3.21 • Organizing pneumonia. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. The ERS-education website provides centralised access to all educational material produced by the European Respiratory Society. FIG. 10. FIG. E: Reticulonodular ILD results from a combination of reticular and nodular opacities, or it can be caused by reticular opacities seen end-on. Patients are usually elderly and have been exposed to amiodarone, usually for at least six months, although there is a poor correlation with dosage or cumulative dose. No etiologic agent is identified. Consolidation is present on CT images in 90% of patients with COP, with a subpleural or peribronchial distribution in up to 50% of cases (4) (Figs. 3.20 • Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD). 3.4). An uncommon pattern of edema is more common than an uncommon cause of ILD. The disease was found to be bilateral in 15 patients. anti-Jo-1 antibody positive interstitial lung disease, interstitial pneumonia with autoimmune features, non-specific interstitial pneumonia (NSIP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), combined pulmonary fibrosis and emphysema (CPFE), All Idiopathic Chronic Lung Diseases aRe Nonspecific, acute unilateral airspace opacification (differential), acute bilateral airspace opacification (differential), acute airspace opacification with lymphadenopathy (differential), chronic unilateral airspace opacification (differential), chronic bilateral airspace opacification (differential), osteophyte induced adjacent pulmonary atelectasis and fibrosis, pediatric chest x-ray in the exam setting, normal chest x-ray appearance of the diaphragm, posterior tracheal stripe/tracheo-esophageal stripe, obliteration of the retrosternal airspace, leflunomide-induced acute interstitial pneumonia, fibrotic non-specific interstitial pneumonia, cellular non-specific interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, diagnostic HRCT criteria for UIP pattern - ATS/ERS/JRS/ALAT (2011), diagnostic HRCT criteria for UIP pattern - Fleischner society guideline (2018)​, domestically acquired particulate lung disease, lepidic predominant adenocarcinoma (formerly non-mucinous BAC), micropapillary predominant adenocarcinoma, invasive mucinous adenocarcinoma (formerly mucinous BAC), lung cancer associated with cystic airspaces, primary sarcomatoid carcinoma of the lung, large cell neuroendocrine cell carcinoma of the lung, squamous cell carcinoma in situ (CIS) of lung, minimally invasive adenocarcinoma of the lung, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), calcifying fibrous pseudotumor of the lung, IASLC (International Association for the Study of Lung Cancer) 8th edition (current), IASLC (International Association for the Study of Lung Cancer) 7th edition (superseeded), 1996 AJCC-UICC Regional Lymph Node Classification for Lung Cancer Staging, autoimmune and collagen vascular diseases. Nonspecific interstitial pneumonia (NSIP) is the next most frequent. The radiological appearances are not specific for the underlying cause of diffuse lung disease in many cases. 3.11 • Cardiogenic pulmonary edema. Radiographics. A: Linear ILD is seen as Kerley lines. ILD may result in four patterns of abnormal opacity on chest radiographs and CT scans: linear, reticular, nodular, and reticulonodular (Fig. Kerley A lines are generally longer (2 to 6 cm), they radiate out from the hilum toward the pleura but are not contiguous with the pleura, and they are most obvious in the upper and middle lungs. 3.19 • Respiratory bronchiolitis. The typical CT feature of NSIP is predominantly basilar ground-glass and reticular opacities (Fig. Because the CT features of NSIP may overlap with those of organizing pneumonia, DIP, and UIP, a surgical lung biopsy should be considered when the CT pattern suggests NSIP (Fig. 2. Check for errors and try again. Alexander J. Procter, Joseph Jacob, Visual vs. computer-based computed tomography analysis for the identification of functional patterns in interstitial lung diseases, Current Opinion in Pulmonary Medicine, 10.1097/MCP.0000000000000589, 25, 5, (426-433), (2019). Minimal changes 1. Table 3.1  DIFFERENTIAL DIAGNOSIS OF INTERSTITIAL LUNG DISEASE, Bugs (especially fungi, Mycoplasma, and viruses), Lymphoid interstitial pneumonia and other idiopathic interstitial pneumonias, Scleroderma and other collagen vascular diseases, Histiocytosis (Langerhans cell histiocytosis). 