The Thorax: Medical, Radiological, and Pathological Assessment

Description

The current medical practice has become more of a team effort rather than an isolated practice.  The current evaluation of patients in the daily practice is essentially performed by medical assessment of the patient in question followed by diagnostic imaging, and when needed and possible the evaluation of tissue for diagnosis with its subsequent assessment of biomarkers and other ancillary tools that play an important role in the evaluation and prognosis.  The book herein proposed will exactly provide such assessment focused in the thoracic assessment of patients afflicted with any particular disease of the thorax.  Expert clinician in pulmonary medicine will provide the state of the art in the evaluation of such patients, which will provide the most important background in the clinical impression and further assessment of these patients.  This assessment in most cases is followed by the evaluation of imaging, which provides a highly important information of not only the exact location of the process but also of the nature of whether is localized, infiltrative, diffuse, bilateral, etc., as well as the possible compromise of other adjacent structures.  Such information is crucial as imaging and clinical information will provide a working diagnosis, which ultimately will be defined by the pathological assessment.  Therefore, in real practice neither one of these subspecialties works alone or in isolation.  On the contrary, each one depends on the other for the final diagnosis and proper management of patients with thoracic diseases.  Based on such experience is that the current text will provide in the same text of the needed information that a clinician, radiologist or pathologist will need in order to arrive to the best possible conclusion. The scope of this book is unique in its nature as currently, even though there are several text on either one of those specialties, those publication are strictly on the either radiology, medicine or pathology.  Our proposed text will bring all those together in a single text and written by experts in their respective field.  This book should be of interest to all of those in the practice of thoracic medicine (radiologist, pulmonologist, pathologists) and any one who in the general practice is confronted with the evaluation of these patients. Preface Acknowledgments Contents Contributors Part I: Serosal Surface 1: The Pleura Introduction Pleural Anatomy General Radiology Concepts Nonneoplastic Fibrous and Fibrinous Pleuritis Histological Features Pleural Infections Histopathological Features Uremia Hemothorax Coronary Artery Bypass Surgery Apical Pleural Plaques Pneumoconiosis Connective Tissue Diseases Drug-Induced Fibrous Pleuritis IgG4-Related Fibrous Pleuritis Pleuroparenchymal Fibroelastosis Mesothelial Hyperplasia Pleural Endometriosis Adenomatoid Tumor Neoplastic Malignant Mesothelioma Radiography Computed Tomography Magnetic Resonance Imaging PET/CT Ultrasound Limitations of Routine Imaging Therapy Response Assessment Volumetric and Future Imaging of MPM Staging Pathological Features Macroscopic Features Microscopic Features Malignant Mesothelioma In Situ Invasive Malignant Mesothelioma Immunohistochemical Features Molecular Analysis Pseudomesotheliomatous Adenocarcinoma Pathological Features Macroscopic Features Microscopic Features Immunohistochemical Features Pleural Thymoma Pathological Features Macroscopic Features Histological Features Immunohistochemmical Features Primary Salivary Gland Type Tumors of the Pleura Mucoepidermoid Carcinoma (MEC) Epithelial-Myoepithelial Carcinoma Solitary Fibrous Tumor Pathological Features Macroscopic Features Histological Features