(BQ) Part 1 book Diagnostic imaging chest presents the following contents: Overview of chest imaging, developmental abnormalities, airway diseases, infections, pulmonary neoplasms, interstitial, diffuse and inhalational lung disease.
Diagnostic Imaging Chest Diagnostic Imaging Chest Table of Contents Authors 13 Dedication .14 Preface 14 Acknowledgements 15 Section - Overview of Chest Imaging 15 Introduction and Overview .15 Approach to Chest Imaging 15 Illustrated Terminology 23 Approach to Illustrated Terminology 23 Acinar Nodules 26 Air Bronchogram 27 Air-Trapping .29 Airspace .30 Architectural Distortion 31 Bulla/Bleb 33 Cavity 34 Centrilobular 36 Consolidation .37 Cyst 39 Ground-Glass Opacity 40 Honeycombing 42 Interlobular Septal Thickening 43 Intralobular Lines .45 Mass 46 Miliary Pattern 48 Mosaic Attenuation 49 Nodule .50 Perilymphatic Distribution 52 Pneumatocele 53 Reticular Pattern 55 Secondary Pulmonary Lobule 56 Traction Bronchiectasis .58 Tree-in-Bud Pattern 59 Chest Radiographic and CT Signs 61 Approach to Chest Radiographic and CT Signs .61 Air Crescent Sign 69 Cervicothoracic Sign 71 Comet Tail Sign 72 CT Halo Sign .73 Deep Sulcus Sign .75 Diagnostic Imaging Chest Fat Pad Sign 76 “Finger in Glove” Sign 77 Hilum Convergence Sign .79 Hilum Overlay Sign .80 Incomplete Border Sign 82 Luftsichel Sign 83 Reverse Halo Sign .84 Rigler Sign 86 S-Sign of Golden 87 Signet Ring Sign 88 Silhouette Sign 90 Atelectasis and Volume Loss 91 Approach to Atelectasis and Volume Loss 91 Right Upper Lobe Atelectasis 96 Middle Lobe Atelectasis 98 Right Lower Lobe Atelectasis 99 Left Upper Lobe Atelectasis 101 Left Lower Lobe Atelectasis 102 Complete Lung Atelectasis 104 Subsegmental Atelectasis 105 Relaxation and Compression Atelectasis 107 Rounded Atelectasis 108 Cicatricial Atelectasis 110 Section - Developmental Abnormalities 112 Introduction and Overview 112 Approach to Developmental Abnormalities 112 Airways 117 Tracheal Bronchus and Other Anomalous Bronchi 117 Paratracheal Air Cyst 123 Bronchial Atresia 126 Tracheobronchomegaly 132 Congenital Lobar Overinflation 135 Congenital Pulmonary Airway Malformation 138 Lung 141 Extralobar Sequestration 141 Intralobar Sequestration 144 Diffuse Pulmonary Lymphangiomatosis 150 Apical Lung Hernia 153 Pulmonary Circulation 156 Proximal Interruption of the Pulmonary Artery 156 Aberrant Left Pulmonary Artery 162 Pulmonary Arteriovenous Malformation 165 Diagnostic Imaging Chest Partial Anomalous Pulmonary Venous Return 168 Scimitar Syndrome 174 Pulmonary Varix 180 Systemic Circulation 183 Accessory Azygos Fissure 183 Azygos and Hemiazygos Continuation of the IVC 186 Persistent Left Superior Vena Cava 191 Aberrant Subclavian Artery 200 Right Aortic Arch 203 Double Aortic Arch 209 Aortic Coarctation 215 Cardiac, Pericardial, and Valvular Defects 221 Atrial Septal Defect 221 Ventricular Septal Defect 227 Bicuspid Aortic Valve 233 Pulmonic Stenosis 239 Heterotaxy 245 Absence of the Pericardium 251 Chest Wall & Diaphragm 254 Poland Syndrome 254 Pectus Deformity 258 Kyphoscoliosis 260 Morgagni Hernia 267 Bochdalek Hernia 270 Congenital Diaphragmatic Hernia 273 Section - Airway Diseases 276 Introduction and Overview 276 Approach to Airways Disease 276 Benign Neoplasms 281 Tracheobronchial Hamartoma 281 Tracheobronchial Papillomatosis 284 Malignant Neoplasms 290 Squamous Cell Carcinoma, Airways 290 Adenoid Cystic Carcinoma 293 Mucoepidermoid Carcinoma 296 Metastasis, Airways 300 Airway Narrowing and Wall Thickening 303 Saber-Sheath Trachea 303 Tracheal Stenosis 305 Tracheobronchomalacia 309 Middle Lobe Syndrome 314 Airway Wegener Granulomatosis 317 Diagnostic Imaging Chest Tracheobronchial Amyloidosis 320 Tracheobronchopathia Osteochondroplastica 323 Relapsing Polychondritis 326 Rhinoscleroma 329 Bronchial Dilatation and Impaction 332 Chronic Bronchitis 332 Bronchiectasis 335 Cystic Fibrosis 341 Allergic Bronchopulmonary Aspergillosis 347 Primary Ciliary Dyskinesia 353 Williams-Campbell Syndrome 359 Broncholithiasis 362 Emphysema and Small Airway Diseases 365 Centrilobular Emphysema 365 Paraseptal Emphysema 371 Panlobular Emphysema 374 Infectious Bronchiolitis 377 Constrictive Bronchiolitis 383 Swyer-James-McLeod 389 Asthma 392 Section - Infections 398 Introduction and Overview 398 Approach to Infections 398 General 403 Bronchopneumonia 403 Community-acquired Pneumonia 406 Healthcare-associated Pneumonia 412 Nosocomial Pneumonia 415 Lung Abscess 418 Septic Emboli 424 Bacteria 430 Pneumococcal Pneumonia 430 Staphylococcal Pneumonia 435 Klebsiella Pneumonia 441 Methicillin-resistant Staphylococcus aureus Pneumonia 445 Legionella Pneumonia 447 Nocardiosis 450 Actinomycosis 453 Mycobacteria and Mycoplasma 456 Tuberculosis 456 Nontuberculous Mycobacterial Infection 466 Mycoplasma Pneumonia 472 Diagnostic Imaging Chest Viruses 475 Influenza Pneumonia 475 Cytomegalovirus Pneumonia 478 Severe Acute Respiratory Syndrome 483 Fungi 486 Histoplasmosis 486 Coccidioidomycosis 492 Blastomycosis 496 Cryptococcosis 499 Aspergillosis 502 Zygomycosis 510 Pneumocystis jiroveci Pneumonia 513 Parasites 519 Dirofilariasis 519 Hydatidosis 522 Strongyloidiasis 525 Section - Pulmonary Neoplasms 529 Introduction and Overview 529 Approach to Pulmonary Neoplasms 529 Solitary Pulmonary Nodule 537 Lung Cancer 545 Adenocarcinoma 545 Squamous Cell Carcinoma 551 Small Cell Carcinoma 557 Mutlifocal Lung Cancer 563 Resectable Lung Cancer 566 Unresectable Lung Cancer 571 Uncommon Neoplasms 577 Pulmonary Hamartoma 577 Bronchial Carcinoid 583 Neuroendocrine Carcinoma 589 Kaposi Sarcoma 592 Lymphoma and Lymphoproliferative Disorders 598 Follicular Bronchiolitis 598 Lymphocytic Interstitial Pneumonia 601 Nodular Lymphoid Hyperplasia 607 Post-Transplant Lymphoproliferative Disease (PTLD) 610 Pulmonary Non-Hodgkin Lymphoma 616 Pulmonary Hodgkin Lymphoma 622 Metastatic Disease 625 Hematogenous Metastases 625 Lymphangitic Carcinomatosis 631 Diagnostic Imaging Chest Tumor Emboli 637 Section - Interstitial, Diffuse, and Inhalational Lung Disease 643 Introduction and Overview 643 Approach to Interstitial, Diffuse, and Inhalational Lung Disease 643 Idiopathic Interstitial Lung Diseases 649 Acute Respiratory Distress Syndrome (ARDS) 649 Acute Interstitial Pneumonia 652 Idiopathic Pulmonary Fibrosis 655 Nonspecific Interstitial Pneumonia 661 Cryptogenic Organizing Pneumonia 667 Sarcoidosis 673 Smoking-related Diseases 682 Respiratory Bronchiolitis and RBILD 682 Desquamative Interstitial Pneumonia 688 Pulmonary Langerhans Cell Histiocytosis 691 Pulmonary Fibrosis Associated with Smoking 697 Pneumoconiosis 700 Asbestosis 700 Silicosis and Coal Worker's Pneumoconiosis 706 Hard Metal Pneumoconiosis 712 Berylliosis 715 Silo-Filler's Disease 718 Other Inhalational Disorders 721 Hypersensitivity Pneumonitis 721 Smoke Inhalation 727 Aspiration 733 Talcosis 736 Eosinophilic Lung Disease 739 Acute Eosinophilic Pneumonia 739 Chronic Eosinophilic Pneumonia 745 Hypereosinophilic Syndrome 751 Metabolic Diseases and Miscellaneous Conditions 754 Alveolar Microlithiasis 754 Metastatic Pulmonary Calcification 757 Lymphangioleiomyomatosis 760 Pulmonary Amyloidosis 766 Pulmonary Alveolar Proteinosis 769 Lipoid Pneumonia 775 Section - Connective Tissue Disorders, Immunological Diseases, and Vasculitis 778 Introduction and Overview 778 Approach to Connective Tissue Disorders, Immunological Diseases, and Vasculitis 778 Immunological and Connective Tissue Disorders 780 Diagnostic Imaging Chest Ovid: Diagnostic Imaging: Chest 780 Scleroderma 786 Mixed Connective Tissue Disease 792 Polymyositis/Dermatomyositis 795 Systemic Lupus Erythematosus 798 Sjögren Syndrome 805 Ankylosing Spondylitis 811 Inflammatory Bowel Disease 814 Erdheim-Chester Disease 817 Thoracic Complications in Immunocompromised Patients 820 Hematopoietic Stem Cell Transplantation 820 Solid Organ Transplantation 826 HIV/AIDS 832 Neutropenia 838 Pulmonary Hemorrhage and Vasculitis 844 Idiopathic Pulmonary Hemorrhage 844 Goodpasture Syndrome 847 Pulmonary Wegener Granulomatosis 852 Churg-Strauss Syndrome 859 Behỗet Syndrome 862 Necrotizing Sarcoid Granulomatosis 865 Section - Mediastinal Abnormalities 868 Introduction and Overview 868 Approach to Mediastinal Abnormalities 868 Primary Neoplasms 876 Thymoma 876 Thymic Malignancy 883 Thymolipoma 886 Mediastinal Teratoma 889 Mediastinal Seminoma 895 Nonseminomatous Malignant Germ Cell Neoplasm 898 Neurogenic Neoplasms of the Nerve Sheath 901 Neurogenic Neoplasms of the Sympathetic Ganglia 907 Neurofibromatosis 910 Lymphadenopathy 916 Metastatic Disease, Lymphadenopathy 916 Mediastinal Hodgkin Lymphoma 922 Mediastinal Hon-Hodgkin Lymphoma 928 Sarcoidosis, Lymphadenopathy 934 Mediastinal Fibrosis 940 Localized Castleman Disease 947 Multicentric Castleman Disease 950 Diagnostic Imaging Chest Cysts 953 Bronchogenic Cyst 953 Esophageal Duplication Cyst 959 Pericardial Cyst 963 Pericardial Cyst 969 Vascular Lesions 972 Mediastinal Vascular Masses 972 Coronary Artery Aneurysm 978 Paraesophageal Varices 981 Mediastinal Lymphangioma 984 Mediastinal Hemangioma 987 Glandular Enlargement 991 Thymic Hyperplasia 991 Achalasia 996 Diseases of the Esophagus 1002 Achalasia 1002 Esophageal Diverticulum 1005 Esophageal Stricture 1009 Esophageal Carcinoma 1011 Miscellaneous Conditions 1017 Mediastinal Lipomatosis 1017 Mediastinitis 1020 Extramedullary Hematopoiesis 1026 Hiatal and Paraesophageal Hernia 1030 Section - Cardiovascular Disorders 1035 Introduction and Overview 1035 Approach to Cardiovascular Disorders 1035 Diseases of the Aorta and Great Vessels 1040 Atherosclerosis 1040 Aortic Aneurysm 1046 Acute Aortic Syndromes 1050 Marfan Syndrome 1055 Takayasu Arteritis 1058 Superior Vena Cava Obstruction 1061 Pulmonary Edema 1067 Cardiogenic Pulmonary Edema 1067 Noncardiogenic Pulmonary Edema 1077 Pulmonary Hypertension and Thromboembolic Disease 1080 Pulmonary Artery Hypertension 1080 Pulmonary Capillary Hemangiomatosis 1085 Pulmonary Venoocclusive Disease 1088 Acute Pulmonary Thromboembolic Disease 1091 Diagnostic Imaging Chest Chronic Pulmonary Thromboembolic Disease 1097 Sickle Cell Disease 1103 Fat Embolism 1109 Hepatopulmonary Syndrome 1112 Illicit Drug Use, Pulmonary Manifestations 1115 Diseases of the Heart and Pericardium 1118 Valve and Annular Calcification 1118 Aortic Valve Disease 1124 Mitral Valve Disease 1130 Left Atrial Calcification 1136 Ventricular Calcification 1139 Coronary Artery Calcification 1142 Post Cardiac Injury Syndrome 1148 Pericardial Effusion 1151 Constrictive Pericarditis 1160 Cardiovascular Neoplasms 1163 Cardiac and Pericardial Metastases 1163 Cardiac Myxoma 1169 Cardiac Sarcoma 1173 Pulmonary Artery Sarcoma 1176 Aortic Sarcoma 1179 Section 10 - Trauma 1182 Airways and Lung 1182 Tracheobronchial Laceration 1182 Pulmonary Contusion/Laceration 1184 Pleura, Chest Wall, and Diaphragm 1190 Traumatic Pneumothorax 1190 Traumatic Hemothorax 1193 Thoracic Splenosis 1195 Rib Fractures and Flail Chest 1198 Spinal Fracture 1207 Sternal Fracture 1210 Diaphragmatic Rupture 1213 Section 11 - Post-Treatment Chest 1219 Introduction and Overview 1219 Approach to Post-Treatment Chest 1219 Life Support Devices 1225 Appropriately Positioned Tubes and Catheters 1225 Abnormally Positioned Tubes and Catheters 1231 Pacemaker/AICD 1237 Surgical Procedures and Complications 1242 Pleurodesis 1242 10 Diagnostic Imaging Chest Reticular opacities on chest radiography Nodules & cysts Predominantly located in upper lung zones Spare lower lung zones Cysts irregular in appearance Affects men & women Lymphocytic Interstitial Pneumonia Normal lung volumes Basilar interlobular septal thickening on chest radiography Thin-walled cysts characteristic Nonspecific ground-glass opacity or consolidation Centrilobular & subpleural nodules Primarily affects women Idiopathic Pulmonary Fibrosis Progressive decrease in lung volumes Bilateral basilar & subpleural reticular opacities, honeycomb lung Adjacent lung parenchyma abnormal & distorted Affects men & women PATHOLOGY General Features Genetics TSC results from mutation in of genes TSC1 or TSC2 (chromosomes & 16) Associated abnormalities Small percentage of cases associated with TSC Extrathoracic findings Renal angiomyolipomas Chylous ascites Abdomen & pelvis lymphangioleiomyomas Uterine leiomyomas Lymphaticoureteric & lymphaticovenous connections Staging, Grading, & Classification LAM histologic scoring system (LHS) Total percentage of parenchyma affected by cysts & LAM cells Strong correlation with overall patient survival Survival at 10 years P.6:82 LHS-1 (1-25% involvement): 100% LHS-2 (25-50%): 75% LHS-3 (> 50%): < 53% Gross Pathologic & Surgical Features Enlarged lungs Numerous cysts along visceral pleura & homogeneously throughout lung parenchyma Cysts typically range in size from 0.5-2.0 cm Parenchymal cysts may be filled with air or chylous/serosanguineous fluid Involved lymph nodes appear white or pale tan Enlargement of thoracic duct & lymphatics Microscopic Features Characterized by cysts & atypical smooth muscle cells (LAM cells) LAM cells are morphologically heterogeneous Small & round, large & spindle-shaped, epithelioid Varying amounts of eosinophilic cytoplasm LAM cells present in cyst walls & along pulmonary lymphatics Infiltration of distal airways leads to narrowing, air-trapping, bullae, & pneumothoraces Obstruction of lymphatics leads to chylous pleural effusions Involvement of pulmonary vessels leads to hemosiderosis & hemoptysis Histologic features of sporadic LAM & LAM associated with TSC are almost indistinguishable 763 Diagnostic Imaging Chest Multifocal micronodular pneumocyte hyperplasia (MMNPH) is only unique feature of TSC Pathognomonic for TSC in patients with LAM Nodules consisting of type pneumocytes that proliferate along alveolar walls CLINICAL ISSUES Presentation Most common signs/symptoms Dyspnea Cough Spontaneous pneumothorax Other signs/symptoms Chest pain, hemoptysis, wheezing Chylothorax, chyloptysis, chylous pericardial effusion Demographics Age Reproductive years Mean age: 34 years Postmenopausal Hormonal replacement therapy Gender Women exclusively Few cases reported in men were in setting of TSC Epidemiology 100 cases/year worldwide incidence case/1,000,000 persons prevalence in United Kingdom, France, & United States Natural History & Prognosis Pulmonary function & imaging findings determine progression Progressive deterioration of pulmonary function typical Respiratory failure & cor pulmonale Recent studies have shown improved survival 91% survival rate at 10 years Greater likelihood of more rapid disease progression Cigarette smoking Pregnancy following onset of symptoms Progesterone treatment Predominant finding of cysts in biopsy specimens associated with poor prognosis Treatment