8. List and identify on a chest radiograph and computed tomographic (CT) scan the four patterns of interstitial lung disease (ILD): linear, reticular, reticulonodular, and nodular. diagnosis of lung diseases that depend on higher resolution images such as interstitial lung diseases and pulmonary vascular diseases. 6. DIP is characterized histologically by spatially homogeneous thickening of alveolar septa, which is associated with intra-alveolar accumulation of macrophages. Recognize the findings of lymphangioleiomyomatosis (LAM) on a chest radiograph and CT scan. When seen as a result of a reversible process, such as viral pneumonia, sarcoidosis, or hypersensitivity pneumonitis, the distribution can be patchy or diffuse. Chest radiographs may show widespread bilateral nodular or reticular opacities, and they may take several weeks to return to normal. CT scan shows multiple bilateral, round, pulmonary nodules. mildly abnormal lung parenchyma by use of a CT value thresholding technique. B: Close-up view of (A), lower right lung, shows short, linear opacities perpendicular to the lateral pleural edge, representing Kerley B lines. 1-6 What every radiologist should know about idiopathic interstitial pneumonias. Pulmonary disease usually results in either an increase or a decrease in radiographic opacity of the lungs. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. CT scan shows multiple circumscribed, round pulmonary nodules, 2 to 3 mm in diameter, and scattered reticular opacities. The parenchymal abnormalities of NSIP may be reversible on follow-up CT scanning. Interstitial lung diseases (ILD) involve several abnormal imaging pat- terns observed in computed tomography (CT) images. The term AIP is reserved for diffuse alveolar damage of unknown origin. Interstitial edema is seen on chest radiographs and CT scans as blurring of the margins of the blood vessels and bronchial walls (peribronchial cuffing), thickening of the fissures (subpleural edema), and thickening of the interlobular septae (Kerley lines) (Fig. Poorly defined, low-density nodules associated with bronchial wall thickening, thickened interlobular septa and mosaic pattern: Churg–Strauss syndrome 181 and eosinophilic pneumonia 236: Indistinct nodules, often with other parenchymal and airways abnormalities FIG. 4. Risk factors include 6: 1. treatment longer than two months 2. age over 60 years 3. daily dose >400 mg 4. antecedent lung disease or surgery 5. prior angiograp… The typical CT findings are ground-glass and reticular opacities, sometimes associated with perivascular cysts (Fig. Organizing pneumonia, which is commonly caused by bleomycin and cyclophosphamide (as well as gold salts and methotrexate), appears on radiographs as hetero- and homogeneous peripheral opacities in both upper and lower lobes and on CT scans as poorly defined nodular consolidation, centrilobular nodules, and bronchial dilatation. This list excludes the relatively uncommon diagnosis of amyloidosis. A reticular pattern results from the summation or superimposition of irregular linear opacities. 3.18 • Desquamative interstitial pneumonia (DIP). 2002;22 Spec No : S151-65. Interstitial lung diseases (ILD) involve several abnormal imaging patterns observed in computed tomography (CT) images. A usual interstitial pneumonia (UIP) pattern on chest CT scans is highly suggestive of UIP pathologic findings; the most common cause of UIP is idiopathic pulmonary fibrosis (IPF) [1–5].Under current guidelines, a UIP pattern on CT images is specific for IPF after a thorough clinical and serologic workup has excluded other causes of interstitial lung disease (ILD) []. This patient had Sjögren syndrome and new respiratory symptoms. Most diseases result in an increase in opacity, usually characteristic for the pulmonary parenchymal component (alveolar, bronchial, interstitial, vascular) involved. The 6 Patterns of Interstitial Lung Disease Prototype: DAD 1. This distance learning portal contains up-to-date study material for the state-of-the-art in Pulmonology. Acute lung injury Ground Glass and Consolidation Fibrosis/ honeycombing Nodules/masses Airtrapping + cysts 20 CT Patterns Histopathology Patterns . The PA chest radiograph shows a diffuse pattern of nodules, 6 to 10 mm in diameter. FIG. One must decide what the predominant pattern is, take into consideration the clinical history and any associated radiographic findings, or further define the pattern(s) and distribution of disease with a CT scan of the lungs. 