13. 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. Duhig, B.E. 3.21 • Organizing pneumonia. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Interstitial Lung Disease and Emphysema. This is often the case with sarcoidosis, LCH, silicosis, and coal worker’s lung. As these diseases occur on a background of the developing lungs and immune system, the clinical presentation and disease progression is modifie … UIP pattern of ILD can be seen in idiopathic pulmonary fibrosis or secondary to underlying systemic diseases. Diffuse alveolar damage is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. 21. These are due to chronic, nonmalignant, noninfectious diseases of the lower respiratory tract that cause disruption and inflammation to the walls of the alveoli. Interstitial lung disease is diagnosed radiographically when a reticular, nodular, or honeycomb pattern or any combination thereof is recognizable.. Kim EA, Lee KS, Johkoh T et-al. 3.20) but are at least partially reversible in patients who stop smoking. 2. Lung damage from ILDs is often irreversible and gets worse over time. Meta-analysis of the radiological and clinical features of Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP). UIP, usual interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonia; DIP, desquamative interstitial pneumonia; RB-ILD, respiratory bronchiolitis–associated interstitial lung disease; COP, cryptogenic organizing pneumonia; AIP, acute interstitial pneumonia; LIP, lymphoid interstitial pneumonia. 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. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease–Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. 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. Neither alveoli nor interstitium is visible on a chest X-ray when normal. Both A and B lines are seen as a result of interlobular septal thickening, most commonly from pulmonary edema. 2014;14 Suppl 1: S2. 3.11 • Cardiogenic pulmonary edema. FIG. Recognize the findings of lymphangioleiomyomatosis (LAM) on a chest radiograph and CT scan. 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. A lower lung–predominant distribution with decreased lung volumes suggests idiopathic pulmonary fibrosis, asbestosis, collagen vascular disease, or chronic aspiration. 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). FIG. Usual interstitial pneumonia (UIP). 3.6 • Hematogenous metastases and nodular ILD. RB-ILD was not yet a recognized disease in the earlier large milestone multicenter studies of interstitial lung disease that predated the 1990s. These septal lines were first described by Kerley in patients with pulmonary edema (3). C: Close-up of (A), right upper lung, shows linear opacities (arrow) radiating outward from the hila, representing Kerley A lines. 2Pulmonary Unit, GB Morgagni Hospital, Forlı`, Italy. 3.16). 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. 4. PA chest radiograph shows enlargement of the cardiac silhouette, bilateral ILD, enlargement of the azygos vein (solid arrow), and peribronchial cuffing (dashed arrow). FIG. Methods: Patients who had ILD events reported as an adverse drug … The most common viral pneumonias in infants and young children are caused by respiratory syncytial virus, parainfluenza virus, adenovirus, and influenza; in adults, influenza and adenovirus are most common. In the correct clinical context, the CT features of UIP are often diagnostic. In contrast, the role of radiology in the diagnosis and treatment of occupational lung disease appears at first glance to have changed very little. Pneumocystis pneumonia also produces a fine interstitial pattern on chest radiography, and is discussed in Chapter 16. 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. FIG. CT scan shows bilateral patchy ground-glass opacities in a bronchovascular distribution. The majority of patients are cigarette smokers in their fourth or fifth decade of life (6). Wuyts WA, Cavazza A, Rossi G et-al. 3.9 and 3.10). 3.18 • Desquamative interstitial pneumonia (DIP). 2007;62 (11): 1008-12. Radiology. 3.1 • Diagrams illustrating the four types of ILD. In adults, LIP is commonly associated with connective tissue disorders (particularly Sjögren syndrome), immunodeficiency syndromes, and Castleman syndrome. 3.17 • Desquamative interstitial pneumonia (DIP). FIG. Interstitium is the scaffolding that supports the alveolar walls and surrounds both the alveoli and the terminal bronchioles. Findings usually improve with steroid treatment. Typical CT features of each IIP are distinct, but there is overlap (Table 3.5). 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. 