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Book Details
Abstract
Clinically focused and designed to provide a to-the-point overview, Interstitial Lung Disease, by Drs. Talmadge King, Harold Collard, and Luca Richeldi, bring you up to date with increased understanding, new treatment protocols, and recent advances in the field. Written by contributing specialists who are global experts in their respective areas, this one-stop reference provides pulmonologists, intensivists, internal medicine physicians, and researchers with a dependable source of information on current treatment options and patient care.
• Evidence for current treatment options for interstitial pneumonia, idiopathic pulmonary fibrosis, and smoking-related interstitial lung diseases.
• Approach to diagnosis of interstitial lung diseases, such as sarcoidosis, hypersensitivity pneumonitis, and Churg-Strauss syndrome.
• Genetic markers for inherited interstitial lung diseases such as dyskeratosis congenita, tuberous sclerosis/LAM, and hyper-IgE syndrome.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Interstitial Lung Disease | i | ||
| Interstitial Lung Disease | iii | ||
| Copyright | iv | ||
| Contributors | v | ||
| Preface | ix | ||
| Contents | xi | ||
| 1 - Genetic Interstitial Lung Disease | 1 | ||
| GENETIC DISORDERS AFFECTING MULTIPLE ORGANS, INCLUDING THE LUNG | 2 | ||
| Lymphangioleiomyomatosis and Tuberous Sclerosis Complex | 2 | ||
| Birt-Hogg-Dubé Syndrome | 4 | ||
| Dyskeratosis Congenita | 5 | ||
| RIDDLE Syndrome | 6 | ||
| Hermansky-Pudlak Syndrome | 6 | ||
| NKX2-1–Related Disorders | 6 | ||
| Neurofibromatosis | 6 | ||
| Poikiloderma, With Tendon Contractures, Myopathy, and Pulmonary Fibrosis | 7 | ||
| Interstitial Lung Disease, Nephrotic Syndrome, and Epidermolysis Bullosa | 7 | ||
| Stimulator of Interferon Genes–Associated Vasculopathy, Infantile-Onset | 7 | ||
| Autoimmune Disease With Facial Dysmorphism | 7 | ||
| Autoimmune Interstitial Lung, Joint, and Kidney Disease | 7 | ||
| GATA2 Deficiency | 8 | ||
| Immunodeficiency Syndromes | 8 | ||
| Inborn Errors of Metabolism | 9 | ||
| GENETIC DISEASES IN WHICH INTERSTITIAL LUNG DISEASE IS THE DOMINANT PHENOTYPE | 9 | ||
| Surfactant Metabolism Dysfunction, Type 1: SFTPB Mutations | 10 | ||
| Surfactant Metabolism Dysfunction, Type 2: SFTPC Mutations | 11 | ||
| Surfactant Metabolism Dysfunction, Type 3: ABCA3 Mutations | 11 | ||
| Surfactant Metabolism Dysfunction, Types 4 and 5: CSF2RA and CSF3RB Mutations | 11 | ||
| Interstitial Lung and Liver Disease | 12 | ||
| Pulmonary Fibrosis, Telomere Related, Type 1 | 12 | ||
| Pulmonary Fibrosis, Telomere Related, Type 2 | 12 | ||
| Pulmonary Fibrosis, Telomere Related, Type 3 | 12 | ||
| Pulmonary Fibrosis, Telomere Related, Type 4 | 13 | ||
| Pulmonary Fibrosis, Other Telomere Related | 13 | ||
| Pulmonary Fibrosis and Adenocarcinoma, Related to Surfactant A Mutations | 13 | ||
| Susceptibility to Pulmonary Fibrosis, Common Variants | 14 | ||
| Pulmonary Alveolar Microlithiasis | 14 | ||
| CONCLUSION | 15 | ||
| REFERENCES | 15 | ||
| 2 - Pathobiology of Novel Approaches to Treatment | 25 | ||
| THE APPROACH TO TREATMENT IN THE OLD DAYS | 25 | ||
| THE ADVENT OF PIRFENIDONE | 26 | ