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Book Details
Abstract
Designed with the practicing clinician in mind, Idiopathic Pulmonary Fibrosis provides a succinct, easy-to-digest overview of this challenging condition in which the cause of thickening lung tissue is unknown. This concise resource by Drs. Kevin K. Brown and Jeff Swigris provides essential information for the physician who sees pulmonary fibrosis patients, including epidemiology, genetics and biomarkers, pathology, diagnosis, disease monitoring, and therapeutics intended to improve the patient’s lifespan and quality of life.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Idiopathic Pulmonary Fibrosis | i | ||
| Idiopathic Pulmonary Fibrosis | iii | ||
| Copyright | iv | ||
| List of Contributors | v | ||
| Preface | vii | ||
| Contents | ix | ||
| 1 - Introduction | 1 | ||
| 2 - Idiopathic Pulmonary Fibrosis Epidemiology | 3 | ||
| THE EPIDEMIOLOGY OF IDIOPATHIC PULMONARY FIBROSIS | 3 | ||
| Background | 3 | ||
| Trends in Idiopathic Pulmonary Fibrosis Incidence and Prevalence | 3 | ||
| The United States | 3 | ||
| The United Kingdom | 4 | ||
| Canada | 5 | ||
| Other countries | 5 | ||
| Mortality Rates and Trends Over Time | 5 | ||
| The United States | 5 | ||
| Other countries | 6 | ||
| Risk Factors | 6 | ||
| Cigarette smoking | 6 | ||
| Occupational and environmental exposures | 6 | ||
| SUMMARY | 7 | ||
| REFERENCES | 7 | ||
| 3 - Mechanisms of Fibrosis | 9 | ||
| INTRODUCTION | 9 | ||
| ANIMAL MODELS OF PULMONARY FIBROSIS | 9 | ||
| Bleomycin Model | 11 | ||
| Repetitive Bleomycin Model | 12 | ||
| Acid Instillation | 12 | ||
| Transforming Growth Factor-β Overexpression | 12 | ||
| Silica | 13 | ||
| Asbestosis | 13 | ||
| Fluorescein Isothiocyanate | 13 | ||
| Radiation-Induced Fibrosis | 13 | ||
| Hermansky-Pudlak Models | 13 | ||
| EPITHELIAL CELLS IN FIBROSIS | 13 | ||
| Endoplasmic Reticulum Stress | 14 | ||
| Developmental Program Reactivation | 14 | ||
| Epithelial-Mesenchymal Transition | 14 | ||
| FIBROBLASTS AND COLLAGEN DEPOSITION | 14 | ||
| Fibroblasts | 14 | ||
| Myofibroblasts | 15 | ||
| Fibrocytes | 15 | ||
| Pericytes | 16 | ||
| Collagen and Extracellular Matrix | 16 | ||
| Matrix Metalloproteinases | 17 | ||
| GROWTH FACTORS, CYTOKINES, AND CHEMOKINES | 17 | ||
| Transforming Growth Factor-β | 17 | ||
| Connective Tissue Growth Factor | 18 | ||
| Platelet-Derived Growth Factor | 18 | ||
| Fibroblast Growth Factor | 18 | ||
| Vascular Endothelial Growth Factor | 18 | ||
| Interleukin 13 | 18 | ||
| Tumor Necrosis Factor-α | 19 | ||
| Interferon-γ | 19 | ||
| Monocyte Chemoattractant Protein 1 | 19 | ||
| Chemokine (C-C motif) Ligand 18 | 19 | ||
| Other Cytokines | 19 | ||
| INFLAMMATION AND IMMUNE CELLS | 19 | ||
| Macrophages | 19 | ||
| Lymphocytes | 20 | ||
| Neutrophils | 20 | ||
| Infections | 21 | ||
| COAGULATION | 21 | ||
| MUCOCILIARY DYSFUNCTION | 22 | ||
| AGING LUNG | 23 | ||
| THERAPEUTIC PERSPECTIVES | 23 | ||
| CONCLUSIONS | 23 | ||
