BOOK
Murray & Nadel's Textbook of Respiratory Medicine E-Book
Robert C Mason | John F. Murray | Jay A. Nadel | Michael Gotway | V.Courtney Broaddus | Joel D Ernst | Talmadge E King | Stephen C. Lazarus | Arthur Slutsky
(2015)
Additional Information
Book Details
Abstract
Ideal for fellows and practicing pulmonologists who need an authoritative, comprehensive reference on all aspects of pulmonary medicine, Murray and Nadel’s Textbook of Respiratory Medicine offers the most definitive content on basic science, diagnosis, evaluation and treatment of the full spectrum of respiratory diseases.
- Full-color design enhances teaching points and highlights challenging concepts.
- Understand clinical applications and the scientific principles of respiratory medicine.
- Detailed explanations of each disease entity allow you to work through differential diagnoses.
- Key Points and Key Reading sections highlight the most useful references and resources for each chapter.
- An expanded sleep section now covers four chapters and includes control of breathing, consequences of sleep disruption, as well as obstructive and central apnea.
- New chapters in the Critical Care section cover Noninvasive Ventilation (NIV) and Extracorporeal Support of Gas Exchange (ECMO). New chapters focusing on diagnostic techniques now include Invasive Diagnostic Imaging and Image-Guided Interventions and Positron Emission Tomography, and a new chapter on Therapeutic Bronchoscopy highlights the interventional role of pulmonologists.
Table of Contents
Section Title | Page | Action | Price |
---|---|---|---|
9780323261937v1_WEB.pdf | 1 | ||
Front Cover | 1 | ||
IFC_Expert Consult Pin Code Ad | 2 | ||
Murray & Nadel’s Textbook of Respiratory Medicine | 5 | ||
Copyright Page | 6 | ||
Dedication for Sixth Edition | 7 | ||
Contributors | 8 | ||
Preface to the Sixth Edition | 22 | ||
Preface to the First Edition | 23 | ||
Table Of Contents | 25 | ||
Videos | 29 | ||
1 Scientific Principles of Respiratory Medicine | 35 | ||
A Anatomy and Development of the Respiratory Tract | 37 | ||
1 Anatomy of the Lungs | 37 | ||
Introduction | 37 | ||
Gross and Subgross Organization | 37 | ||
Airways | 40 | ||
Bronchial Circulation | 43 | ||
Pulmonary Circulation | 43 | ||
Terminal Respiratory Units | 46 | ||
Lymphatics | 50 | ||
Innervation | 50 | ||
The Pleural Space and Pleuras | 51 | ||
Comparison of the Lung of Mice and Humans | 53 | ||
Acknowledgments | 55 | ||
Key Readings | 55 | ||
References | 56 | ||
2 Lung Growth and Development | 61 | ||
Introduction | 61 | ||
Stages of Lung Development | 61 | ||
Tissue Interactions and Lung Development | 64 | ||
Molecular Regulation of Lung Development | 65 | ||
Diffusible Mediators of Lung Development | 65 | ||
Fibroblast Growth Factors and Fibroblast Growth Factor Receptors | 65 | ||
Retinoic Acid | 65 | ||
Sonic Hedgehog | 66 | ||
Transforming Growth Factor-β Superfamily | 66 | ||
Wnts and β-Catenin | 66 | ||
Platelet-Derived Growth Factor | 67 | ||
Vascular Endothelial Growth Factor | 67 | ||
Glucocorticoids | 67 | ||
Transcriptional Regulation of Lung Development | 67 | ||
NKX2-1 | 67 | ||
GLI Genes | 68 | ||
FOX Family | 68 | ||
GATA6 | 68 | ||
SOX Family | 68 | ||
Posttranscriptional Gene Regulation in Lung Development | 68 | ||
Key Readings | 70 | ||
References | 71 | ||
3 Genetics of Lung Disease | 75 | ||
Introduction | 75 | ||
Scope of the Problem | 76 | ||
Potential Impact of Human Genetics | 76 | ||
Molecular Characterization of Genetic Variation | 76 | ||
Genomic Maps | 76 | ||
Comparative Genomics | 78 | ||
Public Databases | 78 | ||
Genetic Epidemiology | 79 | ||
Positional Cloning | 79 | ||
Linkage Studies | 79 | ||
Association Studies | 79 | ||
Genome-Wide Association Studies | 80 | ||
Gene by Environment Interaction | 80 | ||
Epigenetics | 80 | ||
Application to Pulmonary Diseases | 83 | ||
Asthma | 83 | ||
Chronic Obstructive Pulmonary Disease | 83 | ||
Acute Respiratory Distress Syndrome | 83 | ||
Lung Cancer | 84 | ||
Fibrosing Idiopathic Interstitial Pneumonia | 85 | ||
The Path Forward | 86 | ||
Key Readings | 86 | ||
References | 87 | ||
B Respiratory Physiology | 90 | ||
4 Ventilation, Blood Flow, and Gas Exchange | 90 | ||
Introduction | 90 | ||
Ventilation | 90 | ||
Lung Volumes | 91 | ||
Functional Residual Capacity, Residual Volume, and Total Lung Capacity | 91 | ||
Total and Alveolar Ventilation | 92 | ||
Total Ventilation | 92 | ||
Alveolar Ventilation | 92 | ||
Anatomic Dead Space | 93 | ||
Physiologic Dead Space | 93 | ||
Inequality of Ventilation | 94 | ||
Topographic Inequality | 94 | ||
Airway Closure | 94 | ||
Nontopographic Inequality | 94 | ||
Blood Flow | 95 | ||
Pressures of the Pulmonary Circulation | 95 | ||
Pressure Inside Blood Vessels | 95 | ||
Pressures Outside Blood Vessels | 96 | ||
Pulmonary Vascular Resistance | 96 | ||
Pressure-Flow Relations | 97 | ||
Effect of Lung Volume | 98 | ||
Other Factors Affecting Pulmonary Vascular Resistance | 99 | ||
Distribution of Pulmonary Blood Flow | 99 | ||
Normal Distribution | 99 | ||
Three-Zone Model for the Distribution of Blood Flow | 99 | ||
The Effect of Lung Volume on the Distribution of Blood Flow—Zone 4 | 100 | ||
Other Factors Affecting the Distribution of Blood Flow | 100 | ||
Abnormal Patterns of Blood Flow | 101 | ||
Active Control of the Pulmonary Circulation | 101 | ||
Hypoxic Pulmonary Vasoconstriction | 101 | ||
Other Physiologic Substances Affecting the Pulmonary Circulation | 103 | ||
Damage to Pulmonary Capillaries by High Wall Stresses | 103 | ||
Nonrespiratory Functions of the Pulmonary Circulation | 103 | ||
Blood-Gas Transport | 104 | ||
Oxygen | 104 | ||
Carbon Dioxide | 105 | ||
Gas Exchange | 106 | ||
Causes of Hypoxemia | 107 | ||
Hypoventilation | 107 | ||
Diffusion Limitation | 109 | ||
Oxygen Uptake along the Pulmonary Capillary | 109 | ||
Reaction Rates with Hemoglobin | 110 | ||
Diffusing Capacity | 110 | ||
Shunt | 112 | ||
Ventilation-Perfusion Relationships | 113 | ||
Gas Exchange in a Single Lung Unit | 113 | ||
Pattern in the Normal Lung | 114 | ||
Traditional Assessment of Ventilation-Perfusion Inequality | 115 | ||
Distributions of Ventilation-Perfusion Ratios | 116 | ||
Multiple Inert Gas Elimination Technique. | 117 | ||
Distribution in Normal Subjects. | 118 | ||
Distributions in Lung Disease. | 118 | ||
Ventilation-Perfusion Inequality and Carbon Dioxide Retention | 119 | ||
Effect of Changes in Cardiac Output on Gas Exchange in the Presence of Ventilation-Perfusion Inequality | 120 | ||
Oxygen Sensing | 120 | ||
Key Readings | 121 | ||
References | 122 | ||
5 Respiratory System Mechanics and Energetics | 125 | ||
Introduction | 125 | ||
Terminology | 125 | ||
Flow | 125 | ||
Volume | 125 | ||
Pressure | 126 | ||
The Combined Gas Law | 126 | ||
Compliance, Resistance, and Time Constants | 127 | ||
Respiratory Mechanics in Static Conditions | 127 | ||
Elastic Recoil of the Lungs | 127 | ||
