Bronchial wall thickening (white arrow) and small nodules are seen subpleurally along the fissures and lateral chest wall. Fig. Chest Radiology > Pathology > Interstitial Disease. 6.1b). Dr. Sally Suliman presents "Interstitial Lung Disease" by first introducing the classification of ILDs. There is also thickening of the axial interstitium along the bronchi (arrow) and nodules along the fissures (thick arrows). Parenchymal consolidation, which has been referred to as alveolar or airspace disease, is characterized by dense opacification often with air bronchograms (Fig. 7-7) is common in many interstitial lung diseases. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. Interstitial lung disease is diagnosed radiographically when a reticular, nodular, or honeycomb pattern or any combination thereof is recognizable. 6.4). 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. Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic significance. Thick-walled cysts are seen in the both lung bases. For example, histiocytosis, sarcoidosis, silicosis, and coal worker’s pneumoconiosis typically favor the upper lobes, whereas idiopathic pulmonary fibrosis and fibrosis associated with collagen vascular disease tend to be a lower-zone phenomenon. Figure 7-2 Linear opacities of nonspecific interstitial pneumonia. Notice the dilated esophagus (e). They are most numerous at the base of the lungs. 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. 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. Table 6.3 summarizes all disorders that demonstrate radiographically a diffuse reticular or reticulonodular pattern characteristic of interstitial lung disease. Radiology: Volume 270: Number 2—February 2014 n radiology.rsna.org 583 rheumatoid arthritis–associated interstitial lung Disease: Radiologic Identification of Usual Interstitial Pneumonia Pattern1 Deborah Assayag, MD Brett M. Elicker, MD Thomas H. Urbania, MD Thomas V. Colby, MD Bo Hyoung Kang, MD Jay H. Ryu, MD Talmadge E. King, MD Harold R. Collard, MD Dong Soon Kim, MD … 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. Interstitial (in-tur-STISH-ul) lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. They typically produce traction bronchiectasis centrally in the bronchi that they encompass. ILDs may occur in isolation or in association with systemic diseases. When tissue is obtained, the lung parenchyma may contain any combination of abnormalities, including inflammation, fibrosis, and granulomas. 6.1e). 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. Symmetrical, miliary nodulation, preferentially located in the lower-lung fields. Pneumoconiosis caused by radiopaque dusts (iron, tin, barium, antimony and rare-earth compounds) (Figs. Exclusively these symptomatic cases are designated as respiratory bronchiolitis-interstitial lung disease. These disorders are grouped together because of similarities in their clinical presentations, plain chest radiographic appearance, and… Centrilobular abnormalities can also be seen in patients with diseases of the peripheral airways (i.e., bronchioles). Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. Irregular linear opacities (. Coccidioidomycosis, blastomycosis, and Cryptococcosis (Fig. Nonspecific interstitial pneumonia (NSIP): CT appearance. Truly localized interstitial lung disease is relatively rare and most often of an infectious etiology. Varicella (chickenpox) pneumonia (Fig. 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. There are numerous interstitial lung diseases, but in clinical practice only about ten diseases account for approximately 90% of cases. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. They are most numerous at the base of the lungs. 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. Hemosiderosis and pulmonary ossification secondary to mitral stenosis (Fig. There is also thickening of the axial interstitium along the bronchi. Small nodules up to 4 mm in diameter are identified in both lungs. Underlying chILD pathologies are markedly different from adult ILD. Roentgenographic pattern of disseminated alveolar cell carcinoma may be fairly uniform throughout both lungs or vary regionally. Pulmonary arterial hypertension usually indicates end-stage disease with pronounced obliteration of the pulmonary vasculature. c Nodular pattern (silicosis). Figure 7-4 Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. Fibrosis due to collagen vascular disease, Some lymphangitic tumors (Kaposi’s sarcoma), Chronic interstitial pneumonias, idiopathic pulmonary fibrosis. 7-6) occurs in many diseases, such as lymphangitic spread of carcinoma, pulmonary fibrosis, and sarcoidosis. In t… Brendan M. Corcoran, Edinburgh, Scotland. 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. A subpleural line may be defined as a curvilinear opacity that is less than 1 cm from the pleural surface. Prominence of the central dot (Fig. 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). The chest radiograph, however, is often nonspecific. Some are primarily lung disorders, and some others are manifestations of diseases arising elsewhere. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. congestive heart failure). This appearance is virtually diagnostic, although under very rare circumstances both pulmonary papillomatosis and paragonimiasis may mimic cystic bronchiectases. d Ground-glass appearance produced by the summation of innumerable tiny retlculogranular densities (sarcoidosis). Fig. Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. 