The presence of predominant ground-glass and reticular opacities is highly characteristic of NSIP, but there is a subset of patients with UIP who have this pattern and may require biopsy for differentiation from NSIP (Fig. (2020) PLOS ONE. 3.16). Usual interstitial pneumonia (UIP). In the correct clinical context, the CT features of UIP are often diagnostic. Lukas Ebner, Stergios Christodoulidis, Thomai Stathopoulou, Thomas Geiser, Odile Stalder, Andreas Limacher, Johannes T. Heverhagen, Stavroula G. Mougiakakou, Andreas Christe. Infectious pneumonia resulting in a diffuse interstitial pattern is unusual; however, viral, fungal, mycobacterial, and Mycoplasma pneumonias may be predominantly interstitial or interstitial-appearing. A large number of disorders fall into this broad category. 27 28 These diseases may be acute or chronic and have a variety of underlying causes, including infection, exposure to dust or other particles, or an underlying genetic predisposition. Other manifestations of pulmonary drug toxicity include eosinophilic pneumonia, constrictive bronchiolitis, pulmonary hemorrhage, edema, hypertension, and veno-occlusive disease. Jonathan H. Chung, Christian W. Cox, Steven M. Montner, et al. 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. However more recently the term idiopathic pulmonary fibrosis has been applied solely to the clinical syndrome associated with the morphologic pattern of UIP, with the specific exclusion of entities such as non-specific interstitial pneumonia (NSIP) and desquamative interstitial pneumonia (DIP) 1. This 45-year-old woman presented with metastatic gastric carcinoma. B: Close-up view of (A), lower right lung, shows short, linear opacities perpendicular to the lateral pleural edge, representing Kerley B lines. An ILA refers to a subtle or mild parenchymal abnormality identified in more than 5% of lungs on CT scans in patients in whom interstitial lung disease was not previously clinically suspected (Fig 1). 3.15 • Nonspecific interstitial pneumonia (NSIP). FIG. An interstitial lung pattern is a regular descriptive term used when reporting a plain chest radiograph. Reticular opacities can be described as fine, medium, or coarse, as the width of the opacities increases. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified. Respiratory bronchiolitis interstitial lung disease (RB-ILD) is a smoking related interstitial lung disease closely related to respiratory bronchiolitis, but demonstrating more severe histological, imaging and clinical findings. FIG. Table 3.4  PULMONARY EDEMA WITH A NORMAL-SIZED HEART. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. ILD may result in four patterns of abnormal opacity on chest radiographs and CT scans: linear, reticular, nodular, and reticulonodular (Fig. The morphologic, or “pattern,” of each IIP seen at histologic or CT examination is linked to a specific clinical syndrome. FIG. Still, our … FIG. 19. 3.4). 2005;236 (1): 10-21. 3.19 • Respiratory bronchiolitis. The nodules generally range from 1 to 10 mm in size. 3.9 and 3.10). Mechanical ventilation is usually required. FIG. 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. Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema: thin-section CT findings. ... Hospital Build Middle East, Congress of the Brain Tumor Radiology in Neuro-oncology Society. Introduction • Interstitial lung diseases (ILD’s)represent a large number of conditions that involve the parenchyma of lung- the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between these structures, as well as perivascular and lymphatic tissues. In a study from a large tertiary referral center in the United Kingdom, the biopsy specimens in 168 cases over an 18-year period were retrospectively reviewed, and 13 (8%) of these showed a dominant pattern of RB-ILD. Mueller-mang C, Grosse C, Schmid K et-al. 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. The parenchymal abnormalities of NSIP may be reversible on follow-up CT scanning. 4. NSIP occurs most commonly as a manifestation of carmustine toxicity or of toxicity from noncytotoxic drugs such as amiodarone. A: Linear ILD is seen as Kerley lines. 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. 2014;14 Suppl 1: S2. 2Pulmonary Unit, GB Morgagni Hospital, Forlı`, Italy. In these cases, coming up with a differential diagnosis is not as straightforward. Lynch DA, Travis WD, Müller NL et-al. On HRCT there are four patterns: reticular, nodular, high and low attenuation (table). Kerley B lines are shorter lines that contact and are perpendicular to the lateral pleural edge, predominantly in the lower lungs. Background: Spontaneous pneumothorax is a complication that occurs in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD); however, few studies on the clinical implications of pneumothorax for patients with CTD-ILD have been performed. 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. ; We fund research. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?. No etiologic agent is identified. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. CT scan shows multiple bilateral, round, pulmonary nodules. Reticulation is frequently seen but is typically limited to the lung bases. At radiography, it appears as diffuse areas of heterogeneous opacity, whereas early CT scans show diffuse ground-glass opacity, and late CT scans show fibrosis in a basal distribution. Interstitial lung disease (ILD) is a group of many lung conditions.All interstitial lung diseases affect the interstitium, a part of your lungs. Res. Patients with AIP present with respiratory failure developing over days or weeks. Interstitial Lung Disease and Emphysema. 