Lung Connective Tissue | 128 | ||
Alveolar Surface Forces and Surfactant | 128 | ||
Hysteresis and Stress Adaptation | 128 | ||
Recruitment Maneuvers in Acute Respiratory Distress Syndrome | 129 | ||
Elastic Recoil of the Chest Wall | 129 | ||
Respiratory Muscles (see Chapter 97) | 129 | ||
Fatigue of Respiratory Muscles | 130 | ||
Respiratory Muscle Atrophy during Positive-Pressure Mechanical Ventilation | 131 | ||
Chest Wall Compliance (see Chapter 98) | 131 | ||
Integration of Lung and Chest Wall Mechanics | 131 | ||
Calculation of Total Respiratory System Compliance from Lung and Chest Wall Compliance | 132 | ||
Clinical Applications | 132 | ||
The Impact of Positive End-Expiratory Pressure on Pleural Pressure | 132 | ||
Plateau Pressures in Patients Receiving Positive-Pressure Mechanical Ventilation | 133 | ||
Stress and Strain | 133 | ||
The Respiratory System in Dynamic Conditions | 133 | ||
Resistive Work Due to Gas Flow Through Airways | 133 | ||
Laminar versus Turbulent Flow | 133 | ||
The Reynolds Number | 135 | ||
Clinical Effects of Heliox | 135 | ||
Flow Limitation | 135 | ||
Equal Pressure Point Theory (Fig. 5-18). | 136 | ||
The Bernoulli Effect and Wave Speed Theory. | 136 | ||
Other Resistive Work | 137 | ||
Equation of Motion | 137 | ||
Intrinsic PEEP during Positive-Pressure Ventilation of COPD | 137 | ||
Measurement of Static Compliance and Resistance during Mechanical Ventilation | 138 | ||
Energetics and Work of Breathing | 139 | ||
Measuring Work of Breathing Done by a Positive-Pressure Ventilator in a Paralyzed Patient | 139 | ||
Measuring Work of Breathing in a Spontaneously Breathing Patient | 139 | ||
Oxygen Cost of Breathing | 140 | ||
eFigure Image Gallery | 141 | ||
Key Readings | 140 | ||
References | 143 | ||
6 Pulmonary Circulation and Regulation of Fluid Balance | 145 | ||
Introduction | 145 | ||
Anatomy | 145 | ||
General Design | 146 | ||
Bronchial Circulation | 147 | ||
Blood-Gas Interface | 147 | ||
Imaging the Pulmonary Circulation | 148 | ||
Pulmonary Hemodynamics | 145 | ||
Pulmonary Vascular Pressures | 145 | ||
Pulmonary Vascular Resistance | 149 | ||
Vascular Resistance Profile | 150 | ||
Mechanical Effects on Pulmonary Vascular Resistance | 150 | ||
Transmural Pressure. | 150 | ||
Lung Volume. | 150 | ||
Viscosity. | 151 | ||
Pulmonary Vascular Compliance | 151 | ||
Pulmonary Vascular Pressure-Volume Curve | 151 | ||
Changes in Vascular Compliance | 152 | ||
Pulmonary Perfusion | 152 | ||
Distention and Recruitment | 152 | ||
Regional Distribution of Pulmonary Perfusion | 152 | ||
Mechanical Stress and the Lung Circulation | 152 | ||
Shear Stress | 153 | ||
Cyclic Stretch | 153 | ||
Pulmonary Vascular Responses to Hypoxia | 154 | ||
Response Elements | 154 | ||
Neural Control of Pulmonary Vascular Resistance | 156 | ||
Humoral Regulation of Pulmonary Vascular Resistance | 156 | ||
Lung Fluid and Solute Exchange | 156 | ||
Transcapillary Exchange and the Fluid Flux Equation | 156 | ||
Sites of Fluid and Solute Exchange | 157 | ||
Capillary Endothelium | 157 | ||
Alveolar Epithelium | 158 | ||
Alveolar-Capillary Septum | 159 | ||
Pulmonary Lymphatic Vessels | 159 | ||
Lung Interstitium | 159 | ||
Interstitial Pressures | 159 | ||
Composition of Pulmonary Interstitium | 159 | ||
Interstitial Compliance | 159 | ||
Pathogenesis of Pulmonary Edema | 160 | ||
Phases and Sites of Edema Accumulation | 160 | ||
Starling Forces in Pulmonary Edema Formation | 160 | ||
Pulmonary Capillary Pressure and Plasma Osmotic Pressure in Edema Formation | 160 | ||
Lymphatic Insufficiency and Edema Formation | 161 | ||
Safety Factors in Lung Fluid Homeostasis | 161 | ||
Effects of Cellular and Molecular Charge | 161 | ||
Regional Differences in Endothelial Permeability | 161 | ||
Mechanisms of Increased Endothelial Permeability | 162 | ||
Characteristics of Increased Vascular Endothelial Permeability | 162 | ||
Cytoskeletal Alterations | 162 | ||
Intracellular Calcium Shifts and Other Barrier-Regulatory Signals | 163 | ||
Focal Adhesions and Extracellular Matrix Components | 163 | ||
Endothelial Water Permeability and Albumin Transcytosis | 163 | ||
Apoptosis and Endothelial Cell Dysfunction | 164 | ||
Strategies to Reverse Permeability and Restore Barrier Integrity | 164 | ||
Pulmonary Vascular Genomics and Genetics | 165 | ||
Acknowledgments | 165 | ||
eFigure Image Gallery | 166 | ||
Key Readings | 165 | ||
References | 169 | ||
7 Acid-Base Balance | 177 | ||
Fundamental Concepts | 177 | ||
Acid-Base Chemistry | 177 | ||
pH Versus H | 177 | ||
Conjugate Acids and Bases | 177 | ||
Strong Versus Weak Ions | 178 | ||
Buffer Systems. | 178 | ||
Carbon Dioxide and Bicarbonate | 178 | ||
Measurements | 178 | ||
Ventilatory Parameter: Arterial Pco2 | 179 | ||
Metabolic Parameters | 179 | ||
Titrimetry | 179 | ||
Bicarbonate | 179 | ||
Base Excess | 180 | ||
Strong Ion Difference | 181 | ||
Role of the Kidneys | 181 | ||
Nomenclature of Acid-Base Disorders | 182 | ||
Compensations | 184 | ||
Respiratory Acidosis | 184 | ||
Respiratory Alkalosis | 184 | ||
9780323261937v2_WEB | 1459 | ||
Front Cover | 1459 | ||
Murray & Nadel’s Textbook of Respiratory Medicine | 1462 | ||
Copyright Page | 1463 | ||
Dedication for Sixth Edition | 1464 | ||
Contributors | 1465 | ||
Preface to the Sixth Edition | 1479 | ||
Preface to the First Edition | 1480 | ||
Table Of Contents | 1482 | ||
Videos | 1486 | ||
J Neoplasms of the Lung | 1491 | ||
51 Biology of Lung Cancer | 1491 | ||
Introduction | 1491 | ||
Predisposition to Lung Cancer | 1492 | ||
Smoking | 1492 | ||
Genetic Susceptibility and Familial Predisposition | 1492 | ||
Early Events in Lung Tumorigenesis | 1493 | ||
Field Cancerization Effect | 1493 | ||
Genomic Instability | 1493 | ||
Mucosal Response to Injury, the Emergence of Critical Mutations | 1494 | ||
Mutagens | 1496 | ||
Role of Inflammation in Lung Tumorigenesis | 1496 | ||
Role of Viruses in Lung Tumorigenesis | 1496 | ||
Neuroendocrine Tumor Development, Genomics of SCLC | 1497 | ||
Other Molecular Alterations Driving the Tumor Phenotype | 1497 | ||
Chromosomal Changes | 1497 | ||
Epigenetic Alterations of Gene Expression in Lung Cancer | 1498 | ||
DNA Adducts | 1498 | ||
DNA Methylation | 1499 | ||
Histone Deacetylation | 1499 | ||
Regulation by miRNAs | 1499 | ||
Proteomic Alterations | 1499 | ||
Strategies to Deepen Our Understanding of Lung Cancer | 1501 | ||
High-Throughput Profiling Techniques | 1501 | ||
Expression Arrays to Next Generation Sequencing | 1501 | ||
Advanced Proteomic Strategies | 1501 | ||
Molecular Networks—System Biology | 1501 | ||
Translating Lung Cancer Biology to the Clinic | 1502 | ||
Biomarkers | 1502 | ||
Personalized Medicine and Molecular Therapeutics | 1502 | ||
Mechanisms of Resistance | 1504 | ||
Key Readings | 1505 | ||
References | 1506 | ||
52 Epidemiology of Lung Cancer | 1512 | ||
Introduction | 1512 | ||
Patterns | 1512 | ||
Temporal Trends | 1512 | ||
Race and Ethnicity | 1512 | ||
Geographic Patterns | 1513 | ||
LUNG Cancer by Histologic Type | 1514 | ||
The Etiology of Lung Cancer: Overview | 1516 | ||
Environmental and Occupational Agents | 1516 | ||
Smoking (Chapter 46) | 1516 | ||
Overview | 1516 | ||
Quantitative Risks | 1517 | ||
Smoking Cessation | 1517 | ||
The Changing Cigarette and Expanding Marketplace | 1517 | ||
Passive Smoking | 1518 | ||
Diet | 1518 | ||
Environmental Exposures | 1519 | ||
Occupational Exposures | 1519 | ||
Asbestos | 1519 | ||
Radiation | 1519 | ||
High-LET Radiation: Radon. | 1520 | ||
Low-LET Radiation: X-Rays and γ-Rays. | 1520 | ||
Air Pollution | 1520 | ||
Atmospheric Air Pollution | 1520 | ||
Indoor Air Pollution | 1520 | ||
Host Factors | 1521 | ||
Overview | 1521 | ||
Research Findings on the Genetic Basis of LUNG Cancer (Chapter 51) | 1521 | ||
Presence of Human Immunodeficiency Virus | 1522 | ||
Presence of Acquired LUNG Disease | 1523 | ||
Gender | 1523 | ||
Future Directions | 1524 | ||
Key Readings | 1524 | ||
References | 1525 | ||
53 Clinical Aspects of Lung Cancer | 1530 | ||
Introduction | 1530 | ||
Screening for Lung Cancer | 1530 | ||
Presentation | 1532 | ||
Lung Cancer Staging | 1532 | ||
Noninvasive Staging Techniques | 1534 | ||
Chest Radiography | 1534 | ||
Chest Computed Tomography | 1534 | ||
Positron Emission Tomography | 1535 | ||
Magnetic Resonance Imaging | 1536 | ||
Search for Metastatic Disease | 1536 | ||
Adrenal and Hepatic Imaging | 1537 | ||
Brain Imaging | 1537 | ||
Bone Imaging | 1537 | ||
Summary | 1537 | ||
Invasive Diagnostic and Staging Techniques | 1537 | ||
Sputum Cytology | 1538 | ||
Transthoracic Needle Aspiration | 1538 | ||
Fiberoptic Bronchoscopy | 1538 | ||
Submucosal and Peribronchial Lesions | 1538 | ||
Navigational Bronchoscopy | 1538 | ||
Bronchoscopy for Staging Lung Cancer | 1539 | ||
Endoscopic Ultrasound | 1539 | ||
Endobronchial Ultrasound | 1539 | ||
Mediastinoscopy | 1540 | ||
Treatment of Lung Cancer | 1541 | ||
Prognostic Factors for Lung Cancer | 1541 | ||
Non–Small Cell Lung Cancer Treatment by Stage | 1542 | ||
Stage I | 1542 | ||
Stage II | 1543 | ||
Stage IIIA | 1544 | ||
Stage IIIB | 1545 | ||
Stage IV | 1545 | ||
Targeted Therapy | 1545 | ||
Small Cell Lung Cancer | 1546 | ||
Palliative Care | 1547 | ||
Special Considerations In Lung Cancer | 1547 | ||
Superior Sulcus Tumors and Pancoast Syndrome | 1547 | ||
Superior Vena Cava Syndrome | 1548 | ||
Paraneoplastic Syndromes | 1549 | ||
Musculoskeletal Effects | 1549 | ||
Hematologic Effects | 1550 | ||
Hypercalcemia | 1550 | ||
Syndrome of Inappropriate Antidiuretic Hormone Secretion | 1552 | ||
Ectopic Corticotropin Syndrome | 1552 | ||
Neurologic Effects | 1553 | ||
eFigure Image Gallery | 1555 | ||
Key Readings | 1554 | ||
References | 1570 | ||
54 Rare Primary Lung Tumors | 1577 | ||
Introduction | 1577 | ||
Characterization of Rare Lung Tumors | 1577 | ||
Primary Pulmonary Lymphoma and Other Lymphoproliferative Diseases | 1578 | ||
Mucosa-Associated Lymphoid Tissue Lymphoma | 1578 | ||
Lymphomatoid Granulomatosis | 1580 | ||
Other Lymphomas and Lymphoproliferative Diseases | 1580 | ||
Carcinoids and Other Neuroendocrine Tumors | 1581 | ||
Pathologic Classification | 1581 | ||
Carcinoid Tumors | 1581 | ||
Large Cell Neuroendocrine Carcinoma | 1582 | ||
Rare Malignant Primary Pulmonary Epithelial Tumors | 1582 | ||
Mucoepidermoid Carcinoma | 1582 | ||
Pneumoblastoma | 1583 | ||
Sarcomatoid Carcinomas (Other Than Pneumoblastoma) | 1583 | ||
Primary Pulmonary Sarcomas | 1584 | ||
Parenchymal Sarcomas | 1584 | ||
Vascular Sarcomas | 1584 | ||
Intrathoracic Pseudotumors | 1586 | ||
Inflammatory Myofibroblastic Tumor | 1586 | ||
Mediastinal Fibrosis and Hyalinizing Granuloma (see Chapter 84) | 1588 | ||
Other Pseudotumors | 1588 | ||
Borderline Entities | 1589 | ||
Mesenchymal Borderline Disorders | 1589 | ||
Respiratory Tract Papillomatosis | 1589 | ||
Nodular Lymphoid Hyperplasia | 1590 | ||
Amyloidosis | 1590 | ||
Nonamyloidotic Monoclonal Immunoglobulin Deposition Disease | 1591 | ||
Pulmonary Langerhans Cell Histiocytosis | 1591 | ||
Lessons Learned for the Management of Rare Primary Pulmonary Tumors | 1591 | ||
General Issues | 1591 | ||
Distinguishing Primary Tumors From Metastases | 1592 | ||
General Therapeutic Management | 1592 | ||
eFigure Image Gallery | 1593 | ||
Key Readings | 1592 | ||
References | 1618 | ||
55 Metastatic Malignant Tumors | 1622 | ||
Introduction | 1622 | ||
Epidemiology | 1622 | ||
Clinical History | 1622 | ||
Mechanism of Metastasis to the Lung | 1623 | ||
Diagnosis | 1624 | ||
Differential Diagnosis | 1624 | ||
Distinguishing Metastasis From Primary Lung Tumors | 1625 | ||
Pathology | 1625 | ||
Molecular Classification | 1626 | ||
Options for Obtaining a Tissue Diagnosis | 1626 | ||
Bronchoscopy | 1627 | ||
CT-Guided Biopsy | 1627 | ||
Surgery | 1627 | ||
Treatment | 1627 | ||
Multimodality Therapy of Potentially Curable Disease | 1627 | ||
Palliative Care | 1627 | ||
Radiofrequency Ablation of Metastases | 1628 | ||
Surgical Resection of Metastases | 1628 | ||
Special Cases | 1630 | ||
Airway Metastasis | 1630 | ||
Vascular Embolic Metastasis | 1630 | ||
Pleural Metastases (see Chapter 82) | 1631 | ||
eFigure Image Gallery | 1632 | ||
Key Readings | 1631 | ||
References | 1635 | ||
56 Benign Lung Tumors | 1637 | ||
Introduction | 1637 | ||
Clinical Manifestations | 1637 | ||
Benign Epithelial Tumors | 1637 | ||
Papillomas and Adenomas | 1637 | ||
Solitary Papillomas | 1637 | ||
Adenomas | 1638 | ||
Micronodular Pneumocyte Hyperplasia | 1640 | ||
Sclerosing Hemangioma | 1641 | ||
Benign Nonepithelial Lesions | 1642 | ||
Hamartoma and Related Lesions | 1642 | ||
Inflammatory Myofibroblastic Tumor | 1643 | ||
Solitary Fibrous Tumor | 1644 | ||
Meningothelial-Like Nodules and Intrapulmonary Meningioma | 1645 | ||
Miscellaneous | 1646 | ||
Summary | 1646 | ||
eFigure Image Gallery | 1647 | ||
Key Readings | 1646 | ||
References | 1656 | ||
K Disorders of the Pulmonary Circulation | 1660 | ||
57 Pulmonary Thromboembolism | 1660 | ||
Introduction | 1660 | ||
Pathogenesis and Risk Factors | 1662 | ||
Natural History: Deep Venous Thrombosis | 1663 | ||
Natural History: Pulmonary Embolism | 1664 | ||
Clinical Presentation | 1665 | ||
Diagnosis of Venous Thrombosis | 1666 | ||
Contrast Venography | 1666 | ||
Duplex Ultrasonography | 1667 | ||
Magnetic Resonance Imaging | 1668 | ||
Computed Tomography | 1668 | ||
Hemostaseologic Assays | 1668 | ||
Clinical Prediction Rules | 1668 | ||
Diagnosis of Pulmonary Embolism | 1669 | ||
Standard Laboratory Evaluation | 1669 | ||
Echocardiography | 1670 | ||
Ventilation-Perfusion Scanning | 1670 | ||
Computed Tomography Pulmonary Angiography | 1671 | ||
Single-Photon Emission Computed Tomography Ventilation/ Perfusion Scans | 1673 | ||
Lower Extremity Venous Evaluation | 1673 | ||
D-Dimer Testing | 1674 | ||
Pulmonary Angiography | 1674 | ||
Clinical Prediction Rules | 1674 | ||