Pneumothorax Higher recurrence rates with aspiration or intercostal drainage Lower recurrence rates with pleural abrasion, pleurodesis, pleurectomy, thoracoscopic pleurodesis with long-term drainage, or bullectomy with pleurodesis Chylous pleural effusions Thoracentesis, chemical pleurodesis, or parietal pleurectomy Hormonal therapy controversial Lung transplantation Recent studies have shown improved survivals year: 100% years: 90% years: 69% DIAGNOSTIC CHECKLIST Consider LAM in women of childbearing age with numerous thin-walled lung cysts on HRCT Image Interpretation Pearls Consider imaging abdomen & pelvis in patients with LAM to identify additional abnormalities associated with LAM & TSC SELECTED REFERENCES Avila NA et al: Imaging features of lymphangioleiomyomatosis: diagnostic pitfalls AJR Am J Roentgenol 196(4):9826, 2011 Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation Radiographics 25(3):803-28, 2005 764 Diagnostic Imaging Chest Kirchner J et al: Pulmonary lymphangioleiomyomatosis: high-resolution CT findings Eur Radiol 9(1):49-54, 1999 Rappaport DC et al: Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases AJR Am J Roentgenol 152(5):961-4, 1989 P.6:83 Image Gallery (Left) Axial NECT of a patient with LAM shows diffuse thin-walled lung cysts and multiple conspicuous mediastinal lymph nodes Lymphadenopathy is not an uncommon finding in patients with LAM (Right) Axial NECT of a patient with LAM shows small thin-walled cysts in the right lung and visualized left lung and a moderate right chylous pleural effusion Chylous pleural effusions may be hyperdense (secondary to proteinaceous material) or hypodense (secondary to fat) on CT (Left) Axial CECT of a patient with LAM demonstrates large thin-walled cysts throughout both lungs, with little normal intervening lung parenchyma identified (Right) Coronal CECT of the same patient shows that the diffuse lung cysts have replaced a large amount of the normal lung parenchyma LAM cysts typically increase in size and number with disease progression 765 Diagnostic Imaging Chest (Left) Axial NECT of a patient with LAM demonstrates a small right pneumothorax Pneumothorax, a known potential complication of LAM, typically occurs in association with diffuse parenchymal cysts (Right) Axial CECT through the upper abdomen of a patient with LAM demonstrates bilateral heterogeneous renal masses with multiple fat attenuation foci These lesions are consistent with angiomyolipomas, which are often associated with LAM Pulmonary Amyloidosis > Table of Contents > Section - Interstitial, Diffuse, and Inhalational Lung Disease > Metabolic Diseases and Miscellaneous Conditions > Pulmonary Amyloidosis Pulmonary Amyloidosis Tomás Franquet, MD, PhD Key Facts Terminology Heterogeneous group of disorders characterized by abnormal extracellular accumulation of insoluble fibrillar proteins Imaging Tracheobronchial abnormalities Airway wall thickening, mural/intraluminal nodules, submucosal calcification Parenchymal abnormalities Nodules: Single or multiple, 0.5-5 cm, 20% calcify Interstitial disease: Fine linear or reticulonodular opacities; honeycomb lung (uncommon); miliary nodules Lymphadenopathy Stippled, diffuse, or eggshell calcifications Other: Cardiomegaly, pleural effusion/thickening, soft tissue deposition Top Differential Diagnoses Tracheobronchial: Neoplasms, tracheobronchopathia osteochondroplastica, relapsing polychondritis Nodular: Lung cancer, metastases, granulomatous disease Interstitial lung disease: Idiopathic pulmonary fibrosis, scleroderma, rheumatoid arthritis Diffuse or multifocal lung calcification: Granulomatous disease, silicosis Lymphadenopathy: Sarcoidosis, tuberculosis Pleural thickening: Mesothelioma, metastases Clinical Issues Amyloid light chain (AL): 10% of patients with multiple myeloma develop amyloidosis Amyloid A chain (AA): Familial Mediterranean fever & age-related (senile) Poor prognosis for diffuse disease: Survival < years 766 Diagnostic Imaging Chest (Left) Axial NECT of a patient with primary amyloidosis (AL) shows multiple randomly distributed calcified lung nodules of different sizes and lobulated borders Calcified pulmonary nodules are present in 20% of cases of amyloidosis (Courtesy E Marchiori, MD.) (Right) Axial CECT of a 68-year-old patient with multiple myeloma shows right paratracheal lymphadenopathy containing multiple punctate calcifications Lymphadenopathy is common in AL type amyloidosis with systemic disease (Left) Axial NECT of a 54-year-old woman with monoclonal gammopathy shows diffuse pulmonary involvement with peripheral smooth interlobular septal thickening and reticulation Note that small nodules are also seen (Right) High-power photomicrograph (H&E stain) from the biopsy specimen of the same patient shows diffuse smooth interstitial thickening produced by amyloid accumulation Diffuse interstitial amyloidosis is the least common form of