CT scan shows numerous ground-glass nodules in a centrilobular distribution (arrows). On HRCT there are four patterns: reticular, nodular, high and low attenuation (table). AMERICAN THORACIC SOCIETY 25 Broadway New York, NY 10004 United States of America Phone: +1 (212) 315-8600 Fax: +1 (212) 315-6498 Email: atsinfo@thoracic.org FIG. A: PA chest radiograph shows an enlarged cardiac silhouette and bilateral reticular and linear ILD. AIP is a rapidly progressive form of interstitial pneumonia characterized histologically by hyaline membranes within the alveoli and diffuse, active interstitial fibrosis indistinguishable from the histologic pattern found in acute respiratory distress syndrome caused by sepsis and shock. A: PA chest radiograph shows medium to coarse reticular ILD with a predominant bibasilar and subpleural distribution. B: Close-up view of (A), lower right lung, shows short, linear opacities perpendicular to the lateral pleural edge, representing Kerley B lines. 3.17 and 3.18). Most patients improve with cessation of smoking and oral corticosteroids. CT scan shows bibasilar reticular and ground-glass opacities. The most common cause of interlobular septal thickening, producing Kerley A and B lines, is pulmonary edema, as a result of pulmonary venous hypertension and distension of the lymphatics (Figs. The term interstitial lung diseases (ILD) comprises a diverse group of diseases that lead to inflammation and fibrosis of the alveoli, distal airways, and septal interstitium of the lungs. ?Clinical, Histologic, and CT Manifestations . FIG. 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. CT scan shows small nodules, reticular opacities, and septal thickening. The majority of patients are cigarette smokers in their fourth or fifth decade of life (6). Methods: Patients who had ILD events reported as an adverse drug … The idiopathic interstitial pneumonias (IIPs) are a heterogeneous group of diffuse parenchymal lung diseases that have no well-defined cause (4). The diagnostic process in diffuse lung disease begins with a clinical evaluation that includes a history, physical examination, chest radiograph, and lung function tests. In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. Akira M, Inoue Y, Kitaichi M et-al. Lukas Ebner, Stergios Christodoulidis, Thomai Stathopoulou, Thomas Geiser, Odile Stalder, Andreas Limacher, Johannes T. Heverhagen, Stavroula G. Mougiakakou, Andreas Christe. 19 (6): 1114. 3.12). 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. 2020 Jul 9 ... 12 Division of Radiology, Medical School of the University of São Paulo, São Paulo, Brazil. Dr. Sally Suliman presents "Interstitial Lung Disease" by first introducing the classification of ILDs. 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). Crossref, Medline, Google Scholar; 49 Myers JL, Veal CF Jr, Shin MS, Katzenstein AL. This page considers all aspects of the appearances of interstitial and alveolar opacity demonstrated on chest plain film imaging. 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. Although COP is primarily an intra-alveolar process, it is included in the classification of the IIPs because of its idiopathic nature and because its appearance may overlap with that of the other IIPs. Traction bronchiectasis, another sign of end-stage lung fibrosis, is seen in the right middle lobe (arrows). CT scan shows bilateral ground-glass opacity in a predominantly lower lung distribution. 5 Dept of Pathology and Laboratory Medicine (retired), Mayo Clinic, Scottsdale, AZ, USA. Table 3.5  IMAGING FEATURES OF IDIOPATHIC INTERSTITIAL PNEUMONIAS, Morphologic Pattern (Histologic and Radiologic), Basal and subpleural–predominant distribution, reticular opacities (often with honeycombing), traction bronchiectasis, and architectural distortion, Basal-predominant distribution, ground-glass and reticular opacities, Basal and lower lung–predominant distribution, ground-glass opacities, sometimes with cysts, Respiratory bronchiolitis (clinical diagnosis of RB-ILD), Centrilobular distribution, ground-glass opacity, typically nodular, Organizing pneumonia (clinical diagnosis of COP), Basal and subpleural–predominant distribution, ground-glass opacity, and consolidation; bronchovascular distribution is also common, Diffuse alveolar damage (clinical diagnosis of AIP), Diffuse ground-glass opacity and consolidation, Bronchovascular distribution is common, ground-glass and reticular opacities and perivascular cysts. Typical CT features of early-stage AIP are ground-glass opacity, bronchiolar dilatation, and dense airspace opacity. Findings usually improve with steroid treatment. Other causes of Kerley lines are listed in Table 3.2. The presence of honeycombing as a predominant imaging finding is highly specific for UIP and can be used to differentiate it from NSIP, particularly when the distribution is patchy and subpleural-predominant (5). The average rate of progression of honeycombing in patients with idiopathic usual interstitial pneumonia