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). Recognize progressive massive fibrosis/conglomerate masses secondary to silicosis or coal worker’s pneumoconiosis on a chest radiograph and CT scan. 3.13). A middle or upper lung–predominant distribution suggests mycobacterial or fungal disease, silicosis, sarcoidosis, LCH, extrinsic allergic alveolitis (hypersensitivity pneumonitis), or, very rarely, ankylosing spondylitis. Temporal heterogeneity is an important histologic feature and helps to distinguish UIP from DIP. CT features of UIP and organizing pneumonia may be diagnostic in the correct clinical context, but those of NSIP, DIP, RB-ILD, AIP, and LIP are less specific. Table 3.4  PULMONARY EDEMA WITH A NORMAL-SIZED HEART. Kerley A lines radiate out from the hila to the periphery of the lung. CT scan of a patient living in Arizona shows numerous small nodules in a random distribution and pleural effusions. B: Reticular ILD is seen as a network of curvilinear opacities. IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. Other manifestations of pulmonary drug toxicity include eosinophilic pneumonia, constrictive bronchiolitis, pulmonary hemorrhage, edema, hypertension, and veno-occlusive disease. 3.12). Mueller-mang C, Grosse C, Schmid K et-al. Mycoplasma pneumoniae usually affects previously healthy individuals between the ages of 5 and 40 years (7). B: CT scan shows bilateral reticular and ground-glass opacities and areas of consolidation. 3.16). CT scan shows numerous tiny nodules in a random distribution. Hydrostatic pulmonary edema is defined as abnormal water in the lungs secondary to elevated pulmonary venous pressure from a failing left ventricle, mitral stenosis, increased circulating blood volume (as with anemias), renal failure (causing fluid retention), or overhydration. The spatial and temporal homogeneity of this pattern is important in distinguishing NSIP from UIP. Define “asbestos-related pleural disease” and “asbestosis”; identify each on a chest radiograph and CT scan. The chest radiograph may also show associated findings of cardiomegaly, pleural effusions, widening of the vascular pedicle, enlargement of the azygos vein, and vascular redistribution (Fig. FIG. Fibrosis 3. Lung volumes are decreased. 3.24 • Influenza pneumonia. The most common HRCT patterns seen in cases of diffuse interstitial lung diseases are the nodular pattern, linear/reticular opacities, cystic lesions, ground-glass opacities and consolidations. Respiratory bronchiolitis is a histopathologic lesion found in cigarette smokers and is characterized by the presence of pigmented intraluminal macrophages within respiratory bronchioles (4). FIG. It may occur when an injury to the lungs triggers an abnormal healing response. Agarwala S(1), Kale M(2), Kumar D(1), Swaroop R(1), Kumar A(3), Kumar Dhara A(4), Basu Thakur S(5), Sadhu A(6), Nandi D(1). This patient had a long history of cigarette smoking and no respiratory symptoms. Idiopathic LIP is rare. 28 (5): 1383-96. Author information: (1)Department of Computer Science and Engineering, National Institute of Technology Durgapur, Durgapur, 713209, India. Interstitial lung disease (ILD) includes a large group of pulmonary disorders that result in scarring or fibrosis of the lung parenchyma. Attili AK, Kazerooni EA, Gross BH et-al. Accurate classification of these patterns plays a significant role in precise clinical decision making of the extent and nature of the diseases. The diffuse lung diseases tend to cause infiltrative opacification in the periphery of the lung, but patterns vary among the different etiologies. Nodular opacities may be described as miliary (1 to 2 mm, the size of millet seeds), small, medium, or large, as the diameter of the opacities increases (Figs. B: CT scan shows bilateral subpleural honeycombing (dashed arrow), traction bronchiectasis (solid arrows), and a background of ground-glass opacity. Air bronchograms, with mild cylindric bronchial dilatation, are common. 3.3 • Cardiogenic edema and Kerley lines. C: When reticular ILD is seen as a result of chronic, irreversible lung disease, such as usual interstitial pneumonia, honeycombing is seen. Smoking-related interstitial lung disease: radiologic-clinical-pathologic correlation. 3.2 and 3.3). DIP is more common in men than in women. 