Punctate densities (hemosiderosis) and densely calcified 2 to 8 mm nodules (pulmonary ossification), predominantly in the mid and lower lung fields, usually more numerous on the right side. 6.4 Metastases from breast carcinoma. 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. 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. More than 180 disease entities are characterized by acute, subacute, or chronic inflammatory infiltration of alveolar walls by cells, fluid, and connective tissue. Abstract: The idiopathic interstitial pneumonias (IIPs) are the most commonly diagnosed forms of interstitial lung disease. Fig. Linear opacities of nonspecific interstitial pneumonia. Similarly, pleural effusions and diffuse thickening are often associated with collagen vascular disease and asbestos exposure. Both congenital and acquired bronchiectases can be mistaken radiographically for localized interstitial lung disease (Fig. A honeycomb pattern is characterized by round or oval cystic lesions with a diameter up to 1 cm (Fig. Figure 7-11 Airspace nodules in bronchioloalveolar carcinoma. These disorders are grouped together because of similarities in their clinical presentations, plain chest radiographic appearance, and… Thick-walled cysts are seen in the both lung bases. 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. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. 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. Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. It is often accompanied by other signs of interstitial lung disease, especially the patterns associated with reticular opacities and architectural distortion. 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. Parenchymal consolidation (i.e., airspace or alveolar disease). 6.3 Bronchioloalveolar carcinoma. Ill-defined nodules up to 1 cm in diameter are identified in both lungs. It is found, besides in interstitial diseases, also with air-space disease (e.g. 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. Diseases that cause a characteristic honeycomb pattern are summarized in Table 6.2. 6 Interstitial Lung Disease. The patterns are described as nodular, irregular or linear, cystic, ground-glass, and parenchymal consolidation. They are not truly acinar but may be considered airspace nodules (Fig. They are most numerous at the base of the lungs. Miliary nodulation only manifest 2–5 weeks after initial exposure (third phase of disease). Diffuse bilateral poorly defined small nodular densities are present, but only shown for the right lower lung field. A graphic or morphometric classification is a better approach and is enumerated in Box 7-3. A ground-glass appearance (Fig. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. The standard chest radiograph remains the basic and, in some cases, the only imaging technique that is useful. The nodular pattern (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. Idiopathic pulmonary fibrosis (usual interstitial pneumonitis). These structures typically have a central dot that represents the pulmonary artery. Interstitial nodules (Fig. 7-9). They have been called parenchymal bands and long lines. They are usually associated with architectural distortion and volume loss. Kerley A lines are usually best seen in the mid and lower lung fields. Notice the dilated bronchus in the right upper lobe (thin arrow) and the subpleural honeycomb cysts (thick arrow). (e.g., carcinomas from thyroid, lung, breast or gastrointestinal tract, or melanomas, sarcomas and lymphomas) (Fig. Smoking cessation often improves symptoms; the disease also … Figure 7-13 Honeycombing in idiopathic pulmonary fibrosis. It consists of an amorphous opacification or increase in attenuation, which is mildly severe and is not sufficient to obliterate the pulmonary vessels. 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. Finally, fibrotic scars may be the sequelae of virtually any disease capable of damaging the lung parenchyma severely enough. Calcification extremely rare. Cystic bronchiectases may produce a radiographic picture similar to honeycombing. In approximately 50% of cases, they are limited to one lung. Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. 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. Honeycombing represents an end-stage lung that is destroyed by fibrosis. Acute Kerley lines are frequently associated with prominent interlobar fissures caused by subpleural edema. 6.2). Figure 7-2 Linear opacities of nonspecific interstitial pneumonia. Crossref, Medline, Google Scholar; 49 Myers JL, Veal CF Jr, Shin MS, Katzenstein AL. 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. 6.8). Healing may result in punctate calcifications years later. The intralobular bronchiole often becomes visible when there is centrilobular thickening. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. Webb and colleagues describe such HRCT findings in interstitial lung disease further in their work (see Suggested Readings). The size of the lung (i.e., lung volumes) may be a clue to the differential diagnosis (Box 7-7). Thickening of the axial interstitium (i.e., interstitium in a peribronchovascular location) (Fig. 7-7) is common in many interstitial lung diseases. Although these disorders have frequently been referred to as interstitial lung diseases, many also involve the alveolar spaces. There is also thickening of the axial interstitium along the bronchi. 6.6 Histoplasmosis (2 cases). In approximately 50% of cases, they are limited to one lung. After completing this journal-based SA-CME activity, participants will be able to: 1. Some types of autoimmune diseases, such as rheumatoid … There is also thickening of the axial interstitium along the bronchi (arrow) and nodules along the fissures (thick arrows). Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases. 6.13 Stannosis (inhalation of tin oxide). 6.7 Aspergillosis. Many chronic diseases can produce diffuse opacities in the lung. A central dot in the lobule is prominent, and the intralobular bronchiole is visible (arrowhead). The process of evaluating a surgical lung biopsy for disease involves answering several questions. 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. These diseases account for ∼15–20% of general pulmonary practice. Often poorly defined, confluent nodules of varying size. These diseases represent specific clinicopathologic entities characterized by varying degrees of lung parenchymal inflammation and fibrosis. Thick-walled cystic spaces can be seen subpleurally in the bases. They have been called, Septal thickening in lymphangitic carcinomatosis. Exclusively these symptomatic cases are designated as respiratory bronchiolitis-interstitial lung disease. 