Interstitial Lung Disease, 5th edition, is a complete volume addressing interstitial lung diseases with a multidisciplinary approach to diagnosis covering a wide array of disorders such as sarcoidosis, asbestosis, hypersensitivity pneumonitis, drug induced lung disease, connective tissue disease, and pulmonary vasculitis. The editors cover clinical, pathologic, radiologic, and physiologic evaluation of the patient with ILD and provide basic pathobiology and complete descriptions of individual disease entities. Interstitial Lung Disease is divided into two sections. The Clinical Approach section provides the basis for recognizing the key features that allow a specific diagnosis. Basic Mechanisms emphasizes the many advances in genetics and cellular and molecular biology that have expanded our understanding of the biological processes involved in the pathogenesis of interstitial lung diseases.

Interstitial Lung Disease, 5th edition, is a complete volume addressing interstitial lung diseases with a multidisciplinary approach to diagnosis covering a wide array of disorders such as sarcoidosis, asbestosis, hypersensitivity pneumonitis, drug induced lung disease, connective tissue disease, and pulmonary vasculitis. The editors cover clinical, pathologic, radiologic, and physiologic evaluation of the patient with ILD and provide basic pathobiology and complete descriptions of individual disease entities.Interstitial Lung Disease is divided into two sections. The Clinical Approach section provides the basis for recognizing the key features that allow a specific diagnosis. Basic Mechanisms emphasizes the many advances in genetics and cellular and molecular biology that have expanded our understanding of the biological processes involved in the pathogenesis of interstitial lung diseases.

ContributorsPrefaceCLINICAL APPROACHCHAPTER 1Approach to the Evaluation and Diagnosis of Interstitial Lung Disease by Gregory P. Cosgrove and Marvin I. SchwarzClassification, Epidemiology, Pathogenesis, Clinical Evaluation, Treatment and Follow-Up, ReferencesCHAPTER 2Anatomic Distribution and Histopathologic Patterns of Interstitial Lung Disease by Kevin O. Leslie, Thomas V. Colby, and David A. LynchAssessment of Diffuse Pulmonary Disease According to Anatomic Distribution of Lesions, Histologic Evaluation of Interstitial Diseases by Identifying a Reaction Pattern of Lung, Injury and Repair, More Specific Histologic Features of Interstitial, Lung Disease, An Apparently Normal Biopsy in the Setting of Interstitial Lung Disease, Clinicopathologic Correlation in Interstitial Lung Disease, Correlation of Pathology and Radiology in the Study of Interstitial Lung Disease, Examples of Specific Interstitial Lung Diseases, Summary, ReferencesCHAPTER 3Physiology of Interstitial Lung Disease by Chris M. Parker, Michael F. Fitzpatrick, and Denis E. O’DonnellPulmonary Mechanics, Gas Exchange Abnormalities, Pulmonary Hypertension and Cardiac Dysfunction, Exercise Pathophysiology, Sleep and Interstitial Lung Disease, Mechanisms of Dyspnea, Clinical Utility of Physiologic Measurement, Summary , ReferencesCHAPTER 4Pulmonary Hypertension Complicating Interstitial Lung Disease by Joseph P. Lynch III, John A. Belperio, Michael C. Fishbein, and David A. ZismanDiagnosis of PAH, Incidence and Importance of PAH In Interstitial