Prevention of Venous Thromboembolism | 1676 | ||
Low-Dose Unfractionated Heparin | 1676 | ||
Low-Molecular-Weight Heparin | 1677 | ||
Pneumatic Compression Devices | 1677 | ||
Warfarin | 1677 | ||
Fondaparinux | 1677 | ||
Direct Inhibitors of Factor Xa and of Thrombin | 1677 | ||
Management of Venous Thromboembolism | 1678 | ||
Unfractionated Heparin and Low-Molecular-Weight Heparin | 1678 | ||
Fondaparinux | 1679 | ||
Direct Inhibitors of Factor Xa and of Thrombin | 1679 | ||
Inferior Vena Cava Filters | 1680 | ||
Massive Pulmonary Embolism | 1680 | ||
Stratification According to the Risk of Massive Pulmonary Embolism | 1680 | ||
Thrombolytic Therapy | 1681 | ||
Pulmonary Embolectomy | 1681 | ||
Postembolic Prophylaxis | 1682 | ||
Resolution Versus Persistence of Thromboembolism | 1683 | ||
Chronic Thromboembolic Pulmonary Hypertension | 1683 | ||
Diagnosis | 1684 | ||
Treatment | 1686 | ||
Other Forms of Embolism | 1690 | ||
Schistosomiasis | 1690 | ||
Air Embolism | 1690 | ||
Fat Embolism | 1690 | ||
Amniotic Fluid Embolism (see Chapter 96) | 1691 | ||
Septic Embolism | 1691 | ||
Other Emboli | 1691 | ||
eFigure Image Gallery | 1692 | ||
Key Readings | 1689 | ||
References | 1701 | ||
58 Pulmonary Hypertension | 1711 | ||
Introduction | 1711 | ||
Epidemiology | 1711 | ||
Pathology | 1711 | ||
Pathogenesis and Etiology | 1713 | ||
Other Group 1 Conditions | 1713 | ||
Pulmonary Veno-occlusive Disease | 1713 | ||
Pulmonary Arterial Hypertension Associated with Other Conditions | 1713 | ||
Symptoms | 1716 | ||
Physical Findings | 1716 | ||
Diagnosis | 1716 | ||
Echocardiogram | 1718 | ||
Cardiac Catheterization | 1719 | ||
Treatment and Prognosis | 1719 | ||
Supportive Therapies | 1720 | ||
Exercise and Physical Activity | 1720 | ||
Avoidance of Altitude | 1720 | ||
Avoidance of Pregnancy | 1720 | ||
Anticoagulation | 1720 | ||
Warfarin. | 1720 | ||
Factor Xa Inhibitors. | 1721 | ||
Supplemental Oxygen | 1721 | ||
Diuretics | 1721 | ||
Calcium Channel Antagonists | 1721 | ||
Targeted Therapies | 1722 | ||
Prostacyclin Analogues | 1722 | ||
Epoprostenol (Flolan/Veletri). | 1722 | ||
Subcutaneous and Intravenous Treprostinil Sodium (Remodulin). | 1722 | ||
Prostacyclin Dosing | 1723 | ||
Inhaled Iloprost (Ventavis) and Treprostinil (Tyvaso). | 1724 | ||
Endothelin Receptor Antagonists | 1725 | ||
Endothelin-1 Antagonist Therapy | 1725 | ||
Phosphodiesterase Type 5 Inhibitors | 1726 | ||
Tadalafil. | 1726 | ||
Phosphodiesterase-5 Inhibitor Therapy | 1726 | ||
Soluble Guanylyl Cyclase Activators | 1726 | ||
Combination Therapy | 1726 | ||
Lung Transplantation | 1727 | ||
Overall Therapeutic Strategy | 1727 | ||
Survival | 1727 | ||
Acknowledgments | 1728 | ||
eFigure Image Gallery | 1729 | ||
Key Readings | 1728 | ||
References | 1733 | ||
59 Pulmonary Hypertension Due to Lung Disease | 1737 | ||
Introduction | 1737 | ||
Epidemiology of Pulmonary Hypertension Due to Lung Disease | 1737 | ||
Prevalence | 1737 | ||
Chronic Obstructive Pulmonary Disease | 1738 | ||
Idiopathic Pulmonary Fibrosis | 1740 | ||
Other Lung Diseases | 1740 | ||
Pathologic Changes and Pathogenesis | 1740 | ||
Pulmonary Vascular Remodeling | 1741 | ||
Pathogenesis | 1742 | ||
Hypoxic Pulmonary Vasoconstriction | 1742 | ||
Neurohormones | 1742 | ||
Angiotensin. | 1743 | ||
Endothelin. | 1743 | ||
Inflammation | 1743 | ||
Right Ventricle | 1743 | ||
Left Ventricle | 1745 | ||
Lung Mechanics | 1745 | ||
Clinical Presentation | 1745 | ||
Symptoms and Signs | 1745 | ||
Electrocardiography | 1746 | ||
Chest Radiography | 1746 | ||
Echocardiography | 1747 | ||
Computed Tomography | 1748 | ||
Magnetic Resonance Imaging | 1748 | ||
Right Heart Catheterization | 1748 | ||
B-Type Natriuretic Peptides | 1749 | ||
Treatment | 1749 | ||
Treatments Useful in All Patients with PH-LD | 1749 | ||
Lifestyle Modifications | 1749 | ||
Oxygen | 1750 | ||
Diuretics | 1750 | ||
Treatment of Sleep-Disordered Breathing | 1750 | ||
Lung Transplantation | 1750 | ||
Treatments That May be Considered in Selected Patients | 1750 | ||
Anticoagulation | 1750 | ||
Digoxin | 1751 | ||
Lung Volume Reduction Surgery | 1751 | ||
Phlebotomy for Polycythemia | 1751 | ||
Iron Supplementation | 1751 | ||
PAH-Specific Therapies | 1751 | ||
Calcium Channel Blockers. | 1752 | ||
Endothelin Receptor Antagonists. | 1752 | ||
Prostacyclin and Its Analogues. | 1752 | ||
Nitric Oxide. | 1753 | ||
Phosphodiesterase Type 5 Antagonists. | 1753 | ||
Riociguat. | 1753 | ||
Treatment of Decompensated RV Failure | 1751 | ||
Key Readings | 1754 | ||
References | 1755 | ||
60 Pulmonary Vasculitis | 1760 | ||
Introduction and Definitions | 1760 | ||
Classification | 1760 | ||
Epidemiology | 1761 | ||
Normal Vascular Anatomy and Histology | 1761 | ||
Histopathology of Vasculitis | 1762 | ||
Pathogenesis and Etiology | 1762 | ||
Initial Diagnosis | 1763 | ||
Clinical Scenarios Suggestive of Vasculitis | 1763 | ||
Destructive Upper Airway Lesions | 1763 | ||
Chest Imaging Findings of Cavitary or Nodular Disease | 1763 | ||
Diffuse Alveolar Hemorrhage (see Chapter 67) | 1763 | ||
Acute Glomerulonephritis | 1764 | ||
Pulmonary-Renal Syndrome | 1764 | ||
Palpable Purpura | 1764 | ||
Mononeuritis Multiplex | 1764 | ||
Multisystem Disease | 1765 | ||
Specific Testing | 1765 | ||
Antineutrophil Cytoplasmic Antibodies | 1765 | ||
Other Laboratory Studies | 1765 | ||
Chest Imaging | 1766 | ||
Other Imaging Studies | 1766 | ||
Bronchoscopy | 1766 | ||
Diagnostic Biopsy | 1766 | ||
Specific Clinical Disorders | 1766 | ||
Granulomatosis with Polyangiitis | 1766 | ||
Eosinophilic Granulomatosis with Polyangiitis (see Chapter 68) | 1768 | ||
Microscopic Polyangiitis | 1769 | ||
Treatment | 1769 | ||
General Principles | 1769 | ||
Remission-Induction | 1771 | ||
Limited Disease | 1771 | ||
Early Generalized Disease | 1771 | ||
Active Generalized Disease | 1771 | ||
Severe Disease | 1772 | ||
Refractory Disease | 1772 | ||
Maintenance | 1772 | ||
Monitoring for Complications | 1773 | ||
eFigure Image Gallery | 1775 | ||
References | 1780 | ||
Key Readings | 1774 | ||
61 Pulmonary Vascular Abnormalities | 1784 | ||
Introduction | 1784 | ||
Pulmonary Arteriovenous Malformations | 1784 | ||
Etiology | 1784 | ||
Hereditary Hemorrhagic Telangiectasia | 1784 | ||
Clinical Features and Diagnosis. | 1785 | ||
Genetics and Pathogenesis. | 1786 | ||
Phenotype-Genotype Correlations. | 1786 | ||
Cellular Basis. | 1786 | ||
Cavopulmonary Shunts. | 1786 | ||
Pathophysiology | 1786 | ||
Anatomic Basis | 1786 | ||
Physiologic Attributes at Rest | 1787 | ||
Right-to-Left Shunt. | 1787 | ||
Pulmonary Hemodynamics. | 1787 | ||
Pulmonary Function. | 1787 | ||
Physiologic Consequences of Posture and Exercise | 1787 | ||
Posture. | 1787 | ||
Exercise. | 1787 | ||
Pulmonary Hypertension | 1788 | ||
Clinical Features | 1788 | ||
Respiratory Symptoms | 1788 | ||
Dyspnea. | 1788 | ||
Hemoptysis. | 1788 | ||
Chest Pain. | 1788 | ||
