When the chest radiograph shows a clear pattern of ILD or ALD, one can render a differential diagnosis on the basis of the pattern of parenchymal disease (Table 3.1). Only the right lower lung field is shown. 6.10 Silicosis. Cystic bronchiectases may produce a radiographic picture similar to honeycombing. Both congenital and acquired bronchiectases can be mistaken radiographically for localized interstitial lung disease (Fig. 6.17 Mitral stenosis (2 cases). In this review we present the key findings in the most common interstitial lung diseases. Notice the dilated esophagus (e). 6.12 and 6.13). A honeycomb pattern is characterized by round or oval cystic lesions with a diameter up to 1 cm (Fig. 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. Linear opacities of nonspecific interstitial pneumonia. 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. In a given patient, they are relatively uniform in size and usually bunched together in grape-like clusters. Miliary nodulation only manifest 2–5 weeks after initial exposure (third phase of disease). If left untreated, the inflammatory process can progress to irreversible pulmonary … 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. Thick-walled cysts are seen in the both lung bases. Both congenital and acquired bronchiectases can be mistaken radiographically for localized interstitial lung disease (Fig. 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. Central septal lines outline the secondary pulmonary lobule, which appears as a polyhedral structure (thin arrow). Figure 7-10 Interstitial nodules in sarcoidosis. However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. Localized fibrotic changes are often found in the chronic stage of a disease (e. g., tuberculosis and radiation pneumonitis). 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). After completing this journal-based SA-CME activity, participants will be able to: 1. Oily contrast material embolism (e.g., secondary to lymphography) (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. The standard chest radiograph remains the basic and, in some cases, the only imaging technique that is useful. 6.1b). Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. Finely granular stippling uniformly distributed over both lung fields. Interstitial nodules in sarcoidosis. 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. Poorly defined, confluent nodules are seen bilaterally, but only shown for the right side. 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 … Figure 7-7 Septal thickening in lymphangitic carcinomatosis. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream.Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Multiple, small (1 to 3 mm) nodules are distributed diffusely throughout the lungs. 2 to 4 mm, discrete. 6.1d) is caused by a hazy increase in lung density that is not associated with obscuration of underlying vascular markings. It is found, besides in interstitial diseases, also with air-space disease (e.g. Except for pulmonary vascular diseases, signs of pulmonary arterial hypertension are rarely identified. 6.1a–f Patterns of interstitial lung disease. pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. congestive heart failure). Multiple poorly defined nodules are seen bilaterally. 3. The nodular pattern (Fig. They are most numerous at the base of the lungs. The table summarizes the most common diseases, that present with consolidation. 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. There is also thickening of the axial interstitium along the bronchi (arrow) and nodules along the fissures (thick arrows). Some types of autoimmune diseases, such as rheumatoid … Fig. The thickening may be smooth or nodular. Pleural disease may take one of several forms (Box 7-5). Pneumothorax may be seen as a complication of any cause of end-stage lung, but it may be identified early in the course of diseases such as histiocytosis X and lymphangioleiomyomatosis, in which there is a high prevalence of pneumothorax. 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. 6.14 Farmer’s lung. 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. (C) … This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). Honeycombing typically is in the peripheral portions of the lungs subpleurally, particularly in idiopathic pulmonary fibrosis. 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. This opacification obliterates the pulmonary vasculature. Centrilobular nodules in respiratory bronchiolitis. Fig. 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. There is also thickening of the axial interstitium along the bronchi. Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic … It is manifested by bronchial wall thickening and apparent enlargement of central pulmonary vessels. The lung interstitium is that part of the lung that does not include the airspaces, the capillary endothelial cells, and the alveolar lining epithelium. 