Lung Diseases, Pathogenesis of PAH, Therapy of PAH, Validity of End Points for Clinical Trials, ReferencesCHAPTER 5Imaging of Diffuse Parenchymal Lung Diseases by David A. LynchTechniques, CT Findings in Normal Patients, Pattern Recognition in Diffuse Lung Diseases, CT Features of Specific Interstitial Lung Diseases, Clinical Applications of HRCT in Diffuse Lung Disease, Summary, ReferencesCHAPTER 6Bronchoalveolar Lavage by Ulrich Costabel and Josune GuzmanTechnical Principles, Bronchoalveolar Lavage in Healthy Adults, Bronchoalveolar Lavage in Interstitial Lung Disease, Bronchoalveolar Lavage as an Adjunct to Diagnosis, Bronchoalveolar Lavage in the Differential, Diagnosis of Diffuse Parenchymal Lung Disease, Assessing Disease Activity and Prognosis, ReferencesCHAPTER 7Childhood Interstitial Lung Disease by Leland L. Fan and Megan K. DishopClassification, Clinical Presentation, Diagnostic Evaluation, Treatment, Outcome, Summary, ReferencesCHAPTER 8Novel Therapies for Idiopathic Pulmonary Fibrosis by Amy L. Olson and Ganesh RaghuInflammation, Fibrogenesis, or Both, Fibroblastic Foci and the Fibroblastic Reticulum, Source of Fibroblasts and Myofibroblasts: Epithelial-Mesenchymal Transformation and Extrapulmonary Progenitor Cells, Recent Clinical Trials 199, ReferencesBASIC MECHANISMSCHAPTER 9Genetics of Interstitial Lung Diseases by William E. Lawson and James E. LoydApproaches to Genetic Studies, Familial Interstitial Pneumonia, Genetic Causes of IIP, Genetic Polymorphism Associations in IIP, Interleukin-1 Receptor Antagonist and Tumor Necrosis Factor-Alpha, Familial Clustering and Associated Polymorphisms in Other ILDs, Defined Genetic Syndromes with Lung Involvement, ReferencesCHAPTER 10Genomics of Interstitial Lung Disease by Thomas J. Richards and Naftali KaminskiConceptual Understanding of Genome–Scale Studies, New Insights Derived From Gene Expression Studies of Ild, Summary, ReferencesCHAPTER 11Virus-Related Interstitial Lung Disease by Ana L. Mora and Jesse RomanViruses in Interstitial Lung Disorders, Viruses and Interstitial Lung Disorders in Other Mammals, Mechanisms of Virus-Induced Interstitial Lung Disease, Animal Models of Virus-Induced Lung Fibrosis, Implications for Therapy, Research Needs, ReferencesCHAPTER 12Animal Models of Interstitial Lung Disease by Felix Chua, Geoffrey J. Laurent, Jack Gauldie, and Martin R. J. KolbPathogenetic Basis of Pulmonary Fibrosis, Pulmonary Fibrosis in Animals and Animal Models, In Vivo Evaluation of Antifibrotic Compounds, Prominence of IPF/UIP as a “Model” Disease, Future Directions: Striking the Right Balance Between Utility and Expectations, ReferencesCHAPTER 13Inflammation in the Pathogenesis of Interstitial Lung Diseases by Robert M. Tighe and Paul W. NobleInflammatory and Immune Cells Implicated in Interstitial Lung Diseases, Animal Models of Interstitial Lung Disease, Summary, ReferencesCHAPTER 14Coagulation and Fibrinolysis in Lung Injury and Repair by Joao A. M. de Andrade and Mitchell A. OlmanCoagulation and Fibrinolytic Cascades, Coagulation and Fibrinolysis in Lung Injury and Repair, Crosstalk Between Coagulation/Fibrinolytic Cascades and Inflammation, Anticoagulant and Fibrinolytic Therapies for Lung Injury and Repair (ALI and IPF), ReferencesCHAPTER 15Role of the Pulmonary Epithelium in Pathogenesis of