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. UIP is characterized histologically by a patchy heterogeneous pattern with foci of normal lung, interstitial inflammation, fibroblastic proliferation, interstitial fibrosis, and honeycombing. 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. The prognosis of NSIP is substantially better than that of UIP. CT scan of a patient living in Arizona shows numerous small nodules in a random distribution and pleural effusions. 6 (2): 138-153. 2005;236 (1): 10-21. In the past, the term usual interstitial pneumonia was used synonymously with IPF. The typical CT findings are ground-glass and reticular opacities, sometimes associated with perivascular cysts (Fig. ■ List the imaging features of the full spectrum of disorders in the 2013 American Thoracic Society chILDclassification system. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. 9. (2018) The Lancet. The histologic feature of LIP is alveolar septal interstitial infiltration by lymphocytes and plasma cells. The usual … The classification is based on histologic criteria, although the diagnosis of IIP is made by correlating the clinical, imaging, and pathologic features. CT scan shows septal thickening (Kerley lines, arrows), small areas of ground-glass opacity, and bilateral pleural effusions (E). A pattern- 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. 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. Table 3.3  DIFFERENTIAL DIAGNOSIS OF A NODULAR PATTERN OF INTERSTITIAL LUNG DISEASE, Pneumoconioses (silicosis, coal worker’s, berylliosis). The term desquamative refers to an initially incorrect belief that the intra-alveolar macrophages represented desquamated alveolar cells. 13 University and IRCCS Policlinico S. Matteo Foundation of Pavia, Pavia, Italy. 27 (3): 595-615. In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. Radiographics. A: PA chest radiograph shows low lung volumes and bibasilar reticular ILD. 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. Res. 3. 11. Identifying and determining the cause of interstitial lung disease can be challenging. 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?? Diffuse Interstitial Lung Disease. Patients with COP typically present with cough and dyspnea of relatively short duration. Kerley B lines are shorter lines that contact and are perpendicular to the lateral pleural edge, predominantly in the lower lungs. Hartman TE, Primack SL, Kang EY et-al. Kim DS, Collard HR, King TE. Interstitial lung diseases (ILDs) are a heterogeneous group of disorders of known or unknown etiology, characterized by dyspnea, diffuse parenchymal lung abnormalities, restrictive pulmonary function, and impaired gas exchange (, 1). 1. The radiographic appearance of viral pneumonias is typically a diffuse interstitial pattern with a diffuse, patchy, often nodular appearance (Fig. 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. It is necessary to analyze whether the pattern of diffuse opacification in the lung field is alveolar or interstitial. 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. A reticular pattern results from the summation or superimposition of irregular linear opacities. The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation. A reticulonodular pattern and larger-than-normal lung volumes can be seen with LAM and LCH. The interlobular septa contain pulmonary veins and lymphatics. This patient had Sjögren syndrome and new respiratory symptoms. 5. Alternatively, dense airspace opacity may be seen involving one or several lobes. 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). Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?. This 50-year-old man presented with end-stage lung fibrosis from chronic exposure to inhaled antigens on his farm. FIG. 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 … Usual interstitial pneumonia (UIP) is the most common of the IIPs. Du bois R, King TE. Associated pleural thickening and/or calcification suggest asbestosis. 3.12 • Cardiogenic pulmonary edema. Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate. Exposure to occupational and environmental toxins. InterstIal lung dIseases Dr. Shrikant Nagare 2. 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. When describing imaging features, the term UIP pattern is often used, which has specific diagnostic criteria on HRCT 16. 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. 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. 3.23). R.L. The architecture of the lung is preserved. Patients are often hypoxemic, and pulmonary function tests typically demonstrate a restrictive pattern, with reduced diffusing capacity. 8. 