12. • The radiographic and pathologic abnormalities in patients can be classified into acute, subacute, … CT scan shows multiple circumscribed, round pulmonary nodules, 2 to 3 mm in diameter, and scattered reticular opacities. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. 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. In all cases, RB-ILD is typically associated with heavy smoking (usually of 30 pack-years or more) and is often seen in young middle-aged patients (30-40 years of age). On HRCT there are four patterns: reticular, nodular, high and low attenuation (table). FIG. 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. Interstitial lung disease is diagnosed radiographically when a reticular, nodular, or honeycomb pattern or any combination thereof is recognizable.. C: Close-up of (A), right upper lung, shows linear opacities (arrow) radiating outward from the hila, representing Kerley A lines. Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. 1 Presentation is often insidious and indistinguishable from the underlying disease process being treated by the drug. Share Claim CME/SAM Claim CME + Affiliations: 1 Department of Radiology, University of Texas Houston Medical School, 6431 Fannin St, MSB … Anything that causes thickening of the interlobular septa can produce Kerley lines, including edema, inflammation, tumor, or fibrosis. Search; Cart; Admin; HEALTH TOPICS interstitial lung disease radiologyhow to interstitial lung disease radiology for the 1 last update 2021/01/07 Browse by Topic. American journal of roentgenology. In more advanced stages of … Recognize the typical appearance and upper lobe–predominant distribution of irregular lung cysts or nodules on chest CT of a patient with Langerhans cell histiocytosis (LCH). Findings usually improve with steroid treatment. The positive predictive value of CT in the diagnosis of UIP is high and ranges from 70-100% 1. Unable to process the form. 27 28 These diseases may be acute or chronic and have a variety of underlying causes, including infection, exposure to dust or other particles, or an underlying genetic predisposition. FIG. Idiopathic interstitial pneumonias: CT features. 3.5 and 3.6). CT scan of a patient living in Arizona shows numerous small nodules in a random distribution and pleural effusions. A nodular pattern consists of multiple round opacities, generally ranging in diameter from 1 mm to 1 cm, which may be difficult to distinguish from one another as individual nodules on a chest radiograph. Kim DS, Collard HR, King TE. 3.24). The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation. 3.17 and 3.18). Gruden J. American Journal of Roentgenology. HRCT obtained during the accelerated phase of the disease demonstrates a generalized increase in lung attenuation and progression of both the reticular and honeycomb patterns. Despite substantial limitations, radiography remains the most widely used method for diagnosis and monitoring of many occup… 6.1a).Depending on the mesh size, one can distinguish between fine, medium, and coarse reticular patterns, although this distinction has … 3.25). 3. 3.20 • Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD). 3.20) but are at least partially reversible in patients who stop smoking. 15 (1): e0226084. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. 165 (4): 807-11. List two causes of upper lobe–predominant ILD (chronic hypersensitivity pneumonitis, sarcoidosis). idiopathic pulmonary fibrosis). Interstitial lung disease can be triggered by many things — including autoimmune diseases, exposure to organic and inorganic agents in the home or workplace, medications, and some types of radiation. Alternatively, dense airspace opacity may be seen involving one or several lobes. In this section of Radiology Rounds, we will discuss HRCT technique, identification of radiologic features such as honeycombing and reticulation, which are typical for interstitial lung disease, and CT features of comorbid conditions such as pulmonary arterial hypertension. The distribution of UIP characteristically is with an apicobasal gradient with basal and peripheral (subpleural) predominance, although it is often patchy. Exposure to occupational and environmental toxins. 2. This list excludes the relatively uncommon diagnosis of amyloidosis. 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. In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.Some of the following tests may be necessary. In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. These septal lines were first described by Kerley in patients with pulmonary edema (3). The interstitium of the lung is not normally visible radiographically; it becomes visible only when disease (e.g., edema, fibrosis, tumor) increases its volume and attenuation. Interstitial lung disease (ILD) refers to a broad category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis of the lungs. 2020 Jul 9 ... 12 Division of Radiology, Medical School of the University of São Paulo, São Paulo, Brazil. Chest radiographs may show widespread bilateral nodular or reticular opacities, and they may take several weeks to return to normal. 