Strokes and Cerebral Abscess | 1788 | ||
Other Neurologic Events | 1789 | ||
Pregnancy | 1789 | ||
Diagnosis | 1789 | ||
Imaging | 1789 | ||
Measurement of Right-to-Left Shunt | 1790 | ||
Contrast Echocardiography. | 1790 | ||
Screening Patients | 1791 | ||
Management | 1791 | ||
Embolization | 1791 | ||
Long-Term Outcome. | 1791 | ||
Physiologic Outcome. | 1791 | ||
Clinical Outcome. | 1793 | ||
Risks of Embolization | 1793 | ||
Radiation Exposure. | 1793 | ||
Development of Systemic Arterial Supply. | 1793 | ||
Development of Pulmonary Hypertension. | 1793 | ||
Nonembolization Options | 1793 | ||
Surgery. | 1793 | ||
Medical Management | 1794 | ||
Dental Issues. | 1794 | ||
Pregnancy. | 1794 | ||
Thromboembolic Risks. | 1794 | ||
Systemic-to-Pulmonary Vascular Communications | 1794 | ||
Anatomy | 1794 | ||
Adult (Acquired) Systemic-to-Pulmonary Communications | 1795 | ||
Fetal and Perinatal Systemic-to-Pulmonary Artery Collaterals | 1795 | ||
Aberrant Congenital Systemic-to-Pulmonary Communications | 1795 | ||
Cavopulmonary Anastomoses | 1795 | ||
Treatment | 1795 | ||
Managements of Hemoptysis | 1795 | ||
Pulmonary Sequestration | 1796 | ||
Classification | 1796 | ||
Pathogenesis | 1796 | ||
Clinical Features | 1796 | ||
Treatment | 1797 | ||
Pulmonary Varices | 1797 | ||
Diagnosis and Treatment | 1797 | ||
Pulmonary Artery Aneurysms | 1797 | ||
Etiology and Pathogenesis | 1797 | ||
Infection | 1797 | ||
Vascular Trauma | 1798 | ||
Weakness of the Arterial Wall | 1798 | ||
Diagnosis and Treatment | 1798 | ||
eFigure Image Gallery | 1800 | ||
Key Readings | 1799 | ||
References | 1809 | ||
62 Pulmonary Edema | 1814 | ||
Introduction | 1814 | ||
Pathophysiology of Pulmonary Edema | 1814 | ||
Increased Pressure Edema | 1815 | ||
Pathophysiology | 1816 | ||
Mechanisms | 1817 | ||
Increased Microvascular Hydrostatic Pressure. | 1817 | ||
Decreased Perimicrovascular Hydrostatic Pressure. | 1818 | ||
Decreased Transmural Protein Osmotic Pressure Difference. | 1819 | ||
Alveolar Barrier Function. | 1819 | ||
Increased Permeability Edema | 1819 | ||
Pathophysiology | 1820 | ||
Mechanisms | 1821 | ||
Diagnosis | 1822 | ||
Clinical Assessment | 1822 | ||
Definitions | 1822 | ||
Symptoms and Signs | 1823 | ||
Diagnostic Studies | 1824 | ||
Chest Radiographs | 1824 | ||
Arterial Blood Studies | 1825 | ||
Measurement of Pulmonary Edema Fluid Protein Concentration | 1825 | ||
Measurement of Lung Water and Barrier Function | 1825 | ||
Barrier Function | 1826 | ||
Biologic Markers of Lung Injury | 1826 | ||
Treatment | 1826 | ||
Emergency Therapy | 1826 | ||
Increased Pressure Edema | 1826 | ||
General Principles | 1827 | ||
Morphine Sulfate | 1827 | ||
Decreasing Venous Return | 1827 | ||
Ventilatory Strategies | 1828 | ||
Right Heart Catheterization | 1828 | ||
Specific Pharmacologic Therapy | 1828 | ||
Vasodilators. | 1828 | ||
Diuretics. | 1829 | ||
Inotropic Agents. | 1829 | ||
Increased Permeability Edema | 1829 | ||
General Principles | 1829 | ||
Lung-Protective Ventilator Strategies | 1830 | ||
Specific Pharmacologic Therapies | 1831 | ||
Corticosteroids. | 1831 | ||
Neuromuscular Blocking Agents. | 1831 | ||
Outcome | 1832 | ||
Resolution of Pulmonary Edema | 1832 | ||
Increased Pressure Edema | 1833 | ||
Increased Permeability Edema | 1833 | ||
Overview | 1834 | ||
Key Readings | 1835 | ||
References | 1836 | ||
L Infi ltrative and Interstitial Lung Diseases | 1841 | ||
63 Idiopathic Interstitial Pneumonias | 1841 | ||
Introduction | 1841 | ||
Clinical and Histologic Classification of Interstitial Lung Disease | 1842 | ||
Clinical, Radiologic, and Physiologic Features of Interstitial Lung Diseases | 1843 | ||
Past History | 1843 | ||
Occupational and Environmental History | 1843 | ||
Drug History | 1844 | ||
Smoking History | 1844 | ||
Family History | 1844 | ||
Gender | 1844 | ||
Symptoms | 1844 | ||
Physical Findings | 1844 | ||
Radiologic Features | 1844 | ||
Chest Radiography | 1844 | ||
Alveolar Filling Pattern. | 1845 | ||
Interstitial Pattern. | 1845 | ||
Nodular Pattern. | 1846 | ||
Linear or Reticular. | 1846 | ||
Other Radiographic Features. | 1847 | ||
Computed Tomography | 1847 | ||
Functional Assessment | 1849 | ||
Ventilatory Function | 1849 | ||
Elastic Resistance | 1850 | ||
Flow Resistance | 1850 | ||
Gas Exchange | 1851 | ||
Exercise | 1851 | ||
Laboratory Findings | 1852 | ||
Bronchoalveolar Lavage | 1852 | ||
Histologic Diagnosis | 1853 | ||
Transbronchial Biopsy | 1853 | ||
Surgical Lung Biopsy | 1853 | ||
Diagnosis and Management of Idiopathic Interstitial Pneumonias | 1854 | ||
Idiopathic Pulmonary Fibrosis | 1854 | ||
Clinical Features | 1855 | ||
Blood and Serologic Studies | 1855 | ||
Chest Imaging Studies | 1855 | ||
Chest Radiography. | 1855 | ||
Computed Tomography Scan. | 1855 | ||
HRCT Correlation with Physiologic Data in ILD. | 1855 | ||
Ability of HRCT Scanning to Diagnose ILD. | 1855 | ||
Other Imaging Techniques | 1856 | ||
Gallium Scanning. | 1856 | ||
Ventilation-Perfusion Lung Scanning. | 1857 | ||
Magnetic Resonance Imaging for Differentiating Inflammation and Fibrosis. | 1857 | ||
Imaging of Fibrogenesis by Positron Emission Tomography. | 1857 | ||
Pulmonary Function Tests | 1857 | ||
Pulmonary Hemodynamics | 1858 | ||
Abnormalities during Sleep | 1858 | ||
Histopathology | 1859 | ||
Etiologic Factors and Pathogenic Mechanisms | 1860 | ||
Diagnosis | 1861 | ||
Staging of Disease Activity and Predicting Outcome | 1862 | ||
Clinical Features. | 1862 | ||
Serum Studies. | 1862 | ||
Chest Radiography and High-Resolution Computed Tomography Scanning. | 1862 | ||
Pulmonary Physiologic Tests. | 1862 | ||
Bronchoalveolar Lavage. | 1863 | ||
Histopathology. | 1863 | ||
Prediction Models. | 1863 | ||
Outcome and Causes of Death. | 1863 | ||
Therapeutic Approach | 1863 | ||
Corticosteroids. | 1864 | ||
Immunomodulatory and Antifibrotic Agents. | 1864 | ||
Azathioprine. | 1864 | ||
N-Acetylcysteine. | 1864 | ||
Cyclophosphamide. | 1864 | ||
Other Agents. | 1864 | ||
New Therapeutic Approaches. | 1864 | ||
Pirfenidone. | 1864 | ||
Nintedanib. | 1865 | ||
Interferon-γ. | 1865 | ||
Endothelin-Receptor Antagonists. | 1865 | ||
Etanercept. | 1865 | ||
Imatinib. | 1865 | ||
Anticoagulants. | 1865 | ||
Treatment of Gastroesophageal Reflux. | 1865 | ||
Lung Transplantation (see Chapter 106) | 1866 | ||
Rehabilitation | 1866 | ||
Acute Exacerbations of Idiopathic Pulmonary Fibrosis | 1866 | ||
Familial Pulmonary Fibrosis | 1867 | ||
Nonspecific Interstitial Pneumonia | 1867 | ||
Clinical Features | 1867 | ||
Chest Imaging Studies | 1867 | ||
Chest Radiography. | 1867 | ||
Computed Tomography Scan. | 1867 | ||
Pulmonary Function Tests | 1868 | ||
Pathologic Features | 1868 | ||
Differential Diagnosis | 1868 | ||
Clinical Course and Outcome | 1869 | ||
Respiratory Bronchiolitis–Associated Interstitial Lung Disease/Desquamative Interstitial Pneumonia | 1869 | ||
Clinical Features | 1869 | ||
Chest Imaging Studies | 1869 | ||