Immunohistochemical Features Desmoid Tumor Pathological Features Macroscopic Features Histological Features Immunohistochemical Features Calcifying Fibrous Tumor Pathological Features Macroscopic Features Histological Features Immunohistochemical Features Vascular Neoplasms Epithelioid Hemangioendothelioma Pleural Angiosarcoma Histopathological Features Epithelioid Hemangioendothelioma (EH) Pleural Angiosarcoma Immunohistochemical Features Molecular Features Neuroectodermal Tumors Pathological Features Macroscopic Features Microscopic Features Immunohistochemical Features Molecular Features Other Pleural Sarcomas Primary Smooth Muscle Tumors of the Pleura Synovial Sarcoma Pathological Features Macrocopic Features Microscopic Features Immunohistochemical Features Molecular Features Body Cavity Lymphoma Primary Effusion Lymphoma Pyothorax-Associated Lymphoma Pleural Manifestations of Hematologic Malignancy Pleural Metastasis Pleural Effusions Epidemiology and Associated Conditions Management References Part II: The Mediastinum 2: Normal Thymus Radiological Aspects of the Mediastinum Mediastinal Compartments Imaging Modalities The Thymus Practical Embryological Aspects of the Thymus Histological Features Other Cellular Components Thymic Involution Immunohistochemical Features References 3: Thymoma Epidemiological Features Clinical Features Diagnostic Imaging Histological Classification Pathological Staging Pathological Features Clinical Outcome References 4: Thymic Carcinoma Pathogenesis Diagnostic Criteria Epidemiological Features Clinical Features Diagnostic Imaging Pathological Staging Pathological Features Thymic Adenocarcinoma NUT Carcinoma Salivary Gland Type Carcinomas Clinical Features Mucoepidermoid Carcinoma Adenoid Cystic Carcinoma Epithelial-Myoepithelial Carcinoma Basaloid Carcinoma References 5: Neuroendocrine Neoplasms Neuroendocrine Carcinomas Clinical Features Diagnostic Imaging Pathological Staging Histological Grading and Nomenclature Pathological Features Macroscopic Features Microscopic Features Immunohistochemical Features Paraganglioma Diagnostic Imaging Pathological Features Macroscopic Features Histopathological Features Immunohistochemical Features Parathyroid Tumors Diagnostic Imaging Pathological Features Macroscopic Features Microscopic Features Immunohistochemical Features References 6: Mediastinal Germ Cell Tumors Mediastinal Germ Cell Tumors Estimated Occurrence Classification Staging Clinical Symptomatology Diagnostic Imaging Pathological Features Teratoma Immunohistochemical Features Seminoma Non-Seminomatous Germ Cell Tumors Yolk Sac Tumor Embryonal Carcinoma Choriocarcinoma Immunohistochemical Features Clinical Outcome References 7: Mesenchymal Tumors of the Mediastinum Vascular Tumors Lymphangioma Hemangioma Epithelioid Hemangioendothelioma Angiosarcoma Fibroblastic and Fibrohistiocytic Tumors Solitary Fibrous Tumor Sclerosing Epithelioid Fibrosarcoma Low-Grade Fibromyxoid Sarcoma Neurogenic Tumors Benign Peripheral Nerve Sheath Tumors Malignant Peripheral Nerve Sheath Tumors (MPNST) Ganglionic Tumors Myogenic Tumors Smooth Muscle Tumors Skeletal Muscle Tumors Lipomatous Tumors Benign Lipomatous Tumors Malignant Lipomatous Tumors Bone and Cartilaginous Tumors Tumors of Unknown Etiology References 8: Mediastinal Lymphoproliferative Disorders Benign Lymphoproliferative Lesions of the Mediastinum Introduction Sarcoidosis Granulomatous Lymphadenitis Caused by an Infectious Organism or a Known External Substance Castleman Disease Hyaline Vascular Castleman Disease Plasma Cell-Rich Castleman Disease Sclerosing Mediastinitis with Emphasis in Mediastinal IgG4-Related Disease Langerhans Cells Histiocytosis (LCH) Rosai-Dorfman Disease (RDD) Malignant Lymphoproliferative