In a previous analysis using Vigibase data, ILD was found to be one of the most outstanding features of Japanese ADR reporting compared with other countries [].Over-representation of Japanese reporting of ILD has previously been described by others [4, 5]. Fungal disease is discussed in Chapter 7. NSIP is characterized histologically by spatially homogeneous alveolar wall thickening caused by inflammation, fibrosis, or both. 3.18 • Desquamative interstitial pneumonia (DIP). 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. B: CT scan shows basilar subpleural ground-glass opacity and mild airway dilatation. Functional respiratory tests commonly show an abnormal restrictive pattern and reduced diffusing capacity. No etiologic agent is identified. Table 3.2  DIFFERENTIAL DIAGNOSIS OF KERLEY LINES. 3. Consolidation is uncommon and honeycombing is rare. It is the world’s largest CME collection for lung diseases and treatment offering high quality e-learning and teaching resources for respiratory specialists. Histopathologic Patterns 19 . Typical CT features of each IIP are distinct, but there is overlap (Table 3.5). Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. 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. D: CT shows interlobular septal thickening (arrows), representing Kerley lines. 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. Viruses are the major cause of respiratory tract infection in the community, especially in children. Four major CT patterns were identified: usual interstitial pneumonia (n = 26), nonspecific interstitial pneumonia (n = 19), bronchiolitis (n = 11), and organizing pneumonia (n = 5). B: Reticular ILD is seen as a network of curvilinear opacities. 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) []. A: PA chest radiograph shows an enlarged cardiac silhouette and bilateral reticular and linear ILD. In rare cases, patients who are heavy smokers may develop RB-ILD, a condition characterized by pulmonary symptoms, abnormal pulmonary function, and imaging abnormalities, with respiratory bronchiolitis being the only histologic lesion identified on lung biopsy. 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. 3.13). E: Reticulonodular ILD results from a combination of reticular and nodular opacities, or it can be caused by reticular opacities seen end-on. FIG. Describe what a “B reader” is, as related to the evaluation of pneumoconioses. Table 3.3  DIFFERENTIAL DIAGNOSIS OF A NODULAR PATTERN OF INTERSTITIAL LUNG DISEASE, Pneumoconioses (silicosis, coal worker’s, berylliosis). Radiographics. This patient presented with acute shortness of breath and nonproductive cough. The reported prevalence of pulmonary toxicity in patients receiving amiodarone is ~10% (range 2-18%) 8. These septal lines were first described by Kerley in patients with pulmonary edema (3). Respiratory bronchiolitis is a histopathologic lesion found in cigarette smokers and is characterized by the presence of pigmented intraluminal macrophages within respiratory bronchioles (4). 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). 3.6 • Hematogenous metastases and nodular ILD. 3.1 • Diagrams illustrating the four types of ILD. The classification is based on histologic criteria, although the diagnosis of IIP is made by correlating the clinical, imaging, and pathologic features. 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. 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. FIG. The spectrum of conditions included is broad, ranging from occasional self-limited inflammatory processes to severe debilitating fibrosis of the lungs. 3.22 • Organizing pneumonia. 3.16). 3.24 • Influenza pneumonia. 8. The architecture of the lung is preserved. FIG. Identify honeycombing on a chest radiograph and CT scan, state the significance of this finding (end-stage lung disease), and list the common causes of honeycomb lung. B: CT scan shows bilateral reticular and ground-glass opacities and areas of consolidation. The typical CT findings are ground-glass and reticular opacities, sometimes associated with perivascular cysts (Fig. This previously healthy man living in the upper midwestern part of the United States presented with mild symptoms of shortness of breath and cough. This patient had a history of emphysema and acute respiratory symptoms. Kim EA, Lee KS, Johkoh T et-al. 6. It is usually asymptomatic. FIG. The term AIP is reserved for diffuse alveolar damage of unknown origin. The typical CT feature of NSIP is predominantly basilar ground-glass and reticular opacities (Fig. Quantification of Diffuse Parenchyma Lung Disease (DPLD) patterns challenges Computer Aided Diagnosis schemes in Computed Tomography (CT) lung analysis. Interstitial lung disease (ILD) includes a large group of pulmonary disorders that result in scarring or fibrosis of the lung parenchyma. Unable to process the form. The morphologic, or “pattern,” of each IIP seen at histologic or CT examination is linked to a specific clinical syndrome. 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. The curvilinear opacities form small cystic spaces (forming the honeycomb) in a characteristic bibasilar and subpleural distribution. The 4 CT Patterns of Pulmonary Disease . The typical CT features of UIP are a predominantly basal and subpleural reticular interstitial pattern with honeycombing and traction bronchiectasis (Fig. Other causes of Kerley lines are listed in Table 3.2. 2. Diffuse alveolar damage is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. C: When reticular ILD is seen as a result of chronic, irreversible lung disease, such as usual interstitial pneumonia, honeycombing is seen. List two causes of upper lobe–predominant ILD (chronic hypersensitivity pneumonitis, sarcoidosis). 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. The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. Patients with NSIP are more commonly female and generally have a younger mean age than patients with UIP. Clinical evaluation must prove that an interstitial pneumonia is idiopathic and exclude a recognizable cause (e.g., collagen vascular disease). 