Chest Radiography. | 1869 | ||
Computed Tomography Scan. | 1869 | ||
Bronchoalveolar Lavage | 1869 | ||
Pulmonary Function Tests | 1869 | ||
Pathologic Features | 1870 | ||
Clinical Course and Outcome | 1870 | ||
Acute Interstitial Pneumonia | 1871 | ||
Clinical Features | 1871 | ||
Chest Imaging Studies | 1871 | ||
Chest Radiography. | 1871 | ||
Computed Tomography Scan. | 1871 | ||
Pulmonary Function Tests | 1871 | ||
Pathologic Features | 1871 | ||
Clinical Course and Outcome | 1871 | ||
Cryptogenic Organizing Pneumonia | 1871 | ||
Clinical Features | 1872 | ||
Chest Imaging Studies | 1872 | ||
Chest Radiography. | 1872 | ||
Computed Tomography Scan. | 1872 | ||
Pulmonary Function Tests | 1872 | ||
Bronchoalveolar Lavage | 1872 | ||
Pathologic Features | 1872 | ||
Clinical Course and Outcome | 1873 | ||
Idiopathic Lymphocytic Interstitial Pneumonia | 1873 | ||
Clinical Features | 1873 | ||
Chest Imaging Studies | 1874 | ||
Chest Radiography. | 1874 | ||
Computed Tomography Scan. | 1874 | ||
Pulmonary Function Tests | 1874 | ||
Bronchoalveolar Lavage | 1874 | ||
Pathologic Features | 1874 | ||
Clinical Course and Outcome | 1874 | ||
Idiopathic Pleuroparenchymal Fibroelastosis | 1874 | ||
eFigure Image Gallery | 1876 | ||
Key Readings | 1875 | ||
References | 1888 | ||
64 Hypersensitivity Pneumonitis | 1895 | ||
Introduction | 1895 | ||
Etiology | 1895 | ||
Microbial Agents | 1895 | ||
Animal Proteins | 1896 | ||
Chemical Sensitizers | 1896 | ||
Exposure Settings and Risk Factors | 1896 | ||
Epidemiology | 1897 | ||
Clinical Presentation | 1897 | ||
Immunopathogenesis | 1897 | ||
Host Factors | 1898 | ||
Histopathology | 1898 | ||
Clinical Features | 1899 | ||
Signs and Symptoms | 1899 | ||
Lung Function | 1900 | ||
Imaging | 1900 | ||
Bronchoalveolar Lavage and Other Laboratory Testing | 1901 | ||
Diagnosing Hypersensitivity Pneumonitis | 1901 | ||
Exposure History | 1902 | ||
Antibody Testing | 1903 | ||
Bronchoalveolar Lavage | 1903 | ||
Lung Biopsy | 1903 | ||
Inhalation Challenge | 1904 | ||
Natural History and Prognosis | 1904 | ||
Treatment | 1904 | ||
Antigen Avoidance | 1905 | ||
Pharmacologic Therapy | 1905 | ||
Prevention | 1905 | ||
eFigure Image Galley | 1907 | ||
Key Readings | 1906 | ||
References | 1910 | ||
65 Connective Tissue Diseases | 1913 | ||
Introduction | 1913 | ||
Systemic Sclerosis (Scleroderma) | 1913 | ||
Epidemiology and Risk Factors | 1913 | ||
Chemically Induced Scleroderma- Like Disorders | 1914 | ||
Pulmonary Manifestations | 1915 | ||
Interstitial Pulmonary Fibrosis | 1915 | ||
Pathogenesis. | 1915 | ||
predisposition. | 1915 | ||
inflammation amplification. | 1915 | ||
fibrogenetic factors. | 1915 | ||
epithelial damage. | 1915 | ||
Clinical Features | 1916 | ||
Imaging | 1916 | ||
Lung Function Tests | 1917 | ||
Bronchoalveolar Lavage | 1917 | ||
Biopsy | 1918 | ||
Serologic Investigations | 1918 | ||
Prognosis | 1918 | ||
Treatment | 1919 | ||
Pulmonary Vascular Disease in Systemic Sclerosis | 1920 | ||
Other Pulmonary Complications | 1921 | ||
Rheumatoid Arthritis | 1921 | ||
Epidemiology and Risk Factors | 1921 | ||
Pulmonary Manifestations | 1921 | ||
Interstitial Pulmonary Fibrosis | 1921 | ||
Organizing Pneumonia | 1923 | ||
Bronchiolitis Obliterans | 1923 | ||
Follicular Bronchiolitis | 1923 | ||
Bronchiectasis | 1924 | ||
Pulmonary Vasculitis | 1924 | ||
Pulmonary Rheumatoid Nodules | 1924 | ||
Pleural Disease | 1924 | ||
Other Pulmonary Complications | 1924 | ||
Drug-Induced Pulmonary Disease | 1924 | ||
Acute Exacerbations of Interstitial Lung Disease | 1925 | ||
Pulmonary Function Tests | 1925 | ||
Radiologic Features | 1925 | ||
Treatment of Pulmonary Complications | 1925 | ||
Systemic Lupus Erythematosus | 1926 | ||
Epidemiology and Risk Factors | 1926 | ||
Pulmonary Manifestations | 1926 | ||
Diffuse Lung Disease | 1926 | ||
Interstitial Pneumonia. | 1926 | ||
Acute Lupus Pneumonitis. | 1926 | ||
Extrapulmonary Restriction | 1926 | ||
Diffuse Alveolar Hemorrhage (see Chapter 67) | 1927 | ||
Pulmonary Hypertension | 1927 | ||
Pleural Disease | 1927 | ||
Sjögren Syndrome | 1928 | ||
Epidemiology and Risk Factors | 1928 | ||
Pulmonary Manifestations | 1928 | ||
Diffuse Lung Disease | 1928 | ||
Tracheobronchial Disease | 1928 | ||
Polymyositis and Dermatomyositis | 1929 | ||
Epidemiology and Risk Factors | 1929 | ||
Pulmonary Manifestations | 1930 | ||
Diffuse Lung Disease | 1930 | ||
Clinical Features. | 1930 | ||
Imaging. | 1930 | ||
Pulmonary Function. | 1930 | ||
Bronchoalveolar Lavage. | 1930 | ||
Laboratory Tests. | 1930 | ||
Other Pulmonary Manifestations | 1931 | ||
Treatment of Pulmonary Complications | 1931 | ||
Mixed Connective Tissue Disease | 1931 | ||
Undifferentiated Connective Tissue Disease | 1931 | ||
Relapsing Polychondritis | 1932 | ||
Behçet Syndrome | 1933 | ||
Ankylosing Spondylitis | 1934 | ||
Marfan Syndrome | 1934 | ||
eFigure Image Galley | 1936 | ||
Key Readings | 1935 | ||
References | 1943 | ||
66 Sarcoidosis | 1950 | ||
Introduction | 1950 | ||
Epidemiology | 1950 | ||
Etiology | 1950 | ||
Potential Antigens | 1950 | ||
Genetic Aspects | 1951 | ||
Sarcoidosis as the Result of Immune System Exhaustion | 1952 | ||
Diagnostic Approach | 1952 | ||
Clinical Data Collection | 1952 | ||
Evidence of Extrapulmonary Involvement | 1952 | ||
Radiographic Findings | 1952 | ||
Serum Markers for Disease | 1953 | ||
Tissue Examination | 1955 | ||
Bronchoscopy | 1955 | ||
Bronchoalveolar Lavage | 1955 | ||
Extrapulmonary Tissue Biopsy | 1956 | ||
Clinical Phenotypes Suggestive of Sarcoidosis | 1957 | ||
Other Diagnostic Approaches | 1958 | ||
Pathologic Findings | 1958 | ||
Other Idiopathic Multiorgan Granulomatous Diseases | 1960 | ||
Evaluation of Pulmonary Disease | 1960 | ||
Pulmonary Function | 1960 | ||
Lung Imaging | 1960 | ||
Chest Radiograph | 1960 | ||
Chest Computed Tomography Scanning | 1960 | ||
Radionuclide Scanning | 1961 | ||
Health-Related Quality of Life | 1961 | ||
Extrapulmonary Sarcoidosis | 1962 | ||
Eyes | 1962 | ||
Skin | 1962 | ||
Nervous System | 1962 | ||
Heart | 1963 | ||
Ears, Nose, and Throat | 1963 | ||
Parotid Gland | 1964 | ||
Liver and Spleen | 1964 | ||
Joints | 1964 | ||
Peripheral Lymph Nodes | 1964 | ||
Endocrine Glands | 1964 | ||
Blood | 1964 | ||
Treatment of Sarcoidosis | 1964 | ||
Anti-Inflammatory Medications | 1964 | ||
Management of Extrapulmonary Disease | 1966 | ||
Antifibrotic Therapy | 1966 | ||
Supportive Care | 1966 | ||
Management of Complications of Sarcoidosis | 1966 | ||
Pulmonary Hypertension | 1966 | ||
Fatigue | 1967 | ||
Aspergilloma | 1967 | ||
Bone Health | 1967 | ||
Lung Transplantation | 1967 | ||
Key Readings | 1968 | ||
References | 1969 | ||
67 Alveolar Hemorrhage and Rare Infiltrative Diseases | 1976 | ||
Introduction | 1976 | ||
Diffuse Alveolar Hemorrhage | 1976 | ||
Definition | 1976 | ||
Clinical Presentation | 1976 | ||
History and Physical Examination | 1976 | ||