Interstitial Lung Disease by Kevin K. Kim and Harold A. ChapmanDistal Lung Epithelial Cell Development and Function, Epithelial Cells in Initiation of Interstitial Lung Diseases, Dynamic Epithelial Cell Phenotypes: Reepithelization Versus Progressive Fibrosis, Overview and Possible Clinical Correlations. ReferencesCHAPTER 16Cytokine Biology and the Pathogenesis of Interstitial Lung Disease by Michael P. Keane, John A. Belperio, and Robert M. StrieterEarly–Response Proinflammatory Cytokines, Growth Factors, Fibrotic Cytokines, Chemotactic Cytokines, Summary, ReferencesCHAPTER 17Role and Origin of the Fibroblast in the Pathogenesis of Interstitial Lung Disease by Victor J. Thannickal, Galen B. Toews, and Sem H. PhanFibroblasts: Effector Cells in Fibrogenesis, Origins of Tissue Fibroblasts, Emergence of the Myofibroblast Phenotype, Fate of Tissue Myofibroblasts, Summary, ReferencesCHAPTER 18Immunologic Events in the Development of Interstitial Lung Disease: The Paradigm of Sarcoidosis by Gianpietro Semenzato, Monica Facco, and Carlo AgostiniAntigenic and Genetic Factors Involved in Sarcoid Inflammation, How Factors Involved in the Initiation and Maintenance of Inflammatory Responses in ILD, Granuloma Formation and Evolution, Mechanisms Leading to Pulmonary Fibrosis, Molecular Targets for Immunoppressive Drugs in Sarcoidosis, Summary, ReferencesCHAPTER 19Sarcoidosis by Paolo Spagnolo, Paul Cullinan, and Roland M. du BoisMilestones, Pathogenesis, Pathology, Clinical Presentation, Investigations, Treatment, Management Strategies: A Summary, Summary, ReferencesCHAPTER 20The Health Effects of Silica and Coal Dust Exposures by Daniel E. BanksForms of Silica, Occupational Exposure to Silica, Biologic Consequences of Silica Inhalation, Pathology of Silicosis, Radiographic Patterns of Silicosis and “Types” of Silicosis, Symptoms and Physical Findings of Silicosis, Pulmonary Function in Silica-Exposed Workers and Those with Silicosis, ILO Classification and Chest Imaging in Silicosis, Diagnosis of Silicosis, Complications of Silicosis, Treatment of Silicosis, Prevention of Silicosis, Health Effects of Coal Dust Exposure, History, Formation and Characteristics of Coal, Techniques of Mining Coal, Epidemiology, Industrial Bronchitis, Dust Exposure and Changes in Lung Function, Smoking, Dust Exposure, and Airways Obstruction, Incidence and Prevalence of CWP, Mortality Studies, Pathogenesis, Clinical Features of CWP and Imaging, Pathology, Management, ReferencesCHAPTER 21Asbestosis and Asbestos-Induced Pleural Fibrosis by Mark P. Steele and David A. SchwartzCharacteristics of Asbestos, Clinical Aspects of Asbestos-Induced Lung Disease, Cellular and Molecular Effects of Asbestos, Mechanisms of Asbestos-Induced Lung Disease, Prognosis and Treatment, ReferencesCHAPTER 22Beryllium Disease by Andrew P. Fontenot, Lee S. Newman, and Lisa A. MaierHistoric Perspective, Exposure to Beryllium, Pathology, Toxicology and Immunopathogenesis, Clinical Features, Natural History of Chronic Beryllium Disease, Treatment and Follow-Up, Lung Cancer, Surveillance, Prevention, ReferencesCHAPTER 23Interstitial Lung Disease and Other Occupational Exposures (Hard Metal Pneumonocosis) by Lisa A. Maier, Craig S. Glazer, and Karin PachecoPathophysiology, Cobalt-Induced Interstitial Lung Disease, Other Metal-Induced Granulomatous