6.15 Bird-fancier’s lung. CT is more sensitive in the identification of adenopathy and may demonstrate mildly enlarged lymph nodes in idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, fibrosis associated with the collagen vascular diseases, and lymphangioleiomyomatosis. May occasionally be the dominant feature. 6.1a–f Patterns of interstitial lung disease. Acute Kerley lines are frequently associated with prominent interlobar fissures caused by subpleural edema. 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. Calcification occurs. The ILD consist of disorders of known cause (e.g., collagen vascular diseases, drug-related diseases) as well as … 7-3) and honeycombing. 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. 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. Other features should be considered in the differential diagnosis. 6.1b). 6.4). However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. (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. This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). 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. 6.16 Langerhans cell histiocytosis (eosinophilic granuloma). c Nodular pattern (silicosis). 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. Pattern recognition in diffuse interstitial lung disease has been the subject of controversy for many years. 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. The characteristic com-puted tomographic ﬁndings in UIP are predominantly basal and pe-ripheral reticular opacities with honeycombing and traction bronchiec-tasis. Thin-walled cysts are seen in the lungs of a patient with lymphangioleiomyomatosis. Fig. Lung damage from ILDs is often irreversible and gets worse over time. 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). Fig. Interstitial Lung Diseases . The term A subpleural line may be defined as a curvilinear opacity that is less than 1 cm from the pleural surface. Irregular linear opacities (. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. Cystic abnormalities include honeycombing, traction bronchiectasis, lung cysts, and cavitary nodules. 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. 6.17 Mitral stenosis (2 cases). When these bronchiectatic segments become filled with retained secretion, they appear as homogeneous band-like densities (“gloved-finger” shadows). 6.14 Farmer’s lung. 6.1b). 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. Fig. 7-11). This appearance is typical for silicosis and for coal worker’s pneumoconiosis, but it may also occur in end-stage sarcoidosis. Fig. 7-1) is composed of multiple, small nodules that range from 1 mm to 1 cm in diameter. 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 Reticular pattern (Pneumocystis carinii pneumonia). 6.12 Siderosis. 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. The posteroanterior view shows coarse linear opacities distributed more in the lower lungs than upper areas. Pneumoconioses and vascular disorders are discussed in Chapters 8 and 9. Poorly defined, confluent nodules are seen bilaterally, but only shown for the right side. Table 6.3 summarizes all disorders that demonstrate radiographically a diffuse reticular or reticulonodular pattern characteristic of interstitial lung disease. b Kerley A lines (long arrows, touched up) and Kerley B lines (short arrows) (mitral stenosis). Miliary and larger, often indistinctly defined. 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. Multiple, small (1 to 3 mm) nodules are distributed diffusely throughout the lungs. Although rare, they are classified with similar clinical, radiological, physiologic, or pathologic signs. Fig. 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. 7.29). In the lower lobes, there are multiple, small, centrilobular ground-glass nodules. Multiple small nodules are scattered throughout both lung fields. 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). Clinical and radiologic correlation can aid the pathologist and refine the diagnosis. Amyloidosis (diffuse alveolar septal form), Talc granulomatosis secondary to intravenous drug abuse. 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. In t… Miliary and larger, often poorly defined; can form microabscesses. Finally, fibrosis of the interlobular septa can be associated with any form of pulmonary fibrosis, but is most frequently observed with pneumoconiosis. Figure 7-4 Honeycombing pattern in the usual interstitial pneumonitis of scleroderma. 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. Numerous fairly well defined miliary nodules are seen bilaterally besides diffuse reticular changes and early honeycombing, but are only shown for the right side. Discrete and extremely sharply defined, less than 1 mm in diameter. Chapter 56: Interstitial Lung Diseases. Many diseases demonstrate more than one pattern (see, Patterns of Opacities in Interstitial Lung Disease, NODULAR OR RETICULAR NODULAR PATTERN (SMALL, ROUNDED OPACITIES), LINEAR PATTERN (SMALL, IRREGULAR, RETICULAR OPACITIES), PARENCHYMAL CONSOLIDATION (AIRSPACE OR ALVEOLAR DISEASE). These nodules may be seen in patients with lobular pneumonia, endobronchial spread of tuberculosis, or bronchioloalveolar carcinoma. 6.1a). Thickened interlobular septa (hexagons) and a fine reticular pattern are visible within the lobules. A honeycomb pattern is characterized by round or oval cystic lesions with a diameter up to 1 cm (Fig. Notice the subpleural sparing at the fissures. 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. Honeycombing is the only dependable radiographic sign of interstitial fibrosis. Finely granular stippling uniformly distributed over both lung fields. The fibrotic disorders are characterized by marked restriction, and small lungs invariably are seen in idiopathic pulmonary fibrosis and related disorders. Small nodules up to 4 mm in diameter are identified in both lungs. 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. Pleural plaques, an uncommon feature, are produced almost exclusively by asbestos exposure. Density of the tiny nodules depends on the atomic number of the inhaled element. Fig. In this review we present the key findings in the most common interstitial lung diseases. 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 … 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. 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. There is ground-glass opacification and more confluent consolidation in the right lung. Extrinsic allergic alveolitis (e.g., farmer’s lung, bird-fancier’s lung, mushroom-worker’s lung, bagassosis, and others) (Figs. Figure 7-7 Septal thickening in lymphangitic carcinomatosis. Exclusively these symptomatic cases are designated as respiratory bronchiolitis-interstitial lung disease has been the of. Disease further in their work ( see Suggested Readings ) for the right lower lung field ’ lung... 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