Radiology and Laboratory Evaluation | 1976 | ||
Physiology | 1977 | ||
Classification Schema | 1977 | ||
Diagnostic Approach | 1978 | ||
Diagnosis of Diffuse Alveolar Hemorrhage | 1978 | ||
Diagnosis of Underlying Cause | 1978 | ||
Role of Surgical Lung Biopsy and Renal Biopsy | 1978 | ||
Therapeutic Approach | 1979 | ||
Specific Causes | 1980 | ||
Vasculitis (see Chapter 60) | 1980 | ||
Granulomatosis with Polyangiitis (Wegener Granulomatosis). | 1980 | ||
Microscopic Polyangiitis. | 1981 | ||
Isolated Pulmonary Capillaritis. | 1982 | ||
Mixed Cryoglobulinemia. | 1982 | ||
Behçet Syndrome. | 1982 | ||
Henoch-Schönlein Purpura. | 1982 | ||
Pauci-immune Glomerulonephritis. | 1982 | ||
Immunologic | 1982 | ||
Goodpasture Syndrome. | 1982 | ||
Connective Tissue Disease. | 1984 | ||
Immune Complex–Related Crescentic Glomerulonephritis. | 1984 | ||
Acute Pulmonary Allograft Rejection. | 1984 | ||
Coagulation Disorders | 1984 | ||
Idiopathic Pulmonary Hemosiderosis | 1985 | ||
Other Causes of Diffuse Alveolar Hemorrhage | 1985 | ||
Drugs/Toxins. | 1985 | ||
Diffuse Alveolar Damage. | 1985 | ||
Mitral Stenosis. | 1985 | ||
Pulmonary Veno-occlusive Disease. | 1986 | ||
Pulmonary Capillary Hemangiomatosis. | 1986 | ||
Lymphangioleiomyomatosis and Tuberous Sclerosis. | 1987 | ||
Rare Infiltrative Disorders of the Lung | 1987 | ||
Neurofibromatosis | 1987 | ||
Hermansky-Pudlak Syndrome | 1987 | ||
Dyskeratosis Congenita | 1987 | ||
Gaucher Disease | 1988 | ||
Niemann-Pick Disease | 1988 | ||
Pulmonary Alveolar Microlithiasis | 1988 | ||
eFigure Image Gallery | 1990 | ||
Key Readings | 1989 | ||
References | 1996 | ||
68 Eosinophilic Lung Diseases | 2001 | ||
Introduction | 2001 | ||
Eosinophil Biology | 2001 | ||
General Features of Eosinophilic Pneumonias | 2002 | ||
Historical Perspective | 2002 | ||
Clinical Presentation | 2002 | ||
Pathology | 2003 | ||
Diagnosis | 2003 | ||
Eosinophilic Lung Disease of Undetermined Cause | 2005 | ||
Idiopathic Chronic Eosinophilic Pneumonia | 2005 | ||
Clinical Features | 2005 | ||
Imaging | 2005 | ||
Laboratory Studies | 2006 | ||
Bronchoalveolar Lavage | 2006 | ||
Differential Diagnosis | 2006 | ||
Lung Function Tests | 2006 | ||
Treatment and Prognosis | 2006 | ||
Idiopathic Acute Eosinophilic Pneumonia | 2007 | ||
Clinical Features | 2007 | ||
Imaging | 2007 | ||
Laboratory Studies | 2008 | ||
Bronchoalveolar Lavage | 2008 | ||
Lung Function Tests | 2008 | ||
Lung Biopsy | 2008 | ||
Treatment and Prognosis | 2008 | ||
Eosinophilic Granulomatosis with Polyangiitis | 2008 | ||
History and Nomenclature | 2008 | ||
Pathology | 2009 | ||
Clinical Features | 2009 | ||
Imaging | 2010 | ||
Laboratory Studies | 2011 | ||
Pathogenesis | 2011 | ||
Diagnosis | 2011 | ||
Differential Diagnosis | 2012 | ||
Treatment and Prognosis | 2012 | ||
Hypereosinophilic Syndrome | 2013 | ||
Pathogenenesis | 2013 | ||
Clinical Features | 2014 | ||
Imaging | 2014 | ||
Laboratory Studies | 2014 | ||
Treatment and Prognosis | 2014 | ||
Idiopathic Hypereosinophilic Obliterative Bronchiolitis | 2015 | ||
Eosinophilic Lung Disease of Determined Cause | 2015 | ||
Eosinophilic Pneumonias of Parasitic Origin | 2015 | ||
Tropical Eosinophilia | 2015 | ||
Clinical Features. | 2015 | ||
Pathogenesis. | 2015 | ||
Laboratory Studies. | 2016 | ||
Treatment. | 2016 | ||
Ascaris Pneumonia | 2016 | ||
Eosinophilic Pneumonia in Larva Migrans Syndrome | 2016 | ||
Strongyloides stercoralis Infection | 2017 | ||
Eosinophilic Pneumonias in Other Parasitic Infections | 2017 | ||
Eosinophilic Pneumonias of other Infectious Causes | 2017 | ||
Allergic Bronchopulmonary Aspergillosis and Related Syndromes | 2017 | ||
Allergic Bronchopulmonary Aspergillosis | 2017 | ||
Pathogenesis. | 2017 | ||
Diagnostic Criteria. | 2018 | ||
Imaging. | 2018 | ||
Treatment. | 2019 | ||
Other Allergic Bronchopulmonary Syndromes Associated with Fungi or Yeasts | 2019 | ||
Bronchocentric Granulomatosis | 2019 | ||
Drug-, Toxic Agent-, and Radiation-Induced Eosinophilic Pneumonias | 2019 | ||
Drugs | 2019 | ||
Radiation Therapy | 2020 | ||
Miscellaneous Lung Diseases with Associated Eosinophilia | 2020 | ||
Organizing Pneumonia | 2020 | ||
Asthma and Eosinophilic Bronchitis | 2021 | ||
Idiopathic Interstitial Pneumonias | 2021 | ||
Langerhans Cell Histiocytosis | 2021 | ||
Lung Transplantation | 2021 | ||
Other Lung Diseases with Occasional Eosinophilia | 2021 | ||
eFigure Image Gallery | 2023 | ||
Key Readings | 2022 | ||
References | 2028 | ||
69 Lymphangioleiomyomatosis | 2036 | ||
Introduction | 2036 | ||
Epidemiology | 2036 | ||
Genetic and Molecular Basis of Tuberous Sclerosis Complex and Lymphangioleiomyomatosis | 2037 | ||
Inheritance | 2037 | ||
Molecular Pathogenesis | 2037 | ||
Tumor Suppressor Proteins Control Cell Growth | 2037 | ||
The Tuberous Sclerosis Complex Proteins Regulate Signaling through the Akt Pathway | 2038 | ||
Tuberous Sclerosis Complex Mutations Are Found in the Lung and Kidney Lesions of Patients with Sporadic Lymphangioleiomyomatosis | 2038 | ||
Genetic Evidence Suggests That Lymphangioleiomyomatosis Is A Metastatic Neoplasm | 2040 | ||
Role of Lymphangiogenesis and Lymphatic Spread In Dissemination of Lymphangioleiomyomatosis | 2040 | ||
Role of Estrogen | 2040 | ||
Mechanisms of Matrix Remodeling in Lymphangioleiomyomatosis | 2040 | ||
Clinical Features | 2041 | ||
Physical Examination | 2041 | ||
Imaging Studies | 2041 | ||
Pulmonary Function Testing | 2044 | ||
Pathology | 2046 | ||
Diagnosis | 2047 | ||
Clinical Diagnosis | 2047 | ||
Differential Diagnosis | 2047 | ||
Lung Biopsy | 2048 | ||
Trials and Treatment | 2048 | ||
Sirolimus | 2049 | ||
General Recommendations—Treatment and Management | 2050 | ||
Other Therapeutic Considerations | 2050 | ||
General Recommendations—Screening | 2050 | ||
Sporadic LAM | 2050 | ||
Tuberous Sclerosis–Associated LAM | 2050 | ||
Pregnancy | 2050 | ||
Air Travel | 2051 | ||
Pleural Disease | 2051 | ||
Pulmonary Transplantation | 2052 | ||
Angiomyolipomas | 2052 | ||
Progression and Prognosis | 2052 | ||
Future Clinical Trials in Lymphangioleiomyomatosis | 2053 | ||
eFigure Image Gallery | 2055 | ||
Key Readings | 2054 | ||
References | 2062 | ||
70 Pulmonary Alveolar Proteinosis Syndrome | 2067 | ||
Introduction | 2067 | ||
Pathogenesis | 2067 | ||
Surfactant Composition and Homeostasis | 2067 | ||
Granulocyte/Macrophage Colony-Stimulating Factor | 2067 | ||
Murine Models of Disordered Surfactant Homeostasis | 2068 | ||
Role of GM-CSF Autoantibodies in Primary PAP | 2068 | ||
Genetic Factors | 2069 | ||
Disease Associations | 2071 | ||
Environmental Factors | 2071 | ||
Mechanisms of Disruption of Surfactant Homeostasis | 2072 | ||
Primary PAP (Autoimmune and Hereditary) | 2072 | ||
Secondary PAP | 2072 | ||
Pulmonary Surfactant Metabolic Dysfunction | 2072 | ||
Nomenclature and Classification | 2073 | ||
Epidemiology | 2073 | ||
Clinical Presentation | 2074 | ||
Evaluation and Differential Diagnosis | 2075 | ||