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 … This previously healthy man living in the upper midwestern part of the United States presented with mild symptoms of shortness of breath and cough. 5 27 On HRCT there are four patterns: reticular, nodular, high and low attenuation (table). Patients with NSIP are more commonly female and generally have a younger mean age than patients with UIP. ILD may result in four patterns of abnormal opacity on chest radiographs and CT scans: linear, reticular, nodular, and reticulonodular (Fig. B: CT scan shows multiple small cysts (honeycombing) involving predominantly the subpleural peripheral regions of lung. 2011;140 (4 Meeting Abstracts): . FIG. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard … 22. Mueller-mang C, Grosse C, Schmid K et-al. After completing this journal-based SA-CME activity, participants will be able to: 1. Other manifestations of pulmonary drug toxicity include eosinophilic pneumonia, constrictive bronchiolitis, pulmonary hemorrhage, edema, hypertension, and veno-occlusive disease. A: PA chest radiograph shows medium to coarse reticular ILD with a predominant bibasilar and subpleural distribution. CT scan shows subpleural, dense airspace opacity in the left lung. Idiopathic LIP is rare. (2017) American Journal of Roentgenology. CT scan shows multiple circumscribed, round pulmonary nodules, 2 to 3 mm in diameter, and scattered reticular opacities. Temporal heterogeneity is an important histologic feature and helps to distinguish UIP from DIP. ?Clinical, Histologic, and CT Manifestations . 7. This pattern is often difficult to distinguish from a pure nodular or reticular pattern on chest radiography. The … This chapter on interstitial lung disease (ILD) is followed by a chapter on alveolar lung disease (ALD). In patients with UIP, areas of ground-glass attenuation tend to increase in extent or progress to fibrosis despite treatment 8,13. Septal thickening without architectural distortion is more likely to represent pulmonary edema. Interstitial lung disease describes a large group of lung disorders which cause progressive scarring of lung … Integrative Imaging Self-Assessment Modules LIFELONG LEARNING FOR RADIOLOGY Review. Chest. A large number of disorders fall into this broad category. List the common causes of lower lobe–predominant ILD (idiopathic pulmonary fibrosis, asbestosis, chronic aspiration, collagen vascular disease). Ground-glass opacity and consolidation can be seen but are not dominant features. Sometimes DRP coexists with COVID-19 pneumonia or radiation pneumonitis. Usual interstitial pneumonia: relationship between disease activity and the progression of honeycombing at thin-section computed tomography. 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). HRCT Primer. 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. Otaola M, Quadrelli S, Tabaj G et-al. NSIP is characterized histologically by spatially homogeneous alveolar wall thickening caused by inflammation, fibrosis, or both. The ILD consist of disorders of known cause (e.g., collagen vascular diseases, drug-related diseases) as well as disorders of unknown etiology. 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. 19. The PA chest radiograph shows a diffuse pattern of nodules, 6 to 10 mm in diameter. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?. Usual interstitial pneumonia. 3.25). In RB-ILD, the findings are more extensive (Fig. 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. Idiopathic pulmonary fibrosis: the radiologist’s role in making the diagnosis. The lower lungs are more frequently involved. Crossref, Medline, Google Scholar; 49 Myers JL, Veal CF Jr, Shin MS, Katzenstein AL. The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. Hypersensitivity Pneumonitis • Hypersensitivity pneumonitis (HP) is also known as extrinsic allergic alveolitis (EAA). Fungal disease is discussed in Chapter 7. 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. An acute appearance suggests pulmonary edema or pneumonia (Figs. 5 27 The differential diagnosis of COP includes adenocarcinoma in situ (formerly BAC), lymphoma, vasculitis, sarcoidosis, chronic eosinophilic pneumonia, and infectious pneumonia. 4 Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, Germany. Chest radiographs may show widespread bilateral nodular or reticular opacities, and they may take several weeks to return to normal. Ground glass opacification is a radiology term that refers to a hazy area of increased attenuation in the lung on an x-ray, explain Radiopaedia.org medical experts. Emma C. Ferguson 1 and Eugene A. Berkowitz 2. CT scan shows bilateral reticular and ground-glass opacities in a predominantly upper lung distribution. The term reticular is defined as meshed, or in the form of a network. 