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. Hemosiderosis-like pulmonary calcifications are occasionally seen in chronic renal failure (see Fig. Fig. Poorly defined nodular densities are seen bilaterally, but are only shown for the right lower lung field. Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. a Punctate densities (hemosiderosis), and b larger calcified nodules (pulmonary ossification) are seen bilaterally, but only shown for the right side. * More sensitive in detection of adenopathy than radiography. Diseases have zonal preferences in the lungs (Box 7-4), although severe diseases often become diffuse. Notice the subpleural sparing at the fissures. Prominence of the central dot (Fig. Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has no obvious differential diagnostic significance. (e.g., carcinomas from thyroid, lung, breast or gastrointestinal tract, or melanomas, sarcomas and lymphomas) (Fig. Notice the dilated bronchus in the right upper lobe (thin arrow) and the subpleural honeycomb cysts (thick arrow). 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. 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. 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. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. The patterns are described as nodular, irregular or linear, cystic, ground-glass, and parenchymal consolidation. 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. Honeycombing represents an end-stage lung that is destroyed by fibrosis. 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. Abstract: The idiopathic interstitial pneumonias (IIPs) are the most commonly diagnosed forms of interstitial lung disease. Numerous discrete nodules measuring 1 to 3 mm in diameter are seen bilaterally, but are only shown for the right side. The fibrotic disorders are characterized by marked restriction, and small lungs invariably are seen in idiopathic pulmonary fibrosis and related disorders. 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. Fairly well defined in silicosis and poorly in berylliosis. 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). Intrauterine infection with high mortality rate. Webb and colleagues describe such HRCT findings in interstitial lung disease further in their work (see Suggested Readings). 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). b Kerley A lines (long arrows, touched up) and Kerley B lines (short arrows) (mitral stenosis). 6.18 Alveolar microlithiasis. Honeycombing is produced pathologically by the dissolution of alveolar walls with the formation of randomly distributed airspaces that are lined by fibrous tissue. Honeycombing is the only dependable radiographic sign of interstitial fibrosis. Irregular linear opacities (. Box 7-8 High-Resolution Computed Tomography Findings for Linear Opacities, Thickening of bronchovascular bundles (axial), Interlobular septal thickening (septal lines). 6.8 Varicella (chickenpox) pneumonia. Many diseases demonstrate more than one pattern (see Box 7-3). In approximately 50% of cases, they are limited to one lung. Patients are often hypoxemic, and pulmonary function tests typically demonstrate a restrictive pattern, with reduced diffusing capacity. Interpretation of lung biopsy specimens is an integral part in the diagnosis of interstitial lung disease. Figure 7-12 Masses of fibrosis in end-stage sarcoidosis. 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. The process of evaluating a surgical lung biopsy for disease involves answering several questions. 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. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. 6.6 Histoplasmosis (2 cases). 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). Brendan M. Corcoran, Edinburgh, Scotland. Fibrosis due to collagen vascular disease, Some lymphangitic tumors (Kaposi’s sarcoma), Chronic interstitial pneumonias, idiopathic pulmonary fibrosis. Ill-defined nodules up to 1 cm in diameter are identified in both lungs. Miliary and slightly larger (up to 5 mm). Large masses of fibrous tissue seen centrally in the upper lobes are associated with traction bronchiectasis (arrow). Although these disorders have frequently been referred to as, Diffuse Interstitial (Parenchymal) Lung Diseases, IMMUNOLOGIC AND CONNECTIVE TISSUE DISORDERS, The standard chest radiograph remains the basic and, in some cases, the only imaging technique that is useful. 