Disorders and Other Hematopoietic Disorders of the Mediastinum Introduction Non-Hodgkin Lymphomas of B-Cell Origin Primary Mediastinal (Thymic) Large B-Cell Lymphoma (PM-LBCL) Mediastinal Diffuse Large B-Cell Lymphoma (DLBCL) B-Cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classic Hodgkin Lymphoma/Mediastinal Gray Zone Lymphoma (M-GZL) Thymic Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue (Thymic MALT Lymphoma) Mediastinal Plasmacytoma Non-Hodgkin Lymphomas of T-Cell Origin T-Lymphoblastic Leukemia/Lymphoma (T-ALL/ LBL) Anaplastic Large Cell Lymphoma (ALCL) Classic Hodgkin Lymphoma (CHL) Lymphoma Imaging Lymphoma Imaging Classification Diffuse Large B Cell Lymphoma Primary Mediastinal (Thymic) Large B Cell Lymphoma Hodgkin Lymphoma Myeloid Neoplasms: Mediastinal Myeloid Sarcoma Other Tumors: Follicular Dendritic Cell (FDC) Sarcoma References 9: Miscellaneous Conditions Fibrosing/Sclerosing Mediastinitis Diagnostic Imaging Pathological Features Multilocular Thymic Cyst (MTC) Diagnostic Imaging Pathological Features Thymic Hyperplasia and Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features Diagnostic Imaging Pathological Features Ependymoma Pathological Features References Part III: Lung: Neoplastic Conditions 10: Non-Small Cell Carcinoma Introduction Epidemiology Incidence and Prognosis Risk Factors Clinical Presentation Imaging Modalities Staging of Lung Cancer T Classification N Classification M Classification Special Considerations in TNM-8 Imaging Characteristics Non-Small Cell Carcinoma Squamous Cell Carcinoma Adenocarcinoma Invasive Adenocarcinoma Large Cell Carcinoma Adenosquamous Carcinoma Lymphoepithelioma-Like Carcinoma Sarcomatoid Carcinoma Pathological Features Squamous Cell Carcinoma Invasive Squamous Cell Carcinoma Histological Variants Adenocarcinoma Histological Variants Atypical Adenomatous Hyperplasia, In Situ Adenocarcinoma, Minimally Invasive Adenocarcinoma, Adenocarcinoma with Lepidic Growth Pattern, Brochioloalveolar Carcinoma Adenocarcinoma, Histological Variants Other Uncommon Variants Adenosquamous Carcinoma Pathological Features Other Unusual Variants of Non-Small Cell Carcinoma Lymphoepithelioma-Like Carcinoma Rhabdoid Carcinoma Primary Mammary-Like Carcinoma Large Cell Carcinoma Immunohistochemical Antibodies, Biomarkers, and Molecular Biology in Non-Small Cell Carcinoma Sarcomatoid Carcinoma, Pleomorphic Carcinoma, and Giant Cell Carcinoma Summary References 11: Neuroendocrine Neoplasms of the Lung Introduction Pulmonary Neuroendocrine System Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Epidemiology and Demographics Clinical Presentation Diagnostic Evaluation Treatment and Prognosis Pulmonary Carcinoids (Typical and Atypical) Epidemiology and Demographics Clinical Presentation Diagnosis Large Cell Neuroendocrine Carcinoma Epidemiology Clinical Presentation Prognosis Diagnostic Imaging Diffuse Idiopathic Neuroendocrine Cell Hyperplasia Low-Grade (Typical) Carcinoid and Intermediate-Grade (Atypical) Carcinoid High-Grade Large Cell Neuroendocrine Carcinoma Small Cell Carcinoma Epidemiology and Demographics Clinical Presentation Diagnostic Evaluation Staging Systems Prognosis and Prevention High-Grade Small Cell Carcinoma Intrapulmonary Paraganglioma Pathological Features Classifications, Nomenclature, Definitions, and Staging Histological Grading Macroscopic Features Histological Features Low- and Intermediate-Grade Carcinomas Immunohistochemical and Molecular Features Large Cell Neuroendocrine Carcinoma Small Cell Carcinoma Intrapulmonary Paraganglioma Pathological Features References 12: Salivary Gland-Type Tumors of the Lung Introduction Mucoepidermoid Carcinoma Diagnostic Imaging Pathological Features Adenoid Cystic Carcinoma