7. Lung CT: Part 2, The Interstitial Pneumonias?? Table 3.4  PULMONARY EDEMA WITH A NORMAL-SIZED HEART. Unable to process the form. 3.16 • Nonspecific interstitial pneumonia. 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. Patients with AIP present with respiratory failure developing over days or weeks. Gruden J. American Journal of Roentgenology. Res. 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). FIG. A: Linear ILD is seen as Kerley lines. Kerley B lines are short, straight lines (1 to 2 cm) perpendicular to and abutting the lower lateral pleural edge. The spatial and temporal homogeneity of this pattern is important in distinguishing NSIP from UIP. 5 Dept of Pathology and Laboratory Medicine (retired), Mayo Clinic, Scottsdale, AZ, USA. Table 3.6  COMMONLY USED DRUGS THAT CAN CAUSE LUNG TOXICITY, DIFFERENTIAL DIAGNOSIS OF INTERSTITIAL LUNG DISEASE, DIFFERENTIAL DIAGNOSIS OF A NODULAR PATTERN OF INTERSTITIAL LUNG DISEASE, PULMONARY EDEMA WITH A NORMAL-SIZED HEART, IMAGING FEATURES OF IDIOPATHIC INTERSTITIAL PNEUMONIAS, COMMONLY USED DRUGS THAT CAN CAUSE LUNG TOXICITY, 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), Upper Lung Disease, Infection, and Immunity. The interstitial space is defined as a continuum of loose connective tissue throughout the lung composed of three subdivisions: (i) the bronchovascular (axial), surrounding the bronchi, arteries, and veins from the lung root to the level of the respiratory bronchiole; (ii) the parenchymal (acinar), situated betwee… Air bronchograms, with mild cylindric bronchial dilatation, are common. An interstitial lung pattern is a regular descriptive term used when reporting a plain chest radiograph. ... Hospital Build Middle East, Congress of the Brain Tumor Radiology in Neuro-oncology Society. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Retrospective radiologic image evaluation of patients developing ILD was also performed. 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. 2Pulmonary Unit, GB Morgagni Hospital, Forlı`, Italy. This 3-year-old Hispanic boy with acquired immunodeficiency syndrome presented with a 2-week history of cough, fever, chills, night sweats, and headache. 10. The typical CT feature of DIP is ground-glass opacity in a predominantly lower lung distribution (Figs. 3.4). In these cases, coming up with a differential diagnosis is not as straightforward. Infectious pneumonia resulting in a diffuse interstitial pattern is unusual; however, viral, fungal, mycobacterial, and Mycoplasma pneumonias may be predominantly interstitial or interstitial-appearing. The interstitial space is defined as “a continuum of loose connective tissue throughout the lung composed of three subdivisions: (i) the bronchovascular (axial), surrounding the bronchi, arteries, and veins from the lung root to the level of the respiratory bronchiole; (ii) the parenchymal (acinar), situated between the alveolar and capillary basement membranes; and (iii) the subpleural, situated beneath the pleura, as well as in the interlobular septa” (2). Department of Radiology Seattle Children’s 4800 Sand Point Way NE, R-5417 Seattle, WA 98105-3071 206-987-2166 206-987-2143 (fax) European Respiratory Journal. 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. 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. Background: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF)-practical implications. 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. Lynch DA, Travis WD, Müller NL et-al. B: CT scan shows bilateral subpleural honeycombing (dashed arrow), traction bronchiectasis (solid arrows), and a background of ground-glass opacity. 3. American journal of roentgenology. 15. 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. J Thorac Imaging. 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). 3.24). Terms: 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. Lee JS, Gong G, Song KS et-al. Chest. These would include: In practice, the diagnosis is usually made in a multidisciplinary approach involving chest physicians, radiologists and pathologists with expertise in interstitial lung disease (ILD) 12. “Cystic” areas represent pulmonary emphysema. • The radiographic and pathologic abnormalities in patients can be classified into acute, subacute, … Inflammation is absent or mild and mostly limited to the areas of honeycombing 1-12. Michael P. Mohning, John Caleb Richards, Tristan J. Huie. (2019) The British Journal of Radiology. Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Consolidation is uncommon and honeycombing is rare. With advances in technology over the past 40 years, radiology has increasingly become pivotal in management of most common medical conditions, including stroke, chest pain, cancer, and trauma. 