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. This pattern is often difficult to distinguish from a purely reticular or nodular pattern, and in such a case a differential diagnosis should be developed on the basis of the predominant pattern. (2015) BMJ (Clinical research ed.). 3.19 • Respiratory bronchiolitis. Respiratory bronchiolitis–interstitial lung disease 234. and 235. These characteristic opacity changes are called lung patterns. T1 - Patterns of interstitial lung disease and mortality in rheumatoid arthritis. 3.2 and 3.3). Author information: (1)Department of Computer Science and Engineering, National Institute of Technology Durgapur, Durgapur, 713209, India. A reticular pattern results from the summation or superimposition of irregular linear opacities. This 45-year-old woman presented with metastatic gastric carcinoma. 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. Infectious pneumonia resulting in a diffuse interstitial pattern is unusual; however, viral, fungal, mycobacterial, and Mycoplasma pneumonias may be predominantly interstitial or interstitial-appearing. Table 3.4  PULMONARY EDEMA WITH A NORMAL-SIZED HEART. FIG. 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. CT scan shows numerous Kerley B lines (short arrows), thickening of the right major fissure from subpleural edema (arrowheads), patchy areas of ground-glass opacification (long arrows), and a right pleural effusion (curved arrows). FIG. A lower lung–predominant distribution with decreased lung volumes suggests idiopathic pulmonary fibrosis, asbestosis, collagen vascular disease, or chronic aspiration. IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. The disease was found to be bilateral in 15 patients. Please refer to the articles in each specific etiology listed above for specific details on their imaging pattern. 1. 5. Accurate classification of these patterns plays a significant role in precise clinical decision making of the extent and nature of the diseases. Findings usually improve with steroid treatment. Mechanical ventilation is usually required. Lung parenchymal involvement in IBD can be due to nonspecific infection, while true IBD-related interstitial lung disease is rare [8, 10, 12]. 3.20 • Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD). In adults, LIP is commonly associated with connective tissue disorders (particularly Sjögren syndrome), immunodeficiency syndromes, and Castleman syndrome. CT scan of a patient living in Arizona shows numerous small nodules in a random distribution and pleural effusions. 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. 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. Interstitial lung disease may arise in the context of an established connective tissue disease or be the initial manifestation of an otherwise occult autoimmune disorder. FIG. 3.20) but are at least partially reversible in patients who stop smoking. 3.12). This 69-year-old woman presented with left ventricular failure and a predominantly interstitial pattern of pulmonary edema. 7. 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. In the 24 patients with CT indeterminate for UIP, CTD-ILD was the final diagnosis in 33%, followed by desquamative interstitial pneumonia (21%), and IPF A more complete list can be found in the medical literature (8). CT scan shows bilateral reticular and ground-glass opacities in a predominantly upper lung distribution. The radiographic appearance of viral pneumonias is typically a diffuse interstitial pattern with a diffuse, patchy, often nodular appearance (Fig. Identify Kerley A and B lines on a chest radiograph and CT scan, and explain their etiology and significance. Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases. 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 Thin-section CT findings in rheumatoid arthritis-associated lung disease: CT patterns and their courses J Comput Assist Tomogr. Thin-section CT is a noninvasive technique for monitoring disease morphology in RA-associated lung disease. Temporal heterogeneity is an important histologic feature and helps to distinguish UIP from DIP. It may occur when an injury to the lungs triggers an abnormal healing response. Radiographics. FIG. Alveolar filling 5. Most patients improve with cessation of smoking and oral corticosteroids. 9. Chest 2005; 127: 178–184. Most diseases result in an increase in opacity, usually characteristic for the pulmonary parenchymal component (alveolar, bronchial, interstitial, vascular) involved. CT scan shows numerous tiny nodules in a random distribution. A: PA chest radiograph shows low lung volumes and bibasilar reticular ILD. B: Close-up view of (A), lower right lung, shows short, linear opacities perpendicular to the lateral pleural edge, representing Kerley B lines. Chest radiographs may show widespread bilateral nodular or reticular opacities, and they may take several weeks to return to normal. Patients with AIP present with respiratory failure developing over days or weeks. 2. 3.24). Associated lymphadenopathy suggests sarcoidosis; neoplasm (lymphangitic carcinomatosis, lymphoma, metastases); infection (viral, mycobacterial, or fungal); and silicosis. Development of Interstitial Lung Disease (ILD) in Patients With Severe SARS-CoV-2 Infection (COVID-19) (CovILD) ... the similar clinical course in severe cases and overlapping CT patterns in the acute setting, persistent radiological and pulmonary functional changes in survivors are conceivable. Mueller-mang C, Grosse C, Schmid K et-al. 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). Idiopathic LIP is rare. The parenchymal abnormalities of NSIP may be reversible on follow-up CT scanning. The nodules generally range from 1 to 10 mm in size. Lung Pattern Classification for Interstitial Lung Diseases Using a Deep Convolutional Neural Network ... To train and evaluate the CNN, we used a dataset of 14696 image patches, derived by 120 CT scans from different scanners and hospitals.