pulmonary amyloidosis P.6:85 TERMINOLOGY Definitions Generic term for heterogeneous group of disorders characterized by abnormal extracellular accumulation of insoluble fibrillar proteins Amyloid light chain (AL) (primary amyloidosis): Widespread deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains (monoclonal gammopathy or multiple myeloma) Amyloid A chain (AA) (secondary amyloidosis): Extracellular protein deposition caused by underlying chronic inflammatory disease such as, infection, bronchiectasis, rheumatic disease, neoplasms, agerelated (senile) & familial Mediterranean fever Major clinical forms: Systemic & localized 767 Diagnostic Imaging Chest Thoracic amyloidosis: Cardiac > tracheobronchial > parenchymal > lymph nodes > pleura IMAGING General Features Best diagnostic clue Multiple tracheal or pulmonary nodules (± calcification) + diffuse septal thickening + lymphadenopathy (± calcification) Radiographic Findings Radiography Tracheobronchial abnormalities: Mural nodules & diffuse thickening (± calcification) Parenchymal abnormalities Nodules: Single or multiple, 0.5-5 cm, calcification in 20% Interstitial disease: Fine linear or reticulonodular opacities; honeycomb lung (uncommon); miliary nodules Lymphadenopathy: Isolated finding or associated with interstitial disease May exhibit stippled, diffuse, or eggshell calcification Other: Cardiomegaly, pleural effusion, irregular pleural thickening (± calcification) CT Findings NECT Tracheobronchial abnormalities: Airway wall thickening, mural/intraluminal nodules, submucosal calcification Parenchymal abnormalities Diffuse micronodular (± calcification), reticulonodular, or linear opacities Ground-glass opacities, honeycomb lung, thick bronchovascular bundles Lymphadenopathy: Stippled, diffuse, or eggshell calcifications Other: Soft tissue deposition Imaging Recommendations Best imaging tool Chest radiography sufficient for documentation of extent of thoracic involvement HRCT: More sensitive in detecting tracheobronchial involvement & subtle parenchymal abnormalities DIFFERENTIAL DIAGNOSIS Tracheobronchial Primary benign & malignant neoplasms, tracheobronchopathia osteochondroplastica, relapsing polychondritis Nodular Lung cancer, pulmonary metastases, granulomatous disease, rheumatoid nodules Interstitial Lung Disease Idiopathic pulmonary fibrosis, scleroderma, rheumatoid arthritis, drug toxicity Diffuse or Multifocal Lung Calcification Granulomatous infection, alveolar microlithiasis, metastatic calcification, silicosis, sarcoidosis, dendritic calcification, healed varicella Lymphadenopathy Sarcoidosis, tuberculosis Pleural Thickening Mesothelioma, pleural metastases PATHOLOGY General Features Extracellular protein deposition Vascular deposition leads to fragility & bleeding Microscopic Features Amyloid in small blood vessel media, interstitium, & airways (uniform & linear or multiple small nodules) Calcification & foreign body giant cell reaction may be present Apple-green birefringence under polarized light after Congo red staining CLINICAL ISSUES Presentation Tracheobronchial form: Simulates asthma Nodular & interstitial form: Usually asymptomatic Demographics Epidemiology Amyloid light chain (AL): 10% of patients with multiple myeloma develop amyloidosis Amyloid A chain (AA): Familial Mediterranean fever & age-related (senile) 768 Diagnostic Imaging Chest Natural History & Prognosis Poor prognosis for diffuse disease: Survival < years Treatment No therapy is uniformly effective SELECTED REFERENCES Aylwin AC et al: Imaging appearance of thoracic amyloidosis J Thorac Imaging 20(1):41-6, 2005 Chung MJ et al: Metabolic lung disease: imaging and histopathologic findings Eur J Radiol 54(2):233-45, 2005 Pickford HA et al: Thoracic cross-sectional imaging of amyloidosis AJR Am J Roentgenol 168(2):351-5, 1997 Pulmonary Alveolar Proteinosis > Table of Contents > Section - Interstitial, Diffuse, and Inhalational Lung Disease > Metabolic Diseases and Miscellaneous Conditions > Pulmonary Alveolar Proteinosis Pulmonary Alveolar Proteinosis Santiago Martínez-Jiménez, MD Key Facts Terminology Pulmonary alveolar proteinosis (PAP) Alveolar accumulation of proteinaceous & lipid-rich surfactant-like material Imaging Radiography Bilateral central & symmetric reticular, reticulonodular, or heterogeneous opacities Relative sparing of lung apices & bases; may affect lung diffusely Abnormalities appear more severe than clinical status; “clinicoradiologic discrepancy” May mimic pulmonary edema HRCT Autoimmune PAP: “Crazy-paving” pattern (75%) Secondary PAP: Evenly distributed diffuse ground-glass opacities Top Differential Diagnoses Pulmonary edema Pneumocystis jiroveci pneumonia Diffuse alveolar hemorrhage Pathology Firm lung with yellow surface Alveolar lipoproteinaceous material Clinical Issues Symptoms/signs Asymptomatic, dyspnea, cough Opportunistic pneumonia Treatment: Whole lung lavage