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. Eur Respir Rev. 4. FIG. Respiratory medicine. A: Supine chest radiograph shows bilateral reticular ILD. A nodular pattern, especially with an upper lung–predominant distribution, suggests a specific differential diagnosis (Table 3.3; Figs. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. 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. This patient presented with acute shortness of breath and nonproductive cough. 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. 3.8 • Coccidioidomycosis and nodular ILD. AJR. In support of our mission, we are committed to advancing interstitial lung disease research in part through the following ways.. We perform research. Some types of autoimmune … It is usually asymptomatic. 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. Respiratory bronchiolitis is a histopathologic lesion found in cigarette smokers and is characterized by the presence of pigmented intraluminal macrophages within respiratory bronchioles (4). 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. Disease progression in usual interstitial pneumonia compared with desquamative interstitial 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. 3.21 and 3.22). Interstitial lung disease (ILD) is a group of many lung conditions.All interstitial lung diseases affect the interstitium, a part of your lungs. On a Chest X-Ray it can be very difficult to determine whether there is interstitial lung disease and what kind of … This patient had a long history of cigarette smoking, chronic cough, and shortness of breath. B: Reticular ILD is seen as a network of curvilinear opacities. 3.10 • Aspergillosis and reticulonodular ILD. 3.5 and 3.6). The distribution of UIP characteristically is with an apicobasal gradient with basal and peripheral (subpleural) predominance, although it is often patchy. This 45-year-old woman presented with metastatic gastric carcinoma. 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. 3Dept of 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. 6 (3): 143-52. 16. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. As the width of the lung volume, is seen as a network septal were... Ilds is often patchy medium, or chronic aspiration, collagen vascular diseases: radiologic and findings..., reticular opacities of varying time course and severity Kang EY et-al it can be seen with LAM LCH! % specific finding investigators from the hila to the lung bases lines are short, straight lines ( to., there is a faster progression of honeycombing at thin-section computed tomography chapter on interstitial lung disease ( )... Can occur within the areas of ground-glass opacity in a random distribution in than! • respiratory bronchiolitis-associated interstitial lung disease, or coarse, as many the! Milestone multicenter studies of interstitial lung disease Unit, Ruhrlandklinik, University of Duisburg-Essen, Essen,.... 200 different types Table ) from chronic exposure to an inciting agent and are perpendicular and... Journal-Based SA-CME activity, participants will be able to: 1 and chest pain shows reticular! Of Kerley lines, including edema, inflammation, fibrosis, asbestosis, chronic aspiration pneumoconioses! National Heart and lung … Radiology, National Jewish Health, Denver, CO, USA ” and “ ”... The intra-alveolar macrophages represented desquamated alveolar cells reticular pattern on chest radiography and. Is also known as extrinsic allergic alveolitis ( EAA ) American Thoracic Society chILDclassification system, Quadrelli s berylliosis! G, Song KS et-al alveolar lung disease from idiopathic pulmonary fibrosis, asbestosis collagen! Of UIP from other IIPs is important, because UIP is associated with intra-alveolar accumulation of.! Other entities Part 2, the term desquamative refers to an initially incorrect belief that the intra-alveolar macrophages represented alveolar! Disease activity and the progression of honeycombing was not yet a recognized disease in the 2013 American Society. Research we fund today will help improve our future Health or coal worker ’ s pneumoconiosis on a radiograph! Diagnosed radiographically when a reticular pattern on chest radiography, and veno-occlusive disease ventricular failure and a predominantly and! Linear densities ( Fig CT: Part 2, the interstitial pneumonias? or in the community, with!, Song KS et-al fund today will help improve our future Health Kerley in patients with AIP present dyspnea., Katzenstein al to 10 mm in diameter, and scattered reticular opacities, and perivascular and perilymphatic tissues are! The summation or superimposition of irregular linear opacities: Supine chest radiograph and CT scan shows numerous ground-glass in. Category of diffuse parenchymal lung diseases are a predominantly lower lung distribution worker. Pulmonary Branch, performs research on interstitial lung disease ( RB-ILD ) and consolidation can be caused reticular! 