Radiographic Appearance | 2075 | ||
Pulmonary Function Testing | 2076 | ||
Bronchoscopy, Bronchoalveolar Lavage, and Transbronchial Biopsy | 2076 | ||
Surgical Lung Biopsy | 2076 | ||
Laboratory Studies | 2076 | ||
Approach to Diagnosis | 2077 | ||
Natural History | 2078 | ||
Secondary Infections | 2078 | ||
Pulmonary Fibrosis | 2079 | ||
Spontaneous Resolution | 2079 | ||
Treatment | 2079 | ||
Autoimmune PAP | 2079 | ||
Whole-Lung Lavage | 2079 | ||
Granulocyte/Macrophage Colony-Stimulating Factor | 2080 | ||
Rituximab and Other Approaches | 2080 | ||
Hereditary PAP | 2080 | ||
Secondary PAP | 2080 | ||
Pulmonary Surfactant Metabolic Dysfunction Disorders | 2080 | ||
Gaps in Knowledge and Future Directions | 2080 | ||
Etiology | 2080 | ||
Clinical Practice Guidelines | 2080 | ||
Novel Therapeutic Approaches | 2081 | ||
eFigure Image Gallery | 2082 | ||
Key Readings | 2081 | ||
References | 2090 | ||
71 Drug-Induced Pulmonary Disease | 2094 | ||
Introduction | 2094 | ||
Chemotherapeutic Agents | 2094 | ||
Antibiotic-Derived Agents | 2096 | ||
Bleomycin | 2096 | ||
Mitomycin C | 2097 | ||
Other Antibiotic Chemotherapeutic Agents | 2097 | ||
Alkylating Agents | 2098 | ||
Busulfan | 2098 | ||
Cyclophosphamide | 2098 | ||
Chlorambucil | 2098 | ||
Melphalan | 2098 | ||
Ifosfamide | 2098 | ||
Other Alkylating Agents | 2099 | ||
Antimetabolites | 2099 | ||
Methotrexate | 2099 | ||
Azathioprine and 6-Mercaptopurine | 2099 | ||
Cytosine Arabinoside | 2099 | ||
Gemcitabine | 2100 | ||
Fludarabine | 2100 | ||
Piritrexim | 2100 | ||
Nitrosoureas | 2100 | ||
Podophyllotoxins | 2101 | ||
Etoposide and Teniposide | 2101 | ||
Paclitaxel | 2101 | ||
Docetaxel | 2101 | ||
Vinblastine | 2101 | ||
All-Trans Retinoic Acid | 2101 | ||
Irinotecan and Topotecan | 2102 | ||
Targeted Therapy | 2102 | ||
Monoclonal Antibodies | 2102 | ||
Bevacizumab. | 2102 | ||
Cetuximab and Panitumumab. | 2102 | ||
Trastuzumab and Ado-Trastuzumab Emtansine. | 2102 | ||
Rituximab. | 2102 | ||
Tyrosine Kinase Inhibitors | 2103 | ||
Gefitinib. | 2103 | ||
Erlotinib. | 2103 | ||
Imatinib. | 2103 | ||
Dasatinib. | 2103 | ||
Bosutinib. | 2103 | ||
Sunitinib and Sorafenib. | 2103 | ||
Immunomodulatory Agents | 2103 | ||
Interferons. | 2103 | ||
Rapamycin Analogs. | 2104 | ||
Antimicrobial Agents | 2104 | ||
Nitrofurantoin | 2104 | ||
Acute Reaction | 2104 | ||
Chronic Reaction | 2104 | ||
Sulfasalazine | 2105 | ||
Miscellaneous Antimicrobial Drugs | 2105 | ||
Illicit Drugs | 2105 | ||
Heroin | 2105 | ||
Methadone | 2106 | ||
Methylphenidate | 2106 | ||
Cocaine | 2106 | ||
Talc Granulomatosis | 2106 | ||
Cardiovascular Drugs | 2106 | ||
Angiotensin-Converting Enzyme Inhibitors | 2106 | ||
Amiodarone | 2107 | ||
Protamine | 2108 | ||
β-Adrenergic Antagonists | 2108 | ||
Tocainide and Flecainide | 2109 | ||
Anti-Inflammatory Agents | 2109 | ||
Aspirin | 2109 | ||
Other Nonsteroidal Anti-Inflammatory Drugs | 2109 | ||
Penicillamine | 2109 | ||
Leflunomide | 2109 | ||
Biologic Agents | 2109 | ||
Leukotriene Antagonists | 2110 | ||
Corticosteroids | 2110 | ||
Drug-Induced Systemic Lupus Erythematosus | 2110 | ||
Inhalants | 2111 | ||
Oil | 2111 | ||
Oxygen | 2111 | ||
Miscellaneous Drugs/Agents | 2111 | ||
Radiographic Contrast Medium–Induced Leukostasis | 2111 | ||
Tocolytic-Induced Pulmonary Edema | 2112 | ||
Hydrochlorothiazide | 2112 | ||
Methysergide, Bromocriptine, and Cabergoline | 2112 | ||
Dextran | 2112 | ||
Amphetaminergic Agents | 2112 | ||
Esophageal Variceal Sclerotherapy | 2112 | ||
Phenytoin | 2113 | ||
Dantrolene | 2113 | ||
eFigure Image Gallery | 2114 | ||
Key Readings | 2113 | ||
References | 2127 | ||
M Environmental and Occupational Hazards | 2131 | ||
72 Asthma in the Workplace | 2131 | ||
Introduction | 2131 | ||
Sensitizer-Induced Occupational Asthma | 2132 | ||
Epidemiologic Aspects | 2132 | ||
Causal Agents | 2132 | ||
Pathophysiology | 2133 | ||
Immunologic, IgE-mediated | 2133 | ||
Immunologic, non–IgE mediated | 2133 | ||
Risk Factors | 2133 | ||
Environmental Factors | 2133 | ||
Host-Related Factors | 2133 | ||
Diagnosis | 2134 | ||
Outcome and Management of Sensitizer-Induced Asthma | 2136 | ||
Socioeconomic Impact | 2137 | ||
Irritant-Induced Asthma | 2137 | ||
Epidemiologic Aspects | 2137 | ||
Pathophysiology | 2138 | ||
Risk Factors | 2138 | ||
Diagnosis | 2139 | ||
Outcome and Treatment | 2139 | ||
Prevention | 2139 | ||
Work-Exacerbated Asthma | 2140 | ||
Epidemiologic Aspects | 2140 | ||
Pathophysiology | 2140 | ||
Work Exposures Associated with Work-Exacerbated Asthma | 2140 | ||
Diagnosis of Work-Exacerbated Asthma | 2140 | ||
Differentiating Work-Exacerbated Asthma From Non–Work-Related Asthma or Occupational Asthma | 2141 | ||
Socioeconomic Impact of Work-Exacerbated Asthma | 2141 | ||
Key Readings | 2142 | ||
References | 2143 | ||
73 Pneumoconioses | 2146 | ||
Introduction | 2146 | ||
Definitions | 2146 | ||
Accumulation of Dust in the Lung and Tissue Reactions | 2146 | ||
Exposure-Response Relationships | 2146 | ||
Chest Imaging | 2147 | ||
Clinical Issues, Lung Function, and Principles of Management | 2147 | ||
Epidemiology and Implications for Clinical Practice | 2148 | ||
Silicosis | 2149 | ||
Definition | 2149 | ||
Industries and Occupations Still at Risk | 2149 | ||
Pathology | 2149 | ||
Pathogenesis | 2150 | ||
Epidemiology: Secular Trends and Their Implications for the Clinician | 2151 | ||
Tuberculosis | 2151 | ||
Airflow Obstruction and Chronic Bronchitis | 2151 | ||
Connective Tissue Diseases, Renal Disease, and Cardiovascular Disease | 2151 | ||
Lung Cancer | 2151 | ||
Clinical Features | 2151 | ||
Radiographic Features | 2152 | ||
Lung Function | 2153 | ||
Diagnosis and Complications | 2153 | ||
Management and Control | 2153 | ||
Coal Workers’ Pneumoconiosis | 2153 | ||
Definition and Occupations at Risk | 2153 | ||
Pathology | 2154 | ||
Pathogenesis | 2155 | ||
Epidemiology and Natural History | 2156 | ||
Rheumatoid Pneumoconiosis | 2156 | ||
Role of Silica | 2156 | ||
Airflow Obstruction and Chronic Bronchitis | 2156 | ||
Tuberculosis and Cancer | 2156 | ||
Clinical Features | 2156 | ||
Chest Radiography | 2156 | ||
Lung Function | 2157 | ||
Diagnosis, Complications, and Management | 2157 | ||
ASBESTOS-Related Fibrosis of the Lungs (Asbestosis) and Pleura | 2157 | ||
Asbestos Minerals | 2157 | ||
History and Uses | 2157 | ||
Sources of Human Exposure | 2158 | ||
Fate of Inhaled Fibers | 2160 | ||
Exposure versus Dose-Response Relationships | 2160 | ||
Asbestosis (Pulmonary Parenchymal Fibrosis) | 2160 | ||
Pathology and Pathogenesis | 2160 | ||
Epidemiology and Natural History | 2161 | ||
Clinical Features | 2161 | ||
Radiographic Features | 2161 | ||
Lung Function | 2162 | ||
Diagnosis and Complications | 2162 | ||
Asbestosis, Asbestos Exposure, Lung Cancer, and Mesothelioma (see Chapters 52 and 82) | 2163 | ||
Pleural Plaques | 2163 | ||
Pathology and Pathogenesis | 2163 | ||
Clinical Features | 2163 |