| REFERENCES | 23 | ||
| 4 - Genetics of Pulmonary Fibrosis | 33 | ||
| INTRODUCTION | 33 | ||
| USING FAMILIAL PULMONARY FIBROSIS TO IDENTIFY RARE VARIANTS | 33 | ||
| SURFACTANT PROTEIN C MUTATIONS | 33 | ||
| SURFACTANT PROTEIN A MUTATIONS | 34 | ||
| TELOMERASE MUTATIONS | 34 | ||
| ELMOD2 | 34 | ||
| MUCIN 5B | 35 | ||
| GENOME-WIDE ASSOCIATION STUDIES IDENTIFY COMMON VARIANTS | 35 | ||
| TRANSCRIPTIONAL PROFILING | 36 | ||
| GENETIC SYNDROMES | 36 | ||
| MOVING FORWARD | 36 | ||
| REFERENCES | 36 | ||
| 5 - Imaging of Idiopathic Pulmonary Fibrosis | 39 | ||
| INTRODUCTION | 39 | ||
| COMPUTED TOMOGRAPHY FEATURES OF FIBROTIC LUNG DISEASE | 39 | ||
| Honeycombing | 39 | ||
| Reticulation | 39 | ||
| Traction Bronchiectasis | 39 | ||
| Ground Glass | 40 | ||
| CLASSIFICATION OF USUAL INTERSTITIAL PNEUMONIA | 40 | ||
| PROGNOSTIC VALUE OF COMPUTED TOMOGRAPHY IN USUAL INTERSTITIAL PNEUMONIA | 41 | ||
| CHALLENGES IN CLASSIFYING USUAL INTERSTITIAL PNEUMONIA | 41 | ||
| Diagnostic Dilemma of Honeycombing | 41 | ||
| Mosaic Pattern and Air Trapping | 43 | ||
| Asymmetric Fibrosis | 43 | ||
| Atypical Appearance of Idiopathic Pulmonary Fibrosis | 43 | ||
| Familial Pulmonary Fibrosis | 43 | ||
| MULTIDISCIPLINARY EVALUATION | 43 | ||
| COMPLICATIONS | 44 | ||
| Acute Exacerbation | 44 | ||
| Lung Cancer | 44 | ||
| Pulmonary Hypertension | 44 | ||
| Gastroesophageal Reflux | 45 | ||
| Combined Pulmonary Fibrosis and Emphysema | 45 | ||
| DIFFERENTIAL DIAGNOSIS | 46 | ||
| Nonspecific Interstitial Pneumonia | 46 | ||
| Chronic Hypersensitivity Pneumonitis | 46 | ||
| Connective Tissue Disease | 47 | ||
| Drug-Related Pulmonary Fibrosis | 48 | ||
| Asbestosis and Occupational Lung Disease | 48 | ||
| CONCLUSION | 49 | ||
| REFERENCES | 49 | ||
| 6 - The Pathology of Usual Interstitial Pneumonia | 55 | ||
| HISTOLOGIC FEATURES OF USUAL INTERSTITIAL PNEUMONIA | 55 | ||
| GEOGRAPHIC HETEROGENEITY AND FIBROBLASTIC ACTIVITY | 55 | ||
| HONEYCOMB CHANGE | 55 | ||
| VASCULAR CHANGES | 57 | ||
| UIP AND NSIP-LIKE AREAS | 57 | ||
| FEATURES SUGGESTING THE CAUSES OF USUAL INTERSTITIAL PNEUMONIA | 58 | ||
| DIFFERENTIATING USUAL INTERSTITIAL PNEUMONIA FROM OTHER FORMS OF PULMONARY FIBROSIS | 59 | ||
| SELECTED REFERENCES | 63 | ||
| 7 - Making the Diagnosis | 65 | ||
| INTRODUCTION | 65 | ||
| CATEGORIZATION OF ILD BY ETIOLOGY AND PATTERN | 65 | ||
| CLINICAL EVALUATION | 66 | ||
| History | 66 | ||
| Physical Examination | 66 | ||
| Laboratory Testing | 66 | ||
| RADIOGRAPHIC EVALUATION | 66 | ||
| PATHOLOGIC EVALUATION | 67 | ||
| DIAGNOSIS | 67 | ||
| SPECIAL CONSIDERATIONS | 68 | ||
| ADDITIONAL CHALLENGES | 68 | ||
| SUMMARY | 69 | ||
| REFERENCES | 69 | ||
| 8 - IPF Look-Alikes: Chronic Hypersensitivity Pneumonitis, Connective Tissue Disorder–Related Pulmonary Fibrosis, a ... | 73 | ||
| CHRONIC HYPERSENSITIVITY PNEUMONITIS | 73 | ||
| Epidemiology | 73 | ||
| Diagnosis | 73 | ||
| Identifying the Inciting Antigen | 73 | ||
| Imaging | 75 | ||
| Serologic Evaluation | 75 | ||