according to one study was 0.4% of lung volume per month 7. CT scan shows multiple circumscribed, round pulmonary nodules, 2 to 3 mm in diameter, and scattered reticular opacities. In RB-ILD, the findings are more extensive (Fig. Still, our … 1. As capillary pressure rises and interstitial pressure increases, water is forced into the alveolar spaces through the alveolar–capillary membrane; therefore, edema is often seen as a combination of both interstitial and alveolar opacities on the chest radiograph. Wells AU. The spatial and temporal homogeneity of this pattern is important in distinguishing NSIP from UIP. The CT features of patients with asymptomatic respiratory bronchiolitis show ground-glass centrilobular nodules and patchy areas of ground-glass opacity (Fig. The list of diagnostic possibilities to consider when this pattern is seen can be shortened by taking into account the acuity of the disease, the distribution of disease, and associated radiographic abnormalities. A: PA chest radiograph shows low lung volumes and bibasilar reticular ILD. Radiology. Wells AU. 1996;110 (2): 378-82. FIG. 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. Integrative Imaging Self-Assessment Modules LIFELONG LEARNING FOR RADIOLOGY Review. AJR. The prognosis of NSIP is substantially better than that of UIP. 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. Heart … In contrast, the role of radiology in the diagnosis and treatment of occupational lung disease appears at first glance to have changed very little. 6. The imaging features of RB-ILD may be similar to those of hypersensitivity pneumonitis and NSIP. 3.24 • Influenza pneumonia. Introduction: Using real-world Japanese postmarketing data, we characterized interstitial lung disease (ILD) development during the second- or later-line osimertinib treatment for EGFR mutation-positive NSCLC. 4. A key imaging differential on cross-sectional imaging would be: ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. A nodular pattern, especially with an upper lung–predominant distribution, suggests a specific differential diagnosis (Table 3.3; Figs. The Clinical Utility of Bronchoalveolar Lavage Cellular Analysis In Interstitial Lung Disease: An Official ATS Clinical Practice Guideline (2012) - Online Supplement; Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management: An Official ATS/ERS/JRS/ALAT Statement (2011) NIH/NHLBI Notice re: 3-drug Regimen for IPF (2011) The American Thoracic … Lung damage from ILDs is often irreversible and gets worse over time. A reticulonodular pattern results from a combination of reticular and nodular opacities, or it can appear when reticular opacities are seen end-on. An uncommon pattern of edema is more common than an uncommon cause of ILD. 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. B: CT scan shows basilar subpleural ground-glass opacity and mild airway dilatation. 1 Presentation is often insidious and indistinguishable from the underlying disease process being treated by the drug. Interstitial lung disease is the generic term for a group of almost 200 different types of diseases with different causes but with similar clinical and pathological changes. Patients invariably present with dyspnea of varying time course and severity. Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema: thin-section CT findings. 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. Dr. Sally Suliman presents "Interstitial Lung Disease" by first introducing the classification of ILDs. 21. 3.1). The latter include idiopathic interstitial pneumonias, granulomatous lung disorders (e.g., sarcoidosis), and other forms of ILD including lymphangioleiomyomatosis (LAM), Langerhans cell histiocytosis (LCH), and eosinophilic pneumonia (1). List the common causes of lower lobe–predominant ILD (idiopathic pulmonary fibrosis, asbestosis, chronic aspiration, collagen vascular disease). 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. idiopathic pulmonary fibrosis). Interstitial lung disease is much more likely to affect adults, although infants and children sometimes develop the disorder. 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?? Introduction. Res. Similar to the pathology specimen, cross-sectional imaging also reveals heterogeneity, with patchy areas of fibrosis alternating with areas of normal lung 5. 3.21 and 3.22). Proc Am Thorac Soc. 2014;23 (133): 308-19. 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. 2006;3 (4): 285-92. 2Pulmonary Unit, GB Morgagni Hospital, Forlı`, Italy. Department of Radiology Seattle Children’s 4800 Sand Point Way NE, R-5417 Seattle, WA 98105-3071 206-987-2166 206-987-2143 (fax) 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. These septal lines were first described by Kerley in patients with pulmonary edema (3). The differential diagnosis of COP includes adenocarcinoma in situ (formerly BAC), lymphoma, vasculitis, sarcoidosis, chronic eosinophilic pneumonia, and infectious pneumonia. Drug-induced interstitial lung disease (DI-ILD), defined as an interstitial abnormality secondary to administration of a drug, can be acute or chronic. Kerley lines help limit the differential diagnosis (see Table 3.2). Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. 6 (2): 138-153. CT scan shows subpleural, dense airspace opacity in the left lung. Respir. Distinction of UIP from other IIPs is important, because UIP is associated with a poorer prognosis than the other entities. Ground-glass opacities are present in about 60% of cases. Identifying and determining the cause of interstitial lung disease can be challenging. No etiologic agent is identified. HRCT performed (A) before and (B) after clinical deterioration in a patient with biopsy proven usual interstitial pneumonia. 3.19). List two causes of upper lobe–predominant ILD (chronic hypersensitivity pneumonitis, sarcoidosis). FIG. Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate. Res. Viruses that cause pneumonia in immunocompromised patients include Cytomegalovirus, varicella-zoster, and herpesvirus. 2. “Cystic” areas represent pulmonary emphysema. Mechanical ventilation is usually required. Jonathan H. Chung, Christian W. Cox, Steven M. Montner, et al. ; We fund research. 3.1 • Diagrams illustrating the four types of ILD. A more complete list can be found in the medical literature (8). 17. Kerley B lines are short, straight lines (1 to 2 cm) perpendicular to and abutting the lower lateral pleural edge. 5. The typical CT findings are ground-glass and reticular opacities, sometimes associated with perivascular cysts (Fig. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. ■ Describe application of secondary lo… 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. 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 … This article will focus solely on the usual interstitial pneumonia pattern as a radiological or histopathological descriptor, for further discussion in the clinical aspects, please refer to the parental article on the specific underlying clinical diagnosis (e.g. The chest radiograph on the left shows how difficult it can be to identify early interstitial lung disease – it looks normal however the images from the patient’s CT (performed in the prone position) show subpleural, basal-predominant reticular opacities. In recent times some authors have suggested certain signs within a UIP pattern more suggestive of it being due to connective tissue disorder interstitial lung disease over IPF 22. B: Reticular ILD is seen as a network of curvilinear opacities. 3.23). This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). Chest 2005; 127: 178–184. CT scan shows numerous tiny nodules in a random distribution. In the past, the term usual interstitial pneumonia was used synonymously with IPF. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?. Disease progression in usual interstitial pneumonia compared with desquamative interstitial pneumonia. Chung JH, Lynch DA. 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. Both A and B lines are seen as a result of interlobular septal thickening, most commonly from pulmonary edema. Patients with COP typically present with cough and dyspnea of relatively short duration. Adult patients with newly detected interstitial lung disease (ILD) of apparently unknown cause are clinically suspected of having idiopathic pulmonary fibrosis(IPF) if they have unexplained symptomatic or asymptomatic patterns of bilateral fibrosis on a chest FIG. A reticular pattern results from the summation or superimposition of irregular linear opacities. Kerley A lines radiate out from the hila to the periphery of the lung. Temporal heterogeneity is an important histologic feature and helps to distinguish UIP from DIP. Lung volumes are decreased. Overview of Interstitial Lung Disease (ILD) Interstitial lung disease is a broad term for a number of diseases that lead to inflammation or scarring of the lungs, leading to fibrosis. Plain film features are non-specific. 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 a typical biopsy, there are areas of normal lung alternating with interstitial fibrosis and honeycombing. 3.13). Viruses are the major cause of respiratory tract infection in the community, especially in children. A reticulonodular pattern and larger-than-normal lung volumes can be seen with LAM and LCH. 2016;206 (3): . When seen as a result of a reversible process, such as viral pneumonia, sarcoidosis, or hypersensitivity pneumonitis, the distribution can be patchy or diffuse. 2Pulmonary Unit, GB Morgagni Hospital, Forlı`, Italy. Du bois R, King TE. 15. If you work in mining, farming or construction or for any reason are exposed to pollutants known to damage your lungs, your risk of interstitial lung disease is increased. The lower lungs are more frequently involved. The positive predictive value of CT in the diagnosis of UIP is high and ranges from 70-100% 1. 3.22 • Organizing pneumonia. Reticulation is frequently seen but is typically limited to the lung bases. Idiopathic pulmonary fibrosis: the radiologist’s role in making the diagnosis. 