Diagnostic Imaging Pathological Features Immunohistochemical and Molecular Features Pleomorphic Adenoma (Mixed Tumor) Diagnostic Imaging Pathological Features Immunohistochemical Features Epithelial–Myoepithelial Carcinoma Diagnostic Imaging Pathological Features Acinic Cell Carcinoma Diagnostic Imaging Pathological Features Hyalinizing Clear Cell Carcinoma Diagnostic Imaging Pathological Features Oncocytoma Diagnostic Imaging Pathological Features Mucous Gland Adenoma Diagnostic Imaging Pathological Features Summary References 13: Biphasic Neoplasms of the Lung Pulmonary Biphasic Neoplasms Pulmonary Blastoma Clinical Features Diagnostic Imaging Pathologic Features Carcinosarcoma Clinical Features Diagnostic Imaging Pathological Features Summary References 14: Benign and Malignant Mesenchymal Tumors of the Lung Benign Mesenchymal Tumors of the Lung Cartilaginous Hamartoma Leiomyoma Schwannoma Endobronchial Lipoma Solitary Fibrous Tumor (SFT) Malignant Mesenchymal Tumors of the Lung Smooth Muscle Tumors Skeletal Muscle Tumors Synovial Sarcoma Sarcomas of Bone and Cartilage Pulmonary Artery Sarcoma Primary Pulmonary Myxoid Sarcoma Malignant Vascular Neoplasms Kaposi’s Sarcoma (KS) Epithelioid Hemangioendothelioma (EHE) Angiosarcoma Summary References 15: Pulmonary Lymphoproliferative Disorders Benign Lymphoproliferative Lesions of the Lung Introduction Pulmonary Nodular Lymphoid Hyperplasia (PNLH) Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Follicular Bronchiolitis Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Lymphoid Interstitial Pneumonia (LIP)/Diffuse Lymphoid Hyperplasia Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis IgG4-Related Lung Disease (IgG4-RLD) Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Malignant Lymphoproliferative Disorders and Other Hematopoietic Disorders of the Lung Introduction Pulmonary Non-Hodgkin Lymphomas of B-Cell Origin Pulmonary Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue (Pulmonary MALT Lymphoma) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Diffuse Large B-Cell Lymphoma (DLBCL) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Intravascular Large B-Cell Lymphoma (LBCL) Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Lymphomatoid Granulomatosis (LYG) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Grading System Differential Diagnosis Molecular Findings Other Small B-cell Lymphomas: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL), Mantle Zone Lymphoma, Follicular Lymphoma and Lymphoplasmacytic Lymphoma Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Extraosseous (Pulmonary) Plasmacytoma Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Non-Hodgkin Lymphomas of T-Cell Origin Pulmonary Anaplastic Large Cell Lymphoma (ALCL) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL, NOS) Introduction Clinical Features Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Classic Hodgkin Lymphoma (CHL) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Myeloid Neoplasms: Pulmonary Acute Myeloid Leukemia/Myeloid Sarcoma Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Histiocytic Disorders of the Lung Pulmonary Langerhans Cell Histiocytosis (LCH) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Alterations Extranodal Pulmonary Rosai–Dorfman Disease (RDD) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Erdheim–Chester Disease (ECD) Introduction Clinical Features Diagnostic Imaging Pathology Immunohistochemistry and Other Ancillary Studies Differential Diagnosis Molecular Findings Pulmonary Juvenile Xanthogranuloma (PJXG) Introduction Clinical Features Pathology Immunohistochemistry Differential Diagnosis Pulmonary Crystal-Storing Histiocytosis Introduction Pathology Immunohistochemistry References 