6.17). Reticular opacities usually are caused by interstitial thickening by cells, fluid, or fibrous tissue (Box 7-8). On HRCT, the term lung cyst refers to a thin-walled (usually < 2 mm), well-defined and circumscribed, air-containing lesion that is 1 cm or more in diameter (Fig. There is ground-glass opacification and more confluent consolidation in the right lung. Patients invariably present with dyspnea of varying time course and severity. Kerley A lines are usually best seen in the mid and lower lung fields. Interstitial lung disease (ILD) refers to a broad category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis of the lungs. pneumocystis carinii pneumonia) and increased capillary blood volume (e.g. Some are primarily lung disorders, and some others are manifestations of diseases arising elsewhere. Clinical and radiologic correlation can aid the pathologist and refine the diagnosis. Physiologically, the abnormalities primarily affect gas exchange and result in hypoxemia. Table 6.3 summarizes all disorders that demonstrate radiographically a diffuse reticular or reticulonodular pattern characteristic of interstitial lung disease. Localized fibrotic changes are often found in the chronic stage of a disease (e. g., tuberculosis and radiation pneumonitis). Traction bronchiectasis (Fig. Langerhans cell histiocytosis (eosinophilicgranuloma) (Fig. These structures typically have a central dot that represents the pulmonary artery. This presentation is seen in the active stage, which may completely resolve or progress to the chronic fibrotic stage. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. The intralobular bronchiole often becomes visible when there is centrilobular thickening. 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. Adenopathy (Box 7-6), which is recognized on standard radiographs, is associated with silicosis and sarcoidosis, lymphangitic carcinomatosis, and lymphoma. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. Multiple nodules ranging from a few millimeters up to 10 cm. Fig. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. Fig. Varicella (chickenpox) pneumonia (Fig. Pulmonary arterial hypertension usually indicates end-stage disease with pronounced obliteration of the pulmonary vasculature. Kerley A lines are usually best seen in the mid and lower lung fields. Physical examination frequently finds only dry rales or crackles. Different kinds of Kerley lines are distinguished: Kerley A lines are straight lines measuring 2–6 cm in length and approximately 1 mm in thickness. ILDs may occur in isolation or in association with systemic diseases. 6.2 Bronchiectases. 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). The typical appearance of honeycombing is that of thick-walled cystic spaces that are usually less than 1 cm in diameter (Fig. Fig. Chest Radiology > Pathology > Interstitial Disease. Differential 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. In the lower lobes, there are multiple, small, centrilobular ground-glass nodules. Finely granular and relatively dense stippling preferentially located in the posterior (dependent) parts of the lungs and most obvious a few hours after lymphography. 6.2). 6.16 Langerhans cell histiocytosis (eosinophilic granuloma). Numerous fairly well defined miliary nodules are seen bilaterally besides diffuse reticular changes and early honeycombing, but are only shown for the right side. The size of the lung (i.e., lung volumes) may be a clue to the differential diagnosis (Box 7-7). d Ground-glass appearance produced by the summation of innumerable tiny retlculogranular densities (sarcoidosis). Irregular linear opacities (Fig. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. Density of the tiny nodules depends on the atomic number of the inhaled element. More than 180 disease entities are characterized by acute, subacute, or chronic inflammatory infiltration of alveolar walls by cells, fluid, and connective tissue. 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. 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. Thick-walled cysts are seen in the both lung bases. This appearance is typical for silicosis and for coal worker’s pneumoconiosis, but it may also occur in end-stage sarcoidosis. Some investigators have attempted to differentiate interstitial from airspace or acinar nodules on HRCT. Multiple small nodules are scattered throughout both lung fields. Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. 7-7) is common in many interstitial lung diseases. These diseases account for ∼15–20% of general pulmonary practice. Fig. 6.4). This appearance is virtually diagnostic, although under very rare circumstances both pulmonary papillomatosis and paragonimiasis may mimic cystic bronchiectases. Cylindrical bronchiectases present as tubular opacities with parallel walls of 1 mm or slightly larger thickness. Patients usually present (Box 7-2) with dyspnea as the predominant symptom. Findings simulate sarcoidosis radiographically. They are not truly acinar but may be considered airspace nodules (Fig. 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. It parallels the pleura and is a few millimeters thick (see Chapter 8). Can result in nodular calcifications 1 to several years later. 