14 Departamento de Reumatologia, Hospital General Dr. Manuel Gea González, Ciudad de México, México. 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. CT scan shows multiple bilateral, round, pulmonary nodules. Interstitial lung disease (ILD) comprises a diverse group of lung diseases with overlapping clinical, radiological, physiological, and pathological features.1 Interstitial lung abnormalities (ILAs) refer to the presence of CT scan findings that are potentially compatible with ILD in patients who have partial (eg, abdominal CT including C: Close-up of (A), right upper lung, shows linear opacities (arrow) radiating outward from the hila, representing Kerley A lines. 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). This 45-year-old woman presented with metastatic gastric carcinoma. 3.7 • Miliary tuberculosis and nodular ILD. ■ 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. Make a specific diagnosis of ILD when supportive findings are present in the history or on radiologic imaging (e.g., dilated esophagus and ILD in scleroderma; enlarged heart, pacemaker or defibrillator, prior sternotomy, and ILD in a patient with amiodarone drug toxicity). 10Division of Rheumatology, ... Society “Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease”. 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. Nonspecific interstitial pneumonia (NSIP) is the next most frequent. 3 Interstitial Lung Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College, London, UK. Emphysema Patients with IPF are at high risk for having emphysema,11 which carries a significantly poorer outcome than IPF alone.5 Emphysema and pulmonary fibrosis have opposing physiologic effects, often leading to apparent conserved lung function during pulmonary function tests.12 Therefore, recognition of coexistent fibrosis … FIG. 5 27 InterstIal lung dIseases Dr. Shrikant Nagare 2. B: CT scan shows bilateral reticular and ground-glass opacities and areas of consolidation. Eur Respir Rev. FIG. Identifying and determining the cause of interstitial lung disease can be challenging. Kim EA, Lee KS, Johkoh T et-al. CT scan shows bilateral patchy ground-glass opacities in a bronchovascular distribution. 2011;140 (4 Meeting Abstracts): . It may occur when an injury to the … After completing this journal-based SA-CME activity, participants will be able to: 1. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. Diffuse lung disease is a term often used to describe a group of disorders of known cause (e.g., collagen vascular disease, environmental or drug-related) as well as disorders of unknown cause. Honeycombing, particularly if it involves more than 5% of the lung volume, is an almost 100% specific finding. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease–Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. American journal of roentgenology. (2019) The British Journal of Radiology. (2017) American Journal of Roentgenology. A classic reticular pattern is seen with pulmonary fibrosis, in which multiple curvilinear opacities form small cystic spaces along the pleural margins and lung bases (honeycomb lung) (Fig. 12. Interstitial lung disease describes a large group of lung disorders which cause progressive scarring of lung … Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. 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). 3.3 • Cardiogenic edema and Kerley lines. Interstitial Lung Disease and Emphysema. These patterns are more accurately and specifically defined on CT. A linear pattern is seen when there is thickening of the interlobular septa, producing Kerley lines. Associated pleural thickening and/or calcification suggest asbestosis. 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. (2016) AJR. These are due to chronic, nonmalignant, noninfectious diseases of the lower respiratory tract that cause disruption and inflammation to the walls of the alveoli. 3.11 • Cardiogenic pulmonary edema. 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. Respiratory bronchiolitis is a histopathologic lesion found in cigarette smokers and is characterized by the presence of pigmented intraluminal macrophages within respiratory bronchioles (4). Usually, due to the more extensive involvement of the lower lobes, the major fissure is shifted inferiorly which is best seen on the lateral chest radiograph. Founded in 1905 to combat TB, the ATS has grown to tackle asthma, COPD, lung cancer, sepsis, acute respiratory distress, and sleep apnea, among other diseases. 2014;14 Suppl 1: S2. A pattern- Neither alveoli nor interstitium is visible on a chest X-ray when normal. FIG. 3.20) but are at least partially reversible in patients who stop smoking. The novelty of the new … 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. Of Connective Tissue disorders ( particularly Sjögren syndrome ), representing Kerley lines reader ”,. 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