16: Tumors of Uncertain Histogenesis Clear Cell “Sugar” Tumor Pathological Features Inflammatory Pseudotumor (Myofibroblastic Tumor) Pathological Features Pneumocytoma (Sclerosing Hemangioma) Pathological Features Granular Cell Tumor Pathological Features Hemangioblastoma-like Clear Cell Stromal Tumor Pathological Features Summary References 17: Uncommon Tumors of the Lung Glomangioma/Glomangiosarcoma Clinical Features Diagnostic Imaging Pathological Features Bronchial (Pulmonary) Melanoma Clinical Features Diagnostic Imaging Pathological Features Pulmonary Meningioma Clinical Features Pathological Features Pulmonary Meningotheliomatosis Diagnostic Imaging Pathological Features Intrapulmonary Thymoma Clinical Features Diagnostic Imaging Pathological Features Ganglioneuroblastoma Pathological Features Pulmonary Adenofibroma Clinical Features Pathological Features Alveolar Adenoma Pathological Features Angiolymphoid Hyperplasia with Eosinophilia Clinical Features Pathological Features Amyloid Tumor (Amyloidoma) Clinical Features Pathological Features Placental Transmogrification Clinical Features Diagnostic Imaging Pathological Features Summary References Part IV: Lung: Non-neoplastic Conditions 18: Interstitial Lung Diseases Idiopathic Pulmonary Fibrosis Introduction Epidemiology Pathogenesis Risk Factors Cigarette Smoking and Environmental Risk Factors Microbial Agents Gastroesophageal Reflux Genetic Risk Factors Clinical Presentation and Natural History Diagnostic Evaluation Laboratory Testing Pulmonary Function Tests Chest Radiograph Bronchoalveolar Lavage Transbronchial Lung Biopsy Transbronchial Lung Cryobiopsy Surgical Lung Biopsy Diagnosis Multidisciplinary Discussion Desquamative Interstitial Pneumonia Introduction Epidemiology Etiologic Risk Factors Cigarette Smoking Occupational and Environmental Exposures Systemic Diseases and Medications Clinical Presentation Diagnostic Evaluation Laboratory Testing Pulmonary Function Test Imaging Chest Radiographs CT and HRCT Findings Evolution of Findings Differential Diagnosis Bronchoalveolar Lavage Transbronchial Biopsy, Transbronchial Lung Cryobiopsy, and Surgical Lung Biopsy Histopathology Nonspecific Interstitial Pneumonia Introduction Clinical Presentations and Prognosis Diagnostic Evaluation Laboratory Testing Pulmonary Function Tests Bronchoalveolar Lavage Causes and Clinical Entities Multidisciplinary Discussion and Decision to Biopsy Imaging of Nonspecific Interstitial Pneumonia Evolution of Imaging in NSIP Exacerbation Versus Infection Differential Diagnosis Histopathology Usual Interstitial Pneumonia Introduction Clinical Presentations Causes and Clinical Entities Diagnostic Evaluation Laboratory Testing Pulmonary Function Tests Bronchoalveolar Lavage Multidisciplinary Discussion and Decision to Biopsy Imaging of Usual Interstitial Pneumonia (UIP) Chest Radiography HRCT UIP Pattern Probable UIP Indeterminate and Inconsistent UIP Patterns Nonidiopathic UIP Pattern Connective Tissue Disorders Differential Diagnosis Histopathologic Pattern Hypersensitivity Pneumonitis (Extrinsic Allergic Alveolitis) Introduction Epidemiology Pathogenesis Antigens and Physiologic Susceptibility Immune Dysregulation Cigarette Smoking Clinical Presentation Diagnostic Evaluation Exposure Questionnaire Laboratory Testing Pulmonary Function Test Bronchoalveolar Lavage Transbronchial Biopsy and Transbronchial Lung Cryobiopsy Surgical Lung Biopsy Imaging of Hypersensitivity Pneumonitis Chest Radiograph Chest CT Acute /Subacute HP Chronic HP Differential Diagnosis Histopathology Pulmonary Lymphangioleiomyomatosis Introduction Epidemiology Pathogenesis Clinical Manifestations and Diagnosis Differential Diagnosis Imaging of Lymphangiomyomatosis (LAM) Chest Radiograph