Diagnostic Checklist Consider PAP in patient with chronic multifocal airspace or mixed airspace-interstitial processes 769 Diagnostic Imaging Chest (Left) Graphic depicts findings of pulmonary alveolar proteinosis Lipid and proteinaceous-rich material resembling surfactant fill the alveoli (Right) Composite image with sagittal macroscopic lung section (right) and fluid obtained after bronchoalveolar lavage (left) shows areas of yellowish discoloration representing lipid-rich material and “milky” appearance of lavage fluid secondary to lipoproteinaceous components characteristic of PAP (Left) PA chest radiograph of a patient with autoimmune PAP demonstrates characteristic radiographic findings with bilateral central symmetric heterogeneous airspace disease (Right) PA chest radiograph of a patient with autoimmune PAP demonstrates subtle diffuse bilateral reticular opacities prior to treatment with whole lung lavage Most patients experience significant improvement after the initial lavage procedure, although additional lavage treatments may be required P.6:87 TERMINOLOGY Abbreviations Pulmonary alveolar proteinosis (PAP) Synonyms Pulmonary alveolar phospholipoproteinosis, lipoproteinosis Definitions Alveolar accumulation of proteinaceous & lipid-rich surfactant-like material IMAGING General Features Best diagnostic clue Radiography: Mimics pulmonary edema without pleural effusion or cardiomegaly CT: “Crazy-paving” pattern Radiographic Findings Radiography Bilateral central & symmetric reticular, reticulonodular, or heterogeneous opacities Air bronchograms may be present Mimics pulmonary edema without pleural effusions or cardiomegaly Radiographic abnormalities often appear more severe than clinical status (i.e., “clinicoradiologic discrepancy”) Relative sparing of lung apices & bases; may affect lung diffusely Findings suspicious for superimposed infection Pleural effusion Lymphadenopathy Dominant opacity/mass ± cavitation Post-treatment (lavage) Acute complications: Pneumothorax, pneumomediastinum ↑ parenchymal opacities due to retained lavage fluid Gradual improvement in 1st week Marked improvement by weeks, with frequent persistent abnormalities 770 Diagnostic Imaging Chest CT Findings HRCT Autoimmune PAP Random geographic distribution (abnormalities clearly demarcated from adjacent normal lung) “Crazy-paving” pattern (75%) Ground-glass opacities with superimposed thick interlobular septa Widespread & bilateral Consolidation Severity of ground-glass opacity & consolidation correlates with severity of functional parameters Mediastinal lymphadenopathy 1-2 lymph nodes > cm short axis diameter More numerous lymph nodes suggest superinfection or underlying hematologic disorder Hilar lymphadenopathy may be seen in silicoproteinosis-related PAP Fibrosis: Central or peripheral, traction bronchiectasis Secondary PAP Evenly distributed diffuse ground-glass opacities “Crazy-paving” pattern much less frequent MR Findings MR not indicated Lung abnormalities reported to exhibit low signal on T1WI with mild increase in signal intensity on T2WI Nuclear Medicine Findings PET/CT Not indicated Reports of mild & heterogeneous FDG accumulation Ga-67 scintigraphy Not needed; anecdotally may show Ga-67 uptake Imaging Recommendations Best imaging tool HRCT for characterization of diffuse lung disease CECT best for detection & characterization of complications, such as opportunistic infection DIFFERENTIAL DIAGNOSIS Pulmonary Edema May manifest with “crazy-paving” pattern Cardiomegaly & pleural effusion in appropriate clinical scenario Pneumocystis jiroveci Pneumonia May manifest with “crazy-paving” pattern Appropriate history & acute symptoms Diffuse Alveolar Hemorrhage May manifest with “crazy-paving” pattern ↓ hematocrit, sometimes hemoptysis More acute onset Lung Cancer (Invasive Mucinous Adenocarcinoma) May manifest with “crazy-paving” pattern Constitutional symptoms, such as weight loss May exhibit lymphadenopathy Other Entities with “Crazy-Paving” Pattern Sarcoidosis Nonspecific interstitial pneumonia Organizing pneumonia Acute respiratory distress syndrome (ARDS) Lipoid pneumonia PATHOLOGY General Features Etiology Congenital Minority of cases Disorders caused by 771 Diagnostic Imaging Chest Homozygous mutation of genes encoding surfactant protein (SP)-B, SP-C, & ABCA3 transporter Absence of granulocyte/macrophage colony stimulating factor (GM-CSF) receptor Autoimmune P.6:88 Majority of cases (90%) Adults with high prevalence of anti-granulocyte-macrophage colony-stimulating factor (GMCSF) Secondary 5-10% of cases Inhalational exposure: Silica, cement, aluminum, titanium dioxide, nitrogen dioxide, fiberglass Hematologic malignancy: Acute myeloid leukemia, myelodysplastic syndrome, chronic myeloid leukemia Immunodeficiency: Immunosuppressive therapy, HIV Associated abnormalities Superimposed infection in 13% Gross Pathologic & Surgical Features Firm lung with