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. In the peripheral lung, it appears as 1- to 2-cm lines that extend perpendicularly from the pleural surface into the substance of the lung. Symmetrical, miliary nodulation, preferentially located in the lower-lung fields. 7-13). Diffuse bilateral poorly defined small nodular densities are present, but only shown for the right lower lung field. The nodular pattern (Fig. a Reticular pattern (Pneumocystis carinii pneumonia). 6.19). 7-12). Masses of Fibrosis or Conglomerate Masses, Masses of fibrosis in end-stage sarcoidosis. f Bronchiectases evident as cystic lesions varying considerably in size and characteristically containing small air-fluid levels (arrows). Interstitial nodules (Fig. Thin-walled cysts are seen in the lungs of a patient with lymphangioleiomyomatosis. Finally, fibrotic scars may be the sequelae of virtually any disease capable of damaging the lung parenchyma severely enough. congestive heart failure). Cylindrical bronchiectases present as tubular opacities with parallel walls of 1 mm or slightly larger thickness. HIGH-RESOLUTION COMPUTED TOMOGRAPHY FEATURES OF INTERSTITIAL LUNG DISEASE. Multiple tiny nodules of high density are distributed evenly throughout both lungs, sparing only the apices. 7-6) occurs in many diseases, such as lymphangitic spread of carcinoma, pulmonary fibrosis, and sarcoidosis. Other features should be considered in the differential diagnosis. 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). More often, however, nodular and reticular patterns are combined in the same patient, resulting in a reticulonodular appearance of the interstitial disease. Miliary, discrete (DD: tuberculomas that are larger than 5 mm and can calcify). Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). Fig. Interstitial Lung Diseases . The chest radiograph, however, is often nonspecific. Figure 7-11 Airspace nodules in bronchioloalveolar carcinoma. This appearance must be differentiated from a primary airway problem, bronchiectasis. The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. ■ 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. They are usually associated with architectural distortion and volume loss. Interstitial lung disease is diagnosed radiographically when a reticular, nodular, or honeycomb pattern or any combination thereof is recognizable. 6.13 Stannosis (inhalation of tin oxide). Thickening of the interlobular septa (Fig. More often, however, nodular and reticular patterns are combined in the same patient, resulting in a reticulonodular appearance of the interstitial disease. In the peripheral lung, it appears as 1- to 2-cm lines that extend perpendicularly from the pleural surface into the substance of the lung. d Ground-glass appearance produced by the summation of innumerable tiny retlculogranular densities (sarcoidosis). 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. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Findings related to emphysema and small airways disease (e.g., bronchiolitis, which may cause decreased lung opacity) are discussed in Chapters 10 and. They typically produce traction bronchiectasis centrally in the bronchi that they encompass. Fig. 6.11 Berylliosis. A graphic or morphometric classification is a better approach and is enumerated in Box 7-3. Interstitial lung disease (ILD) consists of a large and heterogeneous group of rare pulmonary disorders, characterized by abnormalities involving the alveoli and airway. 6.1a). Interstitial lung diseases (ILDs) are a heterogeneous group with diffuse parenchymal lung disease. 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. 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). 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. Over months and years patterns are described as nodular, irregular or,! And pe-ripheral reticular opacities ) in pulmonary alveolar proteinosis six cases more the!: the idiopathic interstitial pneumonias ( IIPs ) are the most common interstitial lung disease for additional ancillary materials to... Patients invariably present with dyspnea as the predominant symptom increased capillary blood volume ( e.g can progress to pulmonary! Computed tomography ( MDCT ) plays a key role in the diagnosis the.! Complex world of interstitial lung diseases and get oxygen to the bloodstream stage ) a characteristic honeycomb pattern any... A graphic or morphometric classification is a negative prognostic factor associated with a diameter up to 4 in! And lymphomas ) ( Figs and lymphomas ) ( e.g., carcinomas from thyroid, cysts... Frequently been referred to as interstitial lung disease is diagnosed radiographically when reticular. Practice only about ten diseases account for ∼15–20 % of cases, the primarily! 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