Disease, Other Nongranulomatous Metal-Induced ILDs, Uncommon Pneumoconioses, Emerging Occupational ILDs, Summary, Acknowledgment, ReferencesCHAPTER 24Hypersensitivity Pneumonitis by Moisés SelmanHistorical Background, Pathogenesis, Antigens, Prevalence, Smoking and Hypersensitivity Pneumonitis, Clinical Features, Imaging Approaches, Pulmonary Function Tests, Pulmonary Circulation, Laboratory Tests, Bronchoalveolar Lavage, Differential Diagnosis, Inhalation Challenge, Histology, Diagnostic Foresights, Bronchial Hyperreactivity, Asthma, and Hypersensitivity Pneumonitis, Treatment, Prognosis and Survival, ReferencesCHAPTER 25Interstitial Lung Disease from Drugs, Biologics, and Radiation by Philippe CamusCausal Drugs, Epidemiology, Risk Factors, Diagnosis, Clinical-Imaging and Pathologic Patterns of DILD, Infiltrative Lung Disease Due to Specific Drugs or Agents, Conclusion, Acknowledgments, ReferencesCHAPTER 26Connective Tissue Diseases by Talmadge E. King, Jr., Eunice J. Kim, and Brent W. KinderGeneral Considerations, Management of CTD-Associated ILD, Rheumatoid Arthritis, Systemic Lupus Erythematosus, Progressive Systemic Sclerosis (Scleroderma), Polymyositis and Dermatomyositis, Sjögren’s Syndrome, Mixed Connective Tissue Disease, Undifferentiated Connective Tissue Disease, Ankylosing Spondylitis, Psoriatic Arthritis, Behçet’s Disease, Relapsing Polychondritis, ReferencesCHAPTER 27Pulmonary Vasculitis by Ulrich SpecksNomenclature, Disease Classifications, and Definitions, Epidemiology, Wegener’s Granulomatosis, Microscopic Polyangiitis, Churg–Strauss Syndrome, ANCAs, Giant Cell Arteritis, Takayasu’s Arteritis, Behçet’s Disease, Pulmonary Vasculitis Associated with Connective Tissue Disorders, Necrotizing Sarcoid Granulomatosis, ReferencesCHAPTER 28Diffuse Alveolar Hemorrhage by Abigail R. Lara, Stephen K. Frankel, Marvin I. SchwarzHistology, Clinical Presentation, Diagnosis, Wegener’s Granulomatosis, Microscopic Polyangiitis, Isolated Pulmonary Capillaritis, Systemic Lupus Erythematosus, Other Collagen Vascular Diseases, Antiphospholipid Syndromes, Henoch-Schönlein Purpura and Immunoglobulin A Nephropathy, Behçet’s Disease , Cryoglobulinemia, Idiopathic Glomerulonephritis, Acute Lung Allograft Rejection, Miscellaneous Causes of Pulmonary Capillaritis, Goodpasture’s Syndrome (Anti–Basement Membrane Antibody Disease), Idiopathic Pulmonary Hemosiderosis, Penicillamine and Noncytotoxic Drugs, Trimellitic Anhydride and Pyromellitic Dianhydride, Mitral Stenosis, Coagulation Disorders, Diffuse Alveolar Damage and Diffuse Alveolar Hemorrhage, Lymphangioleiomyomatosis and Tuberous Sclerosis, Pulmonary Veno-Occlusive Disease, Pulmonary Capillary Hemangiomatosis, Obstructive Sleep Apnea, Negative Pressure Pulmonary Hemorrhage , Fibrillary Glomerulonephritis, Malignancy and Diffuse Alveolar Hemorrhage, ReferencesCHAPTER 29Eosinophilic Pneumonias by Jean-François Cordier and Vincent CottinThe Eosinophil, Definition and Diagnostic Criteria of Eosinophilic Pneumonia, Description and Classification of the Eosinophilic Pneumonias, Pathology, Eosinophilic Pneumonias of Undetermined Origin, Eosinophilic Pneumonias of Determined Origin, Summary, ReferencesCHAPTER 30Idiopathic Pulmonary Fibrosis by Talmadge E. King, Jr.Classification of the Idiopathic Interstitial Pneumonias, Epidemiology, Etiology