Chest CT Abdominal Imaging Diagnostic Evaluation Laboratory Tests Pulmonary Function Tests Lung Biopsy Histopathology Prognosis Treatment Other Considerations About LAM References 19: Connective Tissue Disease-Associated Interstitial Lung Disease Rheumatoid Arthritis Introduction Interstitial Lung Disease Airway Disease Rheumatoid Nodules Pleural Disease Caplan Syndrome Pulmonary Hypertension Pulmonary Infections Drug-Induced Lung Disease Other Systemic Lupus Erythematosus Introduction Pleural Disease Parenchymal Disease Interstitial Lung Disease Acute Lupus Pneumonitis Vascular Disease Diffuse Alveolar Hemorrhage Pulmonary Hypertension Pulmonary Embolism Diaphragmatic Involvement Shrinking Lung Syndrome Other Pulmonary Involvement Sjogren Syndrome Introduction Airway Disease Interstitial Lung Disease Pulmonary Amyloidosis Pulmonary Lymphoma and Other Hematological Involvement Pulmonary Hypertension Other Respiratory Manifestations Systemic Sclerosis Introduction Etiology Diagnosis Disease Presentation Evaluation Interstitial Lung Disease Pulmonary Hypertension Other Manifestations Dermatomyositis and Polymyositis Interstitial Lung Disease Pulmonary Hypertension Spontaneous Pneumomediastinum Malignancy Other Ankylosing Spondylitis Modified New York criteria [285] Assessment of SpondyloArthritis International Society (ASAS) Criteria [286] Pulmonary Apical Fibrocystic Disease Obstructive Sleep Apnea Other Relapsing Polychondritis Introduction Airway Disease Myelodysplastic Syndrome Other Mixed Connective Tissue Disease Interstitial Lung Disease Pulmonary Arterial Hypertension Other References 20: Pneumoconiosis Asbestos-Related Lung Diseases: A Review Introduction Types of Asbestos and Occupations Associated with Exposure Pathogenesis of Asbestos-Related Lung Conditions Fate of Asbestos Fibers in Lungs Asbestos-Related Pleuro-pulmonary Conditions Benign Conditions Pleural Diseases Pleural Plaque Benign Asbestos Pleural Effusion Rounded Atelectasis Diffuse Pleural Thickening Parenchymal Conditions Asbestosis Malignant Conditions Pleural Diseases Malignant Pleural Mesothelioma Parenchymal Diseases Lung Cancer Silicosis Introduction Epidemiology Occupational Risk for Silicosis and Risk Mitigation Pathogenesis Clinico-Pathologic Types of Silicosis Clinical Features Pulmonary Function Tests (PFT) Radiographic Features Diagnostic Approach Management Hard Metal Lung Disease Introduction Pathogenesis Clinical Features Radiology Diagnosis Pulmonary Function Test Laboratory Tests Bronchoscopy Pathology Management References 21: Pulmonary Vasculitides Part I: Pulmonary Disease in Primary Vasculitides Introduction Takayasu Arteritis Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Pulmonary Disease Lung Parenchyma Vasculature Diagnostic Evaluation Laboratory Data Imaging Histopathological Features Management Prognosis and Conclusion Giant Cell Arteritis Introduction Incidence and Risk factors Pathogenesis Clinical Presentation Pulmonary Disease Upper Respiratory Tract Lower Respiratory Tract and Lung Parenchymal Pleura Vasculature Diagnostic Evaluation Laboratory Data Imaging Management Glucocorticoids Methotrexate Tocilizumab Other DMARDs Prognosis and Conclusion Behcet’s Disease Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Pulmonary Disease Vasculature Lung Parenchyma Diagnostic Evaluation Laboratory Data Imaging Management Pharmacologic Treatment Surgical Treatment Prognosis and Conclusion Polyarteritis Nodosa Introduction Incidence and Risk factors Pathogenesis Clinical Presentation Pulmonary Disease Lung Parenchyma Vasculature Diagnostic Evaluation Laboratory Data Imaging Management Prognosis and Conclusion Necrotizing Sarcoid Granulomatosis Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Pulmonary