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. 6.3 Bronchioloalveolar carcinoma. A central dot in the lobule is prominent, and the intralobular bronchiole is visible (arrowhead). 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. However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. Although these disorders have frequently been referred to as interstitial lung diseases, many also involve the alveolar spaces. The thickening may be smooth or nodular. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. 6.8). 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. Similarly, pleural effusions and diffuse thickening are often associated with collagen vascular disease and asbestos exposure. 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. Miliary and larger with mid and upper lung fields predominance. Fig. (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. 6.18 Alveolar microlithiasis. Hemosiderosis-like pulmonary calcifications are occasionally seen in chronic renal failure (see Fig. 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. Pleural plaques, an uncommon feature, are produced almost exclusively by asbestos exposure. 7-10) tend to be well defined and can be seen in numerous interstitial lung diseases. Kerley lines refer to septal lines that are thickened either by fluid accumulation, cellular infiltration, or connective tissue proliferation within the interlobular septa. Often poorly defined, confluent nodules of varying size. Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic … When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). Box 7-8 High-Resolution Computed Tomography Findings for Linear Opacities, Thickening of bronchovascular bundles (axial), Interlobular septal thickening (septal lines). Fig. Pneumoconioses and vascular disorders are discussed in Chapters 8 and 9. Figure 7-5 Parenchymal consolidation (i.e., airspace or alveolar disease). Cystic abnormalities include honeycombing, traction bronchiectasis, lung cysts, and cavitary nodules. 6.1a). 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. Box 7-1 Diffuse Interstitial (Parenchymal) Lung Diseases, Allergic alveolitis (hypersensitivity pneumonitis), Respiratory bronchiolitis interstitial lung disease, Pneumonia resulting from neurofibromatosis, Histiocytosis X (Langerhans cell histiocytosis), Metastatic carcinoma, lymphangitic carcinomatosis, Hemosiderosis, chronic passive congestion, Multiple emboli and idiopathic pulmonary hypertension. 7-11). Kerley lines refer to septal lines that are thickened either by fluid accumulation, cellular infiltration, or connective tissue proliferation within the interlobular septa. Fig. Clinical and radiologic correlation can aid the pathologist and refine the diagnosis. Abstract: The idiopathic interstitial pneumonias (IIPs) are the most commonly diagnosed forms of interstitial lung disease. 6.10 Silicosis. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. Discrete and extremely sharply defined, less than 1 mm in diameter. Notice the dilated esophagus (e). Fig. 6.1a). Diseases have zonal preferences in the lungs (Box 7-4), although severe diseases often become diffuse. 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. Kerley A lines are usually best seen in the mid and lower lung fields. Commonly, interstitial lung disease (ILD) presents with dyspnea on exertion, diffuse bilateral infiltrates on chest imaging, and restriction with diffusion impairment on physiologic testing. The patterns are described as nodular, irregular or linear, cystic, ground-glass, and parenchymal consolidation. Webb and colleagues describe such HRCT findings in interstitial lung disease further in their work (see Suggested Readings). Honeycombing represents an end-stage lung that is destroyed by fibrosis. 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. The fibrotic disorders are characterized by marked restriction, and small lungs invariably are seen in idiopathic pulmonary fibrosis and related disorders. The reticular pattern consists of a network of linear densities (Fig. Fig. 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. This results in the appearance of large ring shadows. Patients invariably present with dyspnea of varying time course and severity. Usually well defined and of varying size. They may be associated with more confluent areas of airspace consolidation with air bronchograms. a Punctate densities (hemosiderosis), and b larger calcified nodules (pulmonary ossification) are seen bilaterally, but only shown for the right side. In a given patient, they are relatively uniform in size and usually bunched together in grape-like clusters. Fig. Langerhans cell histiocytosis (eosinophilicgranuloma) (Fig. Because the anatomy of the secondary pulmonary lobule can be readily observed on HRCT, this distinction often may be possible, even though overlap in the appearance of interstitial and alveolar nodules occurs and many disease processes involve both compartments. Intralobular interstitial thickening (i.e., reticular opacities) in pulmonary alveolar proteinosis. ILD is also termed diffuse parenchymal lung disease (DPLD). The ILD consist of disorders of known cause (e.g., collagen vascular diseases, drug-related diseases) as well as … Multiple small nodules are scattered throughout both lung fields. 1,2 This category includes various illnesses affecting the lung parenchyma with many different causes, treatments, and prognoses. 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