transplantation, Parenchymal lung disease, Pulmonary Langerhans cell histiocytosis Key Points • For selected parenchymal lung disease (DPLD) patients who fail to respond to medical therapy and demonstrate declines in function that place them at increased risk for mortality, lung transplantation … Kerley lines refer to septal lines that are thickened either by fluid accumulation, cellular infiltration, or connective tissue proliferation within the interlobular septa. Reduced lung volumes may result in a restrictive pattern identified on pulmonary function tests. Symmetrical, miliary nodulation, preferentially located in the lower-lung fields. Thin-slices multi-detector computed tomography (MDCT) plays a key role in the differential diagnosis of interstitial lung disease (ILD). The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. 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. 6.11 Berylliosis. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. Miliary and larger, usually poorly defined (acute stage). Multiple tiny nodules of high density are distributed evenly throughout both lungs, sparing only the apices. Varicose and cystic (saccular) bronchiectases are often evident on plain radiography as cystic lesions up to 2 cm in diameter and often containing a small air-fluid level at the bottom. Finely granular and relatively dense stippling preferentially located in the posterior (dependent) parts of the lungs and most obvious a few hours after lymphography. Adenopathy (Box 7-6), which is recognized on standard radiographs, is associated with silicosis and sarcoidosis, lymphangitic carcinomatosis, and lymphoma. Figure 7-5 Parenchymal consolidation (i.e., airspace or alveolar disease). It is often accompanied by other signs of interstitial lung disease, especially the patterns associated with reticular opacities and architectural distortion. Diseases have zonal preferences in the lungs (Box 7-4), although severe diseases often become diffuse. Predominantly in the mid- and lower-lung fields. However, as many of these pathologies involve beyond or do not involve the interstitium at all, ILD in children and infants (chILD) is often considered a syndrome of diffuse ILD. This appearance must be differentiated from a primary airway problem, bronchiectasis. There is also thickening of the axial interstitium along the bronchi. Kerley B lines are thinner and shorter than Kerley A lines (up to 2 cm) and lie in the lung periphery perpendicular to the lateral pleural surface (Fig. Patients usually present (Box 7-2) with dyspnea as the predominant symptom. Lymphatic obstruction appears to be a major factor in the development of Kerley lines associated with malignancies (e.g., lymphangitic carcinomatosis, bronchogenic carcinoma, and lymphoma), since at least histologically, ipsilateral hilar involvement with tumor is almost invariably present under such conditions. A nodular pattern (Fig. Read "High-resolution CT of diffuse interstitial lung disease: key findings in common disorders, European Radiology" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. congestive heart failure). Parenchymal consolidation, which has been referred to as alveolar or airspace disease, is characterized by dense opacification often with air bronchograms (Fig. Thickening of the interlobular septa (Fig. 6.1a–f Patterns of interstitial lung disease. The patterns are described as nodular, irregular or linear, cystic, ground-glass, and parenchymal consolidation. 6.4). Some are primarily lung disorders, and some others are manifestations of diseases arising elsewhere. Miliary and larger. 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. They may be associated with more confluent areas of airspace consolidation with air bronchograms. 6.1b). 6.1f). 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. Findings simulate sarcoidosis radiographically. The characteristic com-puted tomographic findings in UIP are predominantly basal and pe-ripheral reticular opacities with honeycombing and traction bronchiec-tasis. When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). In the more central portion of the lung, the thickened septa can outline the secondary pulmonary lobules, producing polygonal structures that are 1 to 2.5 cm in diameter. Numerous relatively poorly defined miliary nodules and bilateral hilar enlargement are present, but are only shown for the right side. Innumerable, extremely sharply defined, tiny densities measuring less than 1 mm in diameter are seen bilaterally. Gallium scanning and positron emission tomography (PET) have also been used in the evaluation of interstitial lung disease and are discussed in more detail in, A graphic or morphometric classification is a better approach and is enumerated in Box 7-3. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. This appearance is typical for silicosis and for coal worker’s pneumoconiosis, but it may also occur in end-stage sarcoidosis. Thin-walled cysts are seen in the lungs of a patient with lymphangioleiomyomatosis. However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. They typically produce traction bronchiectasis centrally in the bronchi that they encompass. Although these disorders have frequently been referred to as interstitial lung diseases, many also involve the alveolar spaces. In approximately 50% of cases, they are limited to one lung. 6 Interstitial Lung Disease Some types of autoimmune diseases, such as rheumatoid … Honeycomb spaces usually are 1 cm or less in diameter with relatively thick walls (>2 mm), and they are a pathologic correlate of end-stage lung disease with fibrosis (Fig. The lung interstitium is that part of the lung that does not include the airspaces, the capillary endothelial cells, and the alveolar lining epithelium. 6.1a). The term interstitial lung disease (ILD) (also called diffuse interstitial lung disease, fibrotic interstitial lung disease, pulmonary fibrosis, and pneumoconiosis) refers to a broad group of inflammatory lung disorders. Central septal lines outline the secondary pulmonary lobule, which appears as a polyhedral structure (thin arrow). Kerley A lines are usually best seen in the mid and lower lung fields. Poorly defined nodular densities are seen bilaterally, but are only shown for the right lower lung field. Fig. The standard chest radiograph remains the basic and, in some cases, the only imaging technique that is useful. Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. Interstitial lung disease (ILD) describes a heterogeneous group of over 200 diseases affecting the pulmonary interstitium with varying degrees of involvement of the pleural space, airways, and pulmonary vasculature. Discrete and extremely sharply defined, less than 1 mm in diameter. 6.1b). Miliary and larger, often poorly defined; can form microabscesses. A nodular pattern (Fig. (C) … Fig. * Pattern that is predominant or usually associated with a specific disorder. When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). The six most common causes of diffuse interstitial pulmonary fibrosis are idiopathic (IPF, >50% of cases), collagen vascular disease, cytotoxic agents and nitrofurantoin, pneumoconioses, radiation, and sarcoidosis. a Reticular pattern (Pneumocystis carinii pneumonia). In a given patient, they are relatively uniform in size and usually bunched together in grape-like clusters. They are located in radiating fashion midway between the hilum and pleura and appear to cross over bronchoarterial bundles showing no anatomic relationship with the latter. 6.2 Bronchiectases. 6.1d) is caused by a hazy increase in lung density that is not associated with obscuration of underlying vascular markings. ILD is also termed diffuse parenchymal lung disease (DPLD). The chest radiograph, however, is often nonspecific. Gallium scanning and positron emission tomography (PET) have also been used in the evaluation of interstitial lung disease and are discussed in more detail in Nuclear Medicine: The Requisites. d Ground-glass appearance produced by the summation of innumerable tiny retlculogranular densities (sarcoidosis). Fig. This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic … 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). Honeycombing is the only dependable radiographic sign of interstitial fibrosis. Diffuse bilateral poorly defined small nodular densities are present, but only shown for the right lower lung field. The process of evaluating a surgical lung biopsy for disease involves answering several questions. They have been called, Septal thickening in lymphangitic carcinomatosis. Bilateral small nodules with preferential involvement of the mid and lower lung zones are seen in this arc welder that are not associated with hilar adenopathy or fibrosis and resolved after exposure was discontinued. Fig. 7-7) is common in many interstitial lung diseases. They are usually associated with architectural distortion and volume loss. Acute or transient Kerley lines are usually found with hydrostatic pulmonary edema (elevated microvascular pressure caused by left ventricular failure, renal disease and fluid overload), and occasionally with pneumonia and pulmonary hemorrhage. Septal lines are a common feature of many interstitial lung disorders but are particularly predominant in lymphangitic spread of carcinoma and in congestive heart failure. Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium (the tissue and space around the alveoli (air sacs) of the lungs. Many pulmonary diseases produce pathologic changes in both compartments, and disease processes that are pathologically classified as interstitial may produce an alveolar pattern on the radiograph. (e.g., carcinomas from thyroid, lung, breast or gastrointestinal tract, or melanomas, sarcomas and lymphomas) (Fig. Amyloidosis (diffuse alveolar septal form), Talc granulomatosis secondary to intravenous drug abuse. Bronchial wall thickening (white arrow) and small nodules are seen subpleurally along the fissures and lateral chest wall. Confluent, diffuse consolidation and air bronchograms in both upper lobes can be seen in this example of an alveolar pattern in a patient with interstitial lung disease (i.e., sarcoidosis). Diseases have zonal preferences in the lungs (Box 7-4), although severe diseases often become diffuse. Honeycombing represents an end-stage lung that is destroyed by fibrosis. Fig. Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. Parenchymal consolidation (i.e., airspace or alveolar disease). 2 to 4 mm, discrete. Fig. 1,2 This category includes various illnesses affecting the lung parenchyma with many different causes, treatments, and prognoses. 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). b Kerley A lines (long arrows, touched up) and Kerley B lines (short arrows) (mitral stenosis). Hemosiderosis-like pulmonary calcifications are occasionally seen in chronic renal failure (see Fig. Prominence of the central dot (Fig. It usually involves the more central bronchi. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. There is ground-glass opacification and more confluent consolidation in the right lung. Miliary, discrete (DD: tuberculomas that are larger than 5 mm and can calcify). Fig. 7.29). a Reticular pattern (Pneumocystis carinii pneumonia). ILDs may occur in isolation or in association with systemic diseases. Figure 7-12 Masses of fibrosis in end-stage sarcoidosis. There are two types of cystic patterns: thin-walled cysts (Fig. Note also the lytic involvement of the left fifth rib with pathologic fracture (arrow). Click to share on Twitter (Opens in new window), Click to share on Facebook (Opens in new window), Click to share on Google+ (Opens in new window), Table 6.1 Disseminated Pulmonary Nodules Measuring Less than 1 cm in Diameter, Pulmonary Edema and Symmetrical Bilateral Infiltrates. Reticular opacities usually are caused by interstitial thickening by cells, fluid, or fibrous tissue (Box 7-8). Physiologically, the abnormalities primarily affect gas exchange and result in hypoxemia. Fig. Irregular linear opacities (. Radiology Key; Thoracic Key; Veterinary Medicine; Gold Membership; Contact; Menu. Fairly well defined in silicosis and poorly in berylliosis. 7-10) tend to be well defined and can be seen in numerous interstitial lung diseases. Pattern recognition in diffuse interstitial lung disease has been the subject of controversy for many years. A ground-glass appearance (Fig. Fig. 7-15, Click to share on Twitter (Opens in new window), Click to share on Facebook (Opens in new window), Click to share on Google+ (Opens in new window), Many chronic diseases can produce diffuse opacities in the lung. Multiple small nodules are scattered throughout both lung fields. The patterns are described as nodular, irregular or linear, cystic, ground-glass, and parenchymal consolidation. Oily contrast material embolism (e.g., secondary to lymphography) (Fig. Finally, fibrotic scars may be the sequelae of virtually any disease capable of damaging the lung parenchyma severely enough. The majority of interstitial lung diseases involve both lungs, or stated differently, the interstitial disease is usually diffuse, although some areas may be more affected and others more or less spared. Centrilobular abnormalities can also be seen in patients with diseases of the peripheral airways (i.e., bronchioles). Most patterns can be readily identified on standard radiographs, but ground-glass and cystic disease patterns are much more readily appreciated on HRCT. Thick-walled cystic spaces can be seen subpleurally in the bases. ■ Discuss the importance of temporal division of chILDinto infant and later childhood onset and of a sound understanding of pulmonary organogenesis and surfactant homeostasis in aiding radiologic diagnosis. Langerhans cell histiocytosis (eosinophilicgranuloma) (Fig. Miliary and larger, often indistinctly defined. 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. Clinical and radiologic correlation can aid the pathologist and refine the diagnosis. These structures typically have a central dot that represents the pulmonary artery. Patients invariably present with dyspnea of varying time course and severity. For example, the adult ILD … pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. 6.2). 6.8 Varicella (chickenpox) pneumonia. 6.12 and 6.13). (B) Axial CT in another patient with NSIP caused by nitrofurantoin at the level of the main bronchi shows bilateral ground-glass opacities and reticulation mainly in the peripheral lung. Pattern that is predominant or usually associated with a specific disorder. The following are the main technical components: 0.625- to 1.25-mm-thick sections, use of a high-resolution algorithm, targeted reconstruction to a single lung (optional), and prone scans to evaluate early or minimal basal disease. Bilateral poorly defined nodules are present. 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. Localized fibrotic changes are often found in the chronic stage of a disease (e. g., tuberculosis and radiation pneumonitis).

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