Disease Lung Parenchyma Diagnostic Evaluation Laboratory Data Imaging Management Prognosis and Conclusion Kawasaki Disease Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Pulmonary Disease Lung Parenchyma and Pleural Disease Diagnostic Evaluation Laboratory Data Imaging Management Prognosis and Conclusion Isolated Pauci-immune Pulmonary Capillaritis Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Diagnostic Evaluation Laboratory Data Bronchoscopy and Lung Biopsy Imaging Management Prognosis and Conclusion Part II: Granulomatosis with Polyangiitis Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Pulmonary Disease Airway Disease Diagnostic Evaluation Diagnostic Imaging Chest Radiography CT and HRCT Nodules and Masses Ground Glass Opacities and Consolidation Bands and Linear Opacities Additional Findings Evolution Over Time Tracheobronchial Abnormalities Role of PET and 18F-FDG PET/CT Cardiac Involvement in GPA Differential Diagnosis Pathological Features Management Induction Maintenance Plasma Exchange Other Considerations Prognosis and Conclusions Eosinophilic Granulomatosis with Polyangiitis Introduction Incidence and Risk Factors Pathogenesis Diagnostics Laboratory Data Diagnostic Imaging Radiography CT Clinical Presentation Pulmonary Disease Vasculature Management Microscopic Polyangiitis Introduction Incidence and Risk factors Pathogenesis Clinical Presentation Pulmonary Disease Diagnostic Evaluation Laboratory Data Imaging Management Prognosis and Conclusions Antiglomerular Basement Membrane Disease Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Diagnostic Evaluation Laboratory Data Pulmonary Disease Management Prognosis and Conclusion Pulmonary Hemosiderosis Introduction Incidence and Risk Factors Pathogenesis Clinical Presentation Diagnostic Evaluation Laboratory Data Bronchoscopy Diagnostic Imaging Chest Radiograph and Computed Tomography of IPH Other Imaging Modalities Differential Diagnosis Histopathological Features Management Prognosis and Conclusion References 22: Emphysema and Cystic Lung Disease Introduction Emphysema Epidemiology Pathogenesis Risk Factors Clinical Features Diagnosis Treatment Pharmacotherapy Alpha 1-Antitrypsin Deficiency Epidemiology Pathophysiology Risk Factors Clinical Presentation and Diagnosis Treatment Birt-Hogg-Dube Syndrome Epidemiology Pathogenesis Clinical Presentation Diagnosis Lymphoid Interstitial Pneumonia Pathogenesis Clinical Features Diagnosis Treatment Neurofibromatosis-Related Lung Disease Epidemiology Risk Factors Pathophysiology Clinical Presentation Diagnosis Treatment Ehlers-Danlos Syndrome Epidemiology Pathophysiology Clinical Features Diagnosis Treatment Marfans Syndrome Epidemiology Pathogenesis Clinical Characteristics Diagnosis Treatment and Management Bronchogenic Cysts Epidemiology Clinical Features Diagnosis Treatment Cystic Fibrosis Epidemiology Pathophysiology Clinical Features Diagnosis Treatment Cystic Adenomatoid Malformations Epidemiology Pathophysiology Clinical Features Diagnosis Pathological Features Treatment Light Chain Deposition Disease Epidemiology Pathophysiology Clinical Features Diagnosis Treatment References 23: Airspace Diseases and Pulmonary Nodules Cryptogenic Organizing Pneumonia Introduction Clinical Presentation Diagnostic Imaging Chest Radiograph CT and HRCT Multifocal Alveolar Disease Nodular Form Mixed Ground Glass and Solid Lesions Perilobular Pattern Linear and Band Opacities Fibrotic OP Other Findings Evolution of the Imaging Findings Differential Diagnosis Pathological Features Differential Diagnosis Eosinophilic Lung Disorders Introduction Etiology Clinical Features Chronic Eosinophilic Pneumonia Clinical Findings Imaging of Acute Eosinophilic Pneumonia (AEP) Chest Radiograph CT Chronic Eosinophilic Pneumonia (CEP) Chest Radiograph CT Differential Diagnosis Histopathological Features Eosinophilic Pneumonias of Determined Origin Eosinophilic Pneumonia of Parasitic Origin Tropical Eosinophilia Ascaris Pneumonia Strongyloides stercoralis Infection Allergic Bronchopulmonary Aspergillosis (ABPA) Clinical Presentation Lipoid Pneumonia (Paraffinoma) Pulmonary Sarcoidosis Introduction Clinical Features Staging and Prognosis Diagnostic Imaging Chest Radiography Lymph Node Enlargement Parenchymal Lung Disease Airway Involvement CT and HRCT Reticulonodular Pattern Macronodules and Mass-Like Consolidation Fibrotic Changes Pleural Involvement Lymphadenopathy Large and Small Airway Involvement Pulmonary Hypertension Nuclear Medicine Magnetic Resonance Imaging Cardiac Sarcoidosis (CS) Echocardiography CT Cardiac Magnetic Resonance (CMR) Nuclear Medicine Differential Diagnosis Cardiac Sarcoid Summary Diagnostic Approach Prognosis Factors Major Adverse Effects of Advanced (End-stage) Lung Sarcoidosis Pulmonary Hypertension Chronic Pulmonary Aspergillosis Monitoring and Decision of Treatment Histopathological Features References 24: Infectious Diseases That May Mimic Lung Cancer Introduction Bacteria Actinomycetes Non-Tuberculosis Mycobacteria (NTM) Mycobacteria Tuberculosis (MTB) Endemic Mycosis (Fungal) Blastomycosis Coccidioidomycosis Cryptococcosis Histoplasmosis Paracoccidioidomycosis (PCM) Invasive Fungi Aspergillosis Zygomycosis (Mucormycosis) Parasites Dirofilariasis Immitis Echinococcus Paragonimiasis References 25: Miscellaneous Conditions Hermansky-Pudlak Syndrome (HPS) Niemann-Pick (NP) and Gaucher Diseases Dyskeratosis Congenita (DC) Pulmonary Alveolar Microlithiasis (PAM) Diagnostic Imaging Pathological Features Pulmonary Alveolar Proteinosis (Pulmonary Alveolar Lipoproteinosis) Clinical Presentation Diagnostic Imaging Chest Radiograph CT Primary Versus Secondary form of PAP Evolution of Imaging Findings Differential Diagnosis Pathological Features Pulmonary Hyalinizing Granuloma (PHG)/IgG4-Sclerosing Disease Placental Transmogrification References 26: Iatrogenic Conditions Iatrogenic Conditions: Pulmonary Drug Toxicity Chest Radiograph CT and HRCT Imaging Patterns of Drug-Induced Pulmonary Reaction Pulmonary Edema and Diffuse Alveolar Damage (DAD) Spectrum Organizing Pneumonia (OP): Formerly “Bronchiolitis Obliterans- Organizing Pneumonia (BOOP)” Interstitial Lung Disease with or Without Fibrosis NSIP and UIP Eosinophilic Pneumonia (EP) Hypersensitivity Pneumonitis (HP) Diffuse Alveolar Hemorrhage (DAH) Drug-Induced Sarcoid-like Reaction (DSIR) Pulmonary Hypertension Drug-Related Pleural Abnormalities (DRPA) Imaging Following Targeted Therapy and Immunotherapy Targeted Therapy Angiogenesis Inhibitors Imaging of Tumor Response with Targeted Therapy Imaging of Targeted Therapy Toxicity Immunotherapy Response Assessment Immune-Related Adverse Events Imaging of Immune-Related Adverse Events (irAE) Pathological Response to Therapy Imaging Patterns Following Radiation Therapy Pathological Features of Radiation Pneumonitis References 27: Pathology of the Lung Allograft Introduction Primary Graft Dysfunction Anastomotic Complication Hyperacute Rejection Acute Rejection Acute Cellular Rejection Small Airway Inflammation/Lymphocytic Bronchiolitis Antibody-Mediated Rejection Chronic Lung Allograft Dysfunction (CLAD) Bronchiolitis Obliterans Syndrome (BOS)/Obliterative Bronchiolitis (OB) Restrictive Allograft Syndrome (RAS)/Pleuroparenchymal Fibroelastosis (PPFE) Chronic Vascular Rejection Infection in Lung Allograft Bacterial Infection Viral Infection Fungal Infection Posttransplant Lymphoproliferative Disorder (PTLD) Recurrence of Native Disease References Index