yellow surface (due to lipid) Microscopic Features Alveolar lipoproteinaceous material; periodic-acid Schiff (PAS)-positive Thick alveolar walls due to hyperplasia of type II pneumocytes Variable chronic inflammatory cells & fibrosis CLINICAL ISSUES Presentation Most common signs/symptoms Asymptomatic (33%) Progressive shortness of breath Dry or minimally productive cough Crackles Clubbing Cyanosis Other signs/symptoms Fatigue Weight loss Low-grade fever Chest pain Hemoptysis Clinical profile Autoimmune PAP has strong association with cigarette smoking (75% of cases) Laboratory findings ↑ lactate dehydrogenase Arterial blood gases: Hypoxemia, ↑ arterial oxygen tension (PaO2), ↑ alveolar-arterial oxygen tension (aAPO2) Pulmonary function tests: ↓ diffusion capacity (DLCO), ↓ lung volumes, ↓ compliance Granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies Autoimmune PAP: Positive Secondary: Negative Bronchoalveolar lavage (BAL): “Milky” material Complications Opportunistic pneumonia (13%): Nocardia, Candida, C neoformans, Aspergillus, Cytomegalovirus, M tuberculosis, nontuberculous mycobacteria, H capsulatum, P jiroveci, S pneumoniae Fibrosis (rare): Unclear whether coincidental finding or end-stage of PAP Demographics Age Autoimmune PAP Average age: 40 years (20-50 years) May affect young children 772 Diagnostic Imaging Chest Gender Autoimmune PAP Smokers: Men 3x more commonly affected Nonsmokers: No gender predilection Epidemiology Autoimmune PAP Incidence: 0.36 new cases/million persons/year Prevalence: 3.7 cases/million persons Natural History & Prognosis Good prognosis Disease-specific survival exceeds 80% at years Marked increase in survival over recent decades due to better treatment BAL techniques have markedly improved prognosis Significant pulmonary fibrosis (rare) Treatment Whole lung lavage 25-40 liters of saline, both lungs, usually performed sequentially May be repeated several times Most patients experience significant improvement from single thorough lavage After lavage, up to 70% of patients remain symptom-free at years Few patients require annual or biannual therapeutic lavage Immunomodulation Subcutaneous administration of GM-CSF for treatment of autoimmune PAP has moderate success Experimental treatment includes plasmapheresis DIAGNOSTIC CHECKLIST Consider PAP in patient with chronic multifocal airspace or mixed airspace-interstitial processes Image Interpretation Pearls “Crazy-paving” pattern is not specific for PAP “Crazy-paving” pattern is classic HRCT finding of pulmonary alveolar proteinosis, but this disease is rare SELECTED REFERENCES Ishii H et al: Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis Chest 136(5):1348-55, 2009 Frazier AA et al: From the archives of the AFIP: pulmonary alveolar proteinosis Radiographics 28(3):883-99; quiz 915, 2008 Inoue Y et al: Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan Am J Respir Crit Care Med 177(7):752-62, 2008 Ioachimescu OC et al: Pulmonary alveolar proteinosis Chron Respir Dis 3(3):149-59, 2006 P.6:89 Image Gallery 773 Diagnostic Imaging Chest (Left) PA chest radiograph of a patient with autoimmune PAP shows bilateral scattered, ill-defined, subsegmental, heterogeneous airspace opacities (Right) Axial HRCT of the same patient shows geographic areas of “crazy-paving” characterized by ground-glass opacity and septal thickening Histologically, ground-glass opacities correlated with alveolar filling Superimposed thick septa result from expansion of interlobular septa by edema and massively dilated lymphatic channels (Left) Coronal NECT of a patient with autoimmune PAP shows diffuse pulmonary abnormalities with relative sparing of the left lung base near the costophrenic angle (Right) Axial HRCT of a patient with leukemia and secondary PAP shows characteristic diffuse bilateral ground-glass opacities without thick interlobular septa and mediastinal lymphadenopathy While the “crazy-paving” pattern can be seen in patients with secondary PAP, it is an uncommon imaging manifestation 774 Diagnostic Imaging Chest (Left) Axial CECT of a patient with PAP and nocardiosis shows bilateral diffuse ground-glass opacities and left upper lobe thick- and thin-walled cavities (Right) Axial HRCT of a patient with PAP shows heterogeneous consolidation and reticular opacities with traction bronchiectasis/bronchiolectasis and honeycomb lung The patient was treated with bilateral lung transplantation Histopathological analysis of the native lungs showed pulmonary fibrosis (Courtesy C S Restrepo, MD.) Lipoid Pneumonia > Table of Contents > Section - Interstitial, Diffuse, and Inhalational Lung Disease > Metabolic Diseases and Miscellaneous Conditions > Lipoid Pneumonia Lipoid Pneumonia Tomás Franquet, MD, PhD Key Facts Terminology Endogenous lipoid pneumonia (“golden pneumonia”) Accumulation of alveolar macrophages due to airway obstruction or impaired mucociliary clearance Exogenous