| Specific Inhalation Challenge | 75 | ||
| Bronchoalveolar Lavage and Histopathology | 75 | ||
| Putting It all Together | 76 | ||
| CONNECTIVE TISSUE DISORDER–ASSOCIATED INTERSTITIAL LUNG DISEASE | 77 | ||
| Background | 77 | ||
| Clinical Presentation | 77 | ||
| Role of Autoantibodies | 82 | ||
| Pulmonary Function Testing | 82 | ||
| Imaging Differences | 82 | ||
| Histopathologic Features | 82 | ||
| Treatment Differences | 82 | ||
| Survival Differences | 83 | ||
| Summary | 84 | ||
| OTHER CAUSES OF USUAL INTERSTITIAL PNEUMONIA-PATTERN LUNG INJURY | 84 | ||
| Asbestosis | 84 | ||
| Drug-Induced Lung Disease | 84 | ||
| Radiation-Induced Lung Disease | 84 | ||
| Hermansky-Pudlak Syndrome | 85 | ||
| SUMMARY | 85 | ||
| REFERENCES | 85 | ||
| 9 - Natural History of Idiopathic Pulmonary Fibrosis and Disease Monitoring | 89 | ||
| INTRODUCTION | 89 | ||
| THE NATURAL HISTORY OF UNTREATED IDIOPATHIC PULMONARY FIBROSIS | 89 | ||
| THE NATURAL HISTORY OF TREATED IDIOPATHIC PULMONARY FIBROSIS | 89 | ||
| PROGNOSTIC INDICATORS | 91 | ||
| RECOMMENDATIONS FOR DISEASE MONITORING | 93 | ||
| CONCLUSION | 94 | ||
| REFERENCES | 94 | ||
| 10 - Biomarkers in IPF | 99 | ||
| INTRODUCTION | 99 | ||
| IDIOPATHIC PULMONARY FIBROSIS: BIOMARKERS OF DISEASE SUSCEPTIBILITY | 99 | ||
| Genetic Risk in Familial Fibrosis | 100 | ||
| Telomerase abnormalities | 101 | ||
| Surfactant protein mutations | 101 | ||
| Genetic Risk in Sporadic Idiopathic Pulmonary Fibrosis | 101 | ||
| MUC5B promoter variant as a molecular biomarker | 101 | ||
| TOLLIP | 102 | ||
| IDIOPATHIC PULMONARY FIBROSIS: DIAGNOSTIC BIOMARKERS | 102 | ||
| KL-6 | 102 | ||
| Surfactant Proteins | 102 | ||
| Matrix Metalloproteinases | 103 | ||
| MMP1 | 103 | ||
| MMP7 | 103 | ||
| MMP3 | 103 | ||
| Osteopontin | 104 | ||
| IDIOPATHIC PULMONARY FIBROSIS: PROGNOSTIC BIOMARKERS | 104 | ||
| Matrix Metalloproteinase Degradation Products | 104 | ||
| Gene Profiling | 104 | ||
| Toll-Like Receptors | 105 | ||
| CCL18 | 105 | ||
| YKL40 | 105 | ||
| T Cells | 105 | ||
| CXCL13 | 105 | ||
| IDIOPATHIC PULMONARY FIBROSIS: BIOMARKERS OF DISEASE ACTIVITY AND ACUTE EXACERBATION | 106 | ||
| Defensins | 106 | ||
| Fibrocytes | 106 | ||
| Periostin | 106 | ||
| CONCLUSION | 107 | ||
| REFERENCES | 107 | ||
| FURTHER READING | 112 | ||
| 11 - Therapeutic Options for Patients With Idiopathic Pulmonary Fibrosis | 113 | ||
| INTRODUCTION | 113 | ||
| EXERCISE AND PULMONARY REHABILITATION | 113 | ||
| SUPPLEMENTAL OXYGEN | 115 | ||
| ANTIFIBROTIC THERAPIES | 116 | ||
| GASTROESOPHAGEAL REFLUX | 117 | ||
| PULMONARY HYPERTENSION | 118 | ||
| LUNG TRANSPLANTATION | 121 | ||
| REFERENCES | 123 | ||
| Index | 127 | ||
| A | 127 | ||
| B | 127 | ||
| C | 127 | ||
| D | 128 | ||
| E | 128 | ||
| F | 128 | ||
| G | 129 | ||
| H | 129 | ||
| I | 129 | ||
| K | 130 | ||
| L | 130 | ||
| M | 130 | ||
| N | 130 | ||
| O | 130 | ||
| P | 130 | ||
| R | 131 | ||
| S | 131 | ||
| T | 131 | ||
| U | 132 | ||
| V | 132 | ||
| W | 132 | ||
| Y | 132 |