5. Otaola M, Quadrelli S, Tabaj G et-al. While chest radiographs can be even normal in patients with very early disease, in advanced disease, it may show decreased lung volumes and basal fine to coarse reticulation. 3.23 • Lymphoid interstitial pneumonia (LIP). Honeycombing, particularly if it involves more than 5% of the lung volume, is an almost 100% specific finding. The curvilinear opacities form small cystic spaces (forming the honeycomb) in a characteristic bibasilar and subpleural distribution. D: CT shows interlobular septal thickening (arrows), representing Kerley lines. 3.14 • Systemic lupus erythematosus and UIP. UIP is characterized histologically by a patchy heterogeneous pattern with foci of normal lung, interstitial inflammation, fibroblastic proliferation, interstitial fibrosis, and honeycombing. Michael P. Mohning, John Caleb Richards, Tristan J. Huie. In adults, LIP is commonly associated with connective tissue disorders (particularly Sjögren syndrome), immunodeficiency syndromes, and Castleman syndrome. This page considers all aspects of the appearances of interstitial and alveolar opacity demonstrated on chest plain film imaging. Recently, clinicians have begun to notice the increased incidence of DRP associated with molecular targeted therapy and immunotherapy in patients with cancer and pre-existing interstitial lung disease (ILD) or interstitial lung abnormality (ILA). Interstitial lung disease Katerina M. Antoniou 1, George A. Margaritopoulos , Sara Tomassetti2, Francesco Bonella 3, Ulrich Costabel and Venerino Poletti2 Affiliations: 1Dept of Thoracic Medicine and Laboratory of Cellular and Molecular Pneumonology, Medical School, University of Crete, Crete, Greece. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. In RB-ILD, the findings are more extensive (Fig. The interstitial pneumonias are a heterogeneous group of nonneoplastic diffuse parenchymal lung diseases that result from damage to the lungs by varying combinations of inflammation and fibrosis [1?? Usual interstitial pneumonia. 2 The temporal presentation and correlation are inconsistent and can vary with different drug classes. An uncommon pattern of edema is more common than an uncommon cause of ILD. Radiographers who are able to differentiate alveolar from interstitial lung patterns are operating at a very high level and will find a whole new appreciation of chest radiography. (2019) The British Journal of Radiology. Recognize the changes of congestive heart failure on a chest radiograph (enlarged cardiac silhouette, pleural effusions, vascular redistribution, interstitial or alveolar edema, Kerley lines, enlarged azygos vein, increased ratio of artery-to-bronchus diameter). 3. This is often the case with sarcoidosis, LCH, silicosis, and coal worker’s lung. 13. Table 3.2  DIFFERENTIAL DIAGNOSIS OF KERLEY LINES. A large number of disorders fall into this broad category. The spatial and temporal homogeneity of this pattern is important in distinguishing NSIP from UIP. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. 2011;140 (4 Meeting Abstracts): . 14. Radiology. Septal thickening without architectural distortion is more likely to represent pulmonary edema. Respiratory bronchiolitis, RB-ILD, and DIP are regarded as a continuum of smoking-related lung injuries. Patients with hypersensitivity pneumonitis often have a history of exposure to an inciting agent and are usually nonsmokers. FIG. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF)-practical implications. 20. Fungal disease is discussed in Chapter 7. 5 Particularly interstitial lung disease [ILD] and granulomatous lung disease [GLD] are rare respiratory conditions. 7. Similar to the pathology specimen, cross-sectional imaging also reveals heterogeneity, with patchy areas of fibrosis alternating with areas of normal lung 5. Associated lymphadenopathy suggests sarcoidosis; neoplasm (lymphangitic carcinomatosis, lymphoma, metastases); infection (viral, mycobacterial, or fungal); and silicosis. Interstitial lung disease (ILD) comprises a diverse group of lung diseases with overlapping clinical, radiological, ... their presence is not routinely recorded on radiology reports, even at academic centres.20 ILAs are likely to be increasingly identified with the implementation of lung cancer screening and increased use of CT for other diagnostic purposes. 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). A reticular pattern results from the summation or superimposition of irregular linear opacities. Retrospective radiologic image evaluation of patients developing ILD was also performed. FIG. The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. 2006;3 (4): 285-92. non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), connective tissue disorder interstitial lung disease, diagnostic HRCT criteria for UIP pattern - ATS/ERS/JRS/ALAT (2018), diagnostic HRCT criteria for UIP pattern - Fleischner society guideline (2018), fibrotic non-specific interstitial pneumonia, chronic hypersensitivity pneumonitis (HP), combined pulmonary fibrosis and emphysema (CPFE), 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, 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, connective tissue disorders (CTD associated UIP): falls under the borader spectrum of. 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