lipoid pneumonia Repetitive aspiration or inhalation of mineral oil Imaging Radiography Acute aspiration: Focal or multifocal consolidations, predilection for lower lobes Chronic aspiration: Mass-like or nodular lesion CT Focal/extensive areas of low (fat) attenuation Fat attenuation in nodule or mass-like consolidation “Crazy-paving” pattern on HRCT Top Differential Diagnoses Consolidation Bacterial pneumonia, organizing pneumonia Nodule or mass-like consolidation Lung cancer, focal organizing pneumonia “Crazy-paving” pattern Alveolar proteinosis, adenocarcinoma, P jiroveci pneumonia, pulmonary hemorrhage Clinical Issues Symptoms/signs Acute: Cough, dyspnea, low-grade fever Subacute/chronic: Asymptomatic, chronic cough Diagnostic Checklist Consider lipoid pneumonia in patients with lung nodule, mass, or consolidation with intrinsic fat attenuation or “crazy-paving” pattern on CT 775 Diagnostic Imaging Chest (Left) AP scout CT of a 26-year-old woman with anorexia, repetitive mineral oil aspiration, and exogenous lipoid pneumonia shows bilateral consolidations with air bronchograms in the middle lobe, lingula, and lower lobes (Courtesy A Hidalgo, MD.) (Right) Axial NECT of the same patient shows fat attenuation within middle lobe and lingular consolidations and small bilateral pleural effusions Careful clinical history is required to identify the source of the aspirated material (Courtesy A Hidalgo, MD.) (Left) Composite image shows NECT of lung (left) and mediastinal (right) window of a patient with lipoid pneumonia manifesting as a spiculated mass with intrinsic fat attenuation and surrounding bronchiolitis (Right) HRCT of a 78-year-old man with exogenous lipoid pneumonia due to mineral oil aspiration shows “crazy-paving” pattern characterized by ground-glass opacity with intrinsic interlobular septal thickening and intralobular lines This finding has been described in lipoid pneumonia P.6:91 TERMINOLOGY Synonyms Endogenous lipoid pneumonia (“golden pneumonia”) Definitions Endogenous lipoid pneumonia: Accumulation of alveolar macrophages due to airway obstruction or impaired mucociliary clearance Exogenous lipoid pneumonia: Repetitive aspiration or inhalation of mineral oil or related material into lung Animal or vegetable oils, oral laxatives, oil-based nose drops, liquid paraffin IMAGING General Features 776 Diagnostic Imaging Chest Best diagnostic clue Consolidation with -30 to -150 HU attenuation Location Gravity-dependent areas of lung Radiographic Findings Radiography Acute aspiration: Radiographically visible within 30 minutes of aspiration or inhalation Bilateral or unilateral diffuse consolidation Focal or multifocal segmental consolidations; predominantly in lower lobes Chronic aspiration: Mass-like or nodular lesion with irregular margins; may mimic lung cancer CT Findings Focal/extensive areas of low (fat) attenuation Fat attenuation within mass-like consolidation “Crazy-paving” pattern: Ground-glass opacity with interlobular septal thickening & intralobular lines MR Findings Visualization of fat: High signal on T1WI & T2WI or documentation of fat on chemical shift MR Imaging Recommendations Best imaging tool CT is imaging modality of choice for demonstrating fat attenuation within lipoid pneumonia lesions DIFFERENTIAL DIAGNOSIS Consolidation Bacterial pneumonia Organizing pneumonia Nodule or Mass-like Consolidation Lung cancer (spiculated margins) Focal organizing pneumonia “Crazy-Paving” Pattern Alveolar proteinosis Adenocarcinoma Pneumocystis jiroveci pneumonia Pulmonary hemorrhage PATHOLOGY General Features Etiology Mineral oil is most common agent Animal & vegetable oils also implicated Gross Pathologic & Surgical Features Chronically, lipid is fibrogenic; may produce architectural distortion Microscopic Features Bronchopneumonia, alveolar lipid-laden macrophages Interstitial accumulation of lipid, inflammatory cellular infiltration, variable fibrosis Positive Congo red stain CLINICAL ISSUES Presentation Most common signs/symptoms Acute: Cough, dyspnea, low-grade fever Subacute/chronic (repeated subclinical aspiration): Usually asymptomatic, chronic nonproductive cough Lipid not irritant, silent aspiration while sleeping Demographics Age Any age Epidemiology Patients at risk Neonates, infants with feeding problems Elderly Swallowing dysfunction or esophageal abnormality Natural History & Prognosis Directly related to type & extent of aspiration Recurrent aspiration may lead to fibrosis 777 ... 11 12 Illicit Drug Use, Pulmonary Manifestations 11 15 Diseases of the Heart and Pericardium 11 18 Valve and Annular Calcification 11 18 Aortic... 12 07 Sternal Fracture 12 10 Diaphragmatic Rupture 12 13 Section 11 - Post-Treatment Chest 12 19 Introduction and Overview 12 19... 13 98 Section 13 - Chest Wall and Diaphragm 14 04 11 Diagnostic Imaging Chest Introduction and Overview 14 04 Approach to Chest Wall and Diaphragm 14 04