27 (3): 595-615. Kerley B lines are shorter lines that contact and are perpendicular to the lateral pleural edge, predominantly in the lower lungs. These septal lines were first described by Kerley in patients with pulmonary edema (3). Pneumocystis pneumonia also produces a fine interstitial pattern on chest radiography, and is discussed in Chapter 16. NSIP occurs most commonly as a manifestation of carmustine toxicity or of toxicity from noncytotoxic drugs such as amiodarone. An acute appearance suggests pulmonary edema or pneumonia (Figs. Many patients will then undergo CT scanning of the chest, and, depending on the findings, may proceed to transbronchial biopsy, bronchoalveolar lavage, or surgical lung biopsy. Histologically, organizing pneumonia is distinguished by patchy areas of consolidation characterized by polypoid plugs of loose organizing connective tissue with or without endobronchiolar intraluminal polyps. FIG. Diffuse alveolar damage is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. Conclusion This study concluded that HRCT images are very useful in diagnosing interstitial lung disease related to rheumatoid arthritis. Uncommon patterns of pulmonary edema can result from patient positioning or underlying perfusion abnormalities in the nonedematous lung (e.g., secondary to pulmonary embolism or asymmetric emphysema). Four major CT patterns were identified: usual interstitial pneumonia (n = 26), nonspecific interstitial pneumonia (n = 19), bronchiolitis (n = 11), and organizing pneumonia (n = 5). Mechanical ventilation is usually required. It manifests radiographically as bilateral hetero- or homogeneous opacities usually in the mid- and lower lungs and on CT scans as scattered or diffuse areas of ground-glass opacity. Rheumatoid arthritis–interstitial lung diseases (RA-ILD) was classified into the four CT image patterns (usual interstitial pneumonia (UIP), probable UIP, non-specific interstitial pneumonia (NSIP) and unclassifiable). 3.10 • Aspergillosis and reticulonodular ILD. Functional respiratory tests commonly show an abnormal restrictive pattern and reduced diffusing capacity. Table 3.2  DIFFERENTIAL DIAGNOSIS OF KERLEY LINES. 3.11). 1. AJR Am J Roentgenol. The vertical dotted line indicates the OR of 1 and the x-axis is drawn with a logarithmic scale in the both figures. FIG. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Respiratory bronchiolitis, RB-ILD, and DIP are regarded as a continuum of smoking-related lung injuries. FIG. To investigate the histopathologic pattern and clinical features of patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD) according to the American Thoracic Society (ATS)/European Respiratory Society consensus classification of idiopathic interstitial pneumonia. 3.5 and 3.6). Distinction of UIP from other IIPs is important, because UIP is associated with a poorer prognosis than the other entities. Y1 - 2017/3/1. T1 - Patterns of interstitial lung disease and mortality in rheumatoid arthritis. The 4 CT Patterns of Pulmonary Disease . PA chest radiograph shows enlargement of the cardiac silhouette, bilateral ILD, enlargement of the azygos vein (solid arrow), and peribronchial cuffing (dashed arrow). The histologic features of DIP are similar to those of RB-ILD (a condition seen exclusively in smokers), although the distribution of DIP is diffuse and RB-ILD has a predominantly bronchiolocentric distribution. 3.13 • Usual interstitial pneumonia (UIP). The prognosis of NSIP is substantially better than that of UIP. A conundrum arises when widespread small opacities are difficult to categorize into one group or the other on chest radiography, or when ILD and ALD are both present. B: CT scan shows multiple small cysts (honeycombing) involving predominantly the subpleural peripheral regions of lung. FIG. The histologic feature of LIP is alveolar septal interstitial infiltration by lymphocytes and plasma cells. Cryptogenic organizing pneumonia (COP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis–associated interstitial lung disease (RB-ILD), and acute interstitial pneumonia (AIP) are less common, and lymphoid interstitial pneumonia (LIP) is rare. ; interstitial lung disease: ct patterns CT is a noninvasive technique for monitoring disease morphology in RA-associated lung disease presents nearly insurmountable challenges the. 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