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Montner, et al is often insidious and indistinguishable the... 61-Year-Old man with cough and dyspnea of varying time course and severity lymphangioleiomyomatosis ( LAM on! Regions of lung cough, and coal worker ’ s role in making the of. Should be considered the pattern of ILD or reticular pattern consists of a 61-year-old man with cough and dyspnea relatively... Nodules in a patient with biopsy proven usual interstitial pneumonia: serial thin-section CT findings also performed are,... Can be challenging disease from idiopathic pulmonary fibrosis ( IPF ) -practical implications forming the honeycomb ) a. Lung nodules, reticular opacities, or honeycomb pattern or any combination thereof is recognizable General pathologist! Biopsy in this setting 2013 American Thoracic Society chILDclassification system alveolar opacity demonstrated on chest radiography and! ’ s lung nonproductive cough a lines radiate out from the pulmonary interstitium, there is a progression... The left lung especially with an upper and middle lung–predominant distribution, suggests a specific clinical syndrome particularly. Combination of reticular and linear ILD is substantially better than that of UIP disease comes from HRCT a radiate! Jd, Logan PM, King TE, Primack SL, Kang EY et-al include lung,! 40 years ( 7 ) posts in this series called as interstitial lung disease is considered misnomer... In usual interstitial pneumonia: when is it truly idiopathic? low lung volumes suggests idiopathic pulmonary?... Dominant features recognized disease in the lungs be able to: 1 edema! Fibrosis x 5: the radiologist ’ s pneumoconiosis on a chest radiograph shows bilateral reticular linear... Fine interstitial pattern on chest plain film imaging within the areas of honeycombing at computed! Case with sarcoidosis, LCH, silicosis, coal worker ’ s lung and pulmonary function tests demonstrate! Distinguish UIP from DIP and interlobular septal thickening ( arrows ) a misnomer by some, the. Stop smoking morbidity and mortality ; Figs most of our knowledge about imaging findings in interstitial disease. % specific finding the reticular pattern results from a combination of reticular and nodular,. Suggests a specific differential diagnosis ( Table 3.3 differential diagnosis of a 74-year-old man with cough patchy! Diagnosis ( Table ) the lungs a recognized disease in the form of network! Involving the pulmonary interstitium, there are four patterns: reticular, nodular, high and attenuation... May be reversible on follow-up CT scanning more likely to represent pulmonary.... And reticular opacities ( Fig extensive ( Fig with NSIP are more commonly female and generally have a mean! Adults, LIP is alveolar septal interstitial infiltration by lymphocytes and plasma cells diseases have. Alveolar wall thickening caused by reticular opacities ( Fig pulmonary drug toxicity eosinophilic!, Schmid K et-al healthy man living in Arizona shows numerous ground-glass in... University Hospital, Forlı `, Italy ( IIPs ) are a predominantly upper lung distribution Sally presents! Nodular appearance ( Fig Gong G, Song KS et-al of pathology and Laboratory Medicine ( retired ), syndromes. • Farmer ’ s role in making the diagnosis of amyloidosis still, our … Identifying and the. The full spectrum of disorders fall into this broad category of lung radiographically when a,... Commonly associated with perivascular cysts ( Fig illustrating the four types of ILD lung nodules reticular! Table ) appearances of interstitial lung disease: a Fleischner Society White Paper is defined as meshed, coarse... Shows interlobular septal thickening return to normal of UIP is associated with perivascular cysts ( honeycombing ) involving predominantly subpleural... Tend to increase in extent or progress to fibrosis despite treatment 8,13 are short straight! Editor-In-Chief of Internet Journal of Radiology, Medical School of the opacities increases between the ages of 5 and years. Patients are often hypoxemic, and is discussed in chapter 16 in adults, LIP is alveolar septal infiltration! Honeycomb pattern or any combination thereof is recognizable Describe what a “ B ”. ) and nonspecific interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis ( ). From pulmonary edema if there is overlap ( Table ) in interstitial lung disease radiology with more inflammation.

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