and Pathogenesis, Clinical Findings, Laboratory Findings, Bronchoalveolar Lavage, Physiologic Findings, Lung Imaging Studies, High-Resolution Computed Tomography, Histopathologic Findings, Confirming the Diagnosis of IPF, Natural History, Management and Outcome, Other Management Issues in Patients with IPF, Risk Factors for Progressive Disease and Survival in IPF, ReferencesCHAPTER 31Nonspecific Interstitial Pneumonia by Fernando J. Martinez and Kevin R. FlahertyHistorical Perspective, Etiology, Pathogenesis, Clinical Assessment, Pathology, Prognosis, Management and Treatment, ReferencesCHAPTER 32Smoking-Related Interstitial Lung Diseases by Robert Vassallo, Andrew H. Limper, and Jay H. RyuRespiratory Bronchiolitis-Associated ILD, Desquamative Interstitial Pneumonia, Pulmonary Langerhans’ Cell Histiocytosis, Acute Eosinophilic Pneumonia, Summary, ReferencesCHAPTER 33Organizing Pneumonia by Talmadge E. King, Jr.Secondary Forms of Organizing Pneumonia, Cryptogenic Organizing Pneumonia, ReferencesCHAPTER 34Acute Interstitial Pneumonia by Harold R. Collard and Kevin K. BrownDefinition and Differential Diagnosis, Etiology and Pathobiology, Epidemiology and Clinical Features, Management, Natural History and Prognosis, ReferencesCHAPTER 35Bronchiolitis by Talmadge E. King, Jr.General Considerations, Clinical Syndromes Associated with Bronchiolitis, ReferencesCHAPTER 36Lymphoplasmocytic Infiltrations of the Lung by Gregory P. Cosgrove and Marvin I. SchwarzEtiology, Clinical Presentation, Prognosis and Treatment, Pathogenesis, Plasma Cell Infiltrations of the Lung, Angioimmunoblastic T-Cell Lymphoma, Lymphomatoid Granulomatosis (Angiocentric Lymphoma), ReferencesCHAPTER 37Lymphangioleiomyomatosis by Arnold S. Kristof and Joel MossEpidemiology, Etiology, Pathology, Clinical Manifestations, Laboratory Tests, Imaging Studies, Pulmonary Function Tests, Pathologic-Radiologic-Physiologic Correlation, Diagnosis, Treatment, Natural History and Prognosis, Acknowledgments, ReferencesCHAPTER 38Pulmonary Alveolar Proteinosis by Maurizio Luisetti and Bruce C. TrapnellPulmonary Surfactant: Composition, Function, and Homeostasis, Pulmonary Alveolar Proteinosis, Surfactant Metabolic Dysfunction Disorders, Summary and Future Research, ReferencesCHAPTER 39Miscellaneous Interstitial Lung Diseases by Tristan J. Huie and Marvin I. SchwarzRespiratory Tract Amyloidosis, Light Chain Deposition Disease, Agnogenic Myeloid Metaplasia, Common Variable Immunodeficiency, Gaucher’s Disease, Niemann-Pick Disease, Fabry’s Disease, Hermansky-Pudlak Syndrome, Lysinuric Protein Intolerance, Telomerase-Associated Pulmonary Fibrosis of the Lung, Erdheim-Chester Disease, Idiopathic Pleuroparenchymal Fibroelastosis, Birt-Hogg-Dubé Syndrome, Nephrogenic Systemic Fibrosis, Neurofibromatosis (Von Recklinghausen’s Disease), Hypocalciuric Hypercalcemia and ILD, Pulmonary Calcification, Pulmonary Alveolar Microlithiasis, Diseases of Autoimmunity Associated with ILD, Granulomatous Pneumonitis Induced by Bacille Calmette-Guérin, Pulmonary Fibrosis as a Consequence of the Acute Respiratory Distress Syndrome, ILD Following Pulmonary Infections, ILD Complicating Hematopoietic Stem Cell Transplantation, ILD Following Aspiration, Lipoid Pneumonia, Bronchioloalveolar Cell Carcinoma, Lymphangitic Carcinomatosis, ReferencesIndex