This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). Kerley lines refer to septal lines that are thickened either by fluid accumulation, cellular infiltration, or connective tissue proliferation within the interlobular septa. 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. 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. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. Diffuse bilateral poorly defined small nodular densities are present, but only shown for the right lower lung field. 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. INTRODUCTION This chapter presents the background and special considerations for assessing and developing exercise prescriptions for individuals with asthma and interstitial lung disease (ILD). congestive heart failure). Poorly defined, confluent nodules are seen bilaterally, but only shown for the right side. There are two types of cystic patterns: thin-walled cysts (Fig. 6.9 Sarcoidosis. Web Chapter 56. This usually extends from the centrilobular vessel peripherally to join a thickened septum. 3. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. They have been called, Septal thickening in lymphangitic carcinomatosis. 6.2). a Reticular pattern (Pneumocystis carinii pneumonia). The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. A graphic or morphometric classification is a better approach and is enumerated in Box 7-3. Healing may result in punctate calcifications years later. HIGH-RESOLUTION COMPUTED TOMOGRAPHY FEATURES OF INTERSTITIAL LUNG DISEASE. Pattern that is predominant or usually associated with a specific disorder. Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. Fig. Linear opacities of nonspecific interstitial pneumonia. Parenchymal consolidation, which has been referred to as alveolar or airspace disease, is characterized by dense opacification often with air bronchograms (Fig. After completing this journal-based SA-CME activity, participants will be able to: 1. A subpleural line may be defined as a curvilinear opacity that is less than 1 cm from the pleural surface. 6.1a–f Patterns of interstitial lung disease. Most patterns can be readily identified on standard radiographs, but ground-glass and cystic disease patterns are much more readily appreciated on HRCT. f Bronchiectases evident as cystic lesions varying considerably in size and characteristically containing small air-fluid levels (arrows). 6.1b). More often, however, nodular and reticular patterns are combined in the same patient, resulting in a reticulonodular appearance of the interstitial disease. Fig. When tissue is obtained, the lung parenchyma may contain any combination of abnormalities, including inflammation, fibrosis, and granulomas. a Reticular pattern (Pneumocystis carinii pneumonia). Fig. Often poorly defined, confluent nodules of varying size. 7-8) within the secondary pulmonary lobule (i.e., centrilobular vessel) may occur in a number of interstitial lung diseases. pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. 6.12 and 6.13). Discrete and extremely sharply defined, less than 1 mm in diameter. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. 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. Peripheral septal lines lie perpendicular to the pleural surface (open arrow). Poorly defined nodular densities are seen bilaterally, but are only shown for the right lower lung field. Some investigators have attempted to differentiate interstitial from airspace or acinar nodules on HRCT. This appearance is typical for silicosis and for coal worker’s pneumoconiosis, but it may also occur in end-stage sarcoidosis. Permanent Kerley lines are most often present in chronic and severe pulmonary venous hypertension (especially mitral stenosis) that eventually results in fibrosis and hemosiderin deposition within the interlobular septa. The ILD consist of disorders of known cause (e.g., collagen vascular diseases, drug-related diseases) as well as … Numerous nodules measuring only a few millimeters in diameter are present bilaterally, but are only shown for the right lower lung field. 6.13 Stannosis (inhalation of tin oxide). Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. Intrauterine infection with high mortality rate. Traction bronchiectasis (Fig. Thick-walled cysts are seen in the both lung bases. Interpretation of lung biopsy specimens is an integral part in the diagnosis of interstitial lung disease. Kerley A lines are usually best seen in the mid and lower lung fields. 6.14 Farmer’s lung. Findings related to emphysema and small airways disease (e.g., bronchiolitis, which may cause decreased lung opacity) are discussed in Chapters 10 and 13. bronchiolitis–associated interstitial lung disease (RB-ILD); desquama-tive interstitial pneumonia (DIP); lymphoid interstitial pneumonia (LIP); and acute interstitial pneumonia (AIP). There is ground-glass opacification and more confluent consolidation in the right lung. a Punctate densities (hemosiderosis), and b larger calcified nodules (pulmonary ossification) are seen bilaterally, but only shown for the right side. Only the right lower lung field is shown. The reticular pattern consists of a network of linear densities (Fig. Multiple, small (1 to 3 mm) nodules are distributed diffusely throughout the lungs. A central dot in the lobule is prominent, and the intralobular bronchiole is visible (arrowhead). Fibrosis due to collagen vascular disease, Some lymphangitic tumors (Kaposi’s sarcoma), Chronic interstitial pneumonias, idiopathic pulmonary fibrosis. Some are primarily lung disorders, and some others are manifestations of diseases arising elsewhere. There is also thickening of the axial interstitium along the bronchi (arrow) and nodules along the fissures (thick arrows). Brendan M. Corcoran, Edinburgh, Scotland. Interstitial lung diseases (ILDs) encompass a wide range of diffuse pulmonary disorders, characterized by a variable degree of inflammatory and fibrotic changes of the alveolar wall and eventually the distal bronchiolar airspaces. Honeycombing is the only dependable radiographic sign of interstitial fibrosis. Acute Kerley lines are frequently associated with prominent interlobar fissures caused by subpleural edema. Fig. Irregular linear opacities (. These structures typically have a central dot that represents the pulmonary artery. Extensive, predominantly cystic bronchiectases in the right lung and left lower lobe are associated with loss of volume in the affected lung and compensatory overinflation of the nonaffected left upper lobe. Smoking cessation often improves symptoms; the disease also … In patients with isolated bronchiectasis, there are no other signs of lung disease. 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. 6.17). 6.1a–f Patterns of interstitial lung disease. 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). 6.2 Bronchiectases. Development of high-resolution computed tomography (HRCT) has resulted in markedly improved accuracy in diagnosing interstitial lung disease. b Kerley A lines (long arrows, touched up) and Kerley B lines (short arrows) (mitral stenosis). Development of high-resolution computed tomography (HRCT) has resulted in markedly improved accuracy in diagnosing interstitial lung disease. 6.4). This results in the appearance of large ring shadows. Thin-walled cysts are seen in the lungs of a patient with lymphangioleiomyomatosis. 6.17 Mitral stenosis (2 cases). Figure 7-3 Thin-walled cysts are seen in the lungs of a patient with lymphangioleiomyomatosis. First described in asbestosis, a subpleural line occasionally is seen in normal lungs and results from dependent atelectasis. Pleural disease may take one of several forms (. 6.5 Miliary tuberculosis. Honeycombing in idiopathic pulmonary fibrosis. Figure 7-4 Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. However, the clinical course of ILD in myositis is to a large extent unknown, and a high variability in the clinical course, response to treatment, and prognosis is suggested from previous reports ( 3 , 4 ). Reduced lung volumes may result in a restrictive pattern identified on pulmonary function tests. Central septal lines outline the secondary pulmonary lobule, which appears as a polyhedral structure (thin arrow). Interstitial lung disease is diagnosed radiographically when a reticular, nodular, or honeycomb pattern or any combination thereof is recognizable. Interstitial nodules (Fig. The process of evaluating a surgical lung biopsy for disease involves answering several questions. Miliary, discrete (DD: tuberculomas that are larger than 5 mm and can calcify). Publicationdate 2007-12-20. 7-11). Notice the subpleural sparing at the fissures. Pulmonary arterial hypertension usually indicates end-stage disease with pronounced obliteration of the pulmonary vasculature. 6.15 Bird-fancier’s lung. Notice the subpleural sparing at the fissures. Findings related to emphysema and small airways disease (e.g., bronchiolitis, which may cause decreased lung opacity) are discussed in Chapters 10 and. Figure 7-9 Intralobular interstitial thickening (i.e., reticular opacities) in pulmonary alveolar proteinosis. Both congenital and acquired bronchiectases can be mistaken radiographically for localized interstitial lung disease (Fig. There are numerous interstitial lung diseases, but in clinical practice only about ten diseases account for approximately 90% of cases. 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. Finally, fibrotic scars may be the sequelae of virtually any disease capable of damaging the lung parenchyma severely enough. Honeycombing represents an end-stage lung that is destroyed by fibrosis. Traction bronchiectasis of usual interstitial pneumonitis in scleroderma. Interstitial lung disease (ILD) refers to a broad category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis of the lungs. Masses of Fibrosis or Conglomerate Masses, Masses of fibrosis in end-stage sarcoidosis. Thin-slices multi-detector computed tomography (MDCT) plays a key role in the differential diagnosis of interstitial lung disease (ILD). 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. 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. In a given patient, they are relatively uniform in size and usually bunched together in grape-like clusters. Innumerable, extremely sharply defined, tiny densities measuring less than 1 mm in diameter are seen bilaterally. 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. Usually well defined and of varying size. Physical examination frequently finds only dry rales or crackles. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. Ill-defined nodules up to 1 cm in diameter are identified in both lungs. 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. 6.8). 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. 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). Interstitial lung diseases (ILDs) are a heterogeneous group with diffuse parenchymal lung disease. Bilateral miliary (a) and larger (b) scattered calcified nodules are present, but only shown for the right side. 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 fibrotic disorders are characterized by marked restriction, and small lungs invariably are seen in idiopathic pulmonary fibrosis and related disorders. Permanent Kerley lines are most often present in chronic and severe pulmonary venous hypertension (especially mitral stenosis) that eventually results in fibrosis and hemosiderin deposition within the interlobular septa. 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. 6 Interstitial Lung Disease Fig. 7-6) occurs in many diseases, such as lymphangitic spread of carcinoma, pulmonary fibrosis, and sarcoidosis. Fig. 6.10 and 6.11). A nodular pattern (Fig. Abstract: The idiopathic interstitial pneumonias (IIPs) are the most commonly diagnosed forms of interstitial lung disease. 6.1d) is caused by a hazy increase in lung density that is not associated with obscuration of underlying vascular markings. 6.6 Histoplasmosis (2 cases). 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. Honeycombing is produced pathologically by the dissolution of alveolar walls with the formation of randomly distributed airspaces that are lined by fibrous tissue. 6.2). 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). Intralobular interstitial thickening (i.e., reticular opacities) in pulmonary alveolar proteinosis. Multiple small nodules are scattered throughout both lung fields. ■ 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. 7-7) is common in many interstitial lung diseases. Idiopathic pulmonary fibrosis (usual interstitial pneumonitis). Interstitial (in-tur-STISH-ul) lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. Hemosiderosis and pulmonary ossification secondary to mitral stenosis (Fig. 6.1a). Nodular pattern of miliary tuberculosis. 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. Pleural plaques, an uncommon feature, are produced almost exclusively by asbestos exposure. They typically produce traction bronchiectasis centrally in the bronchi that they encompass. Pneumoconiosis (inorganic dust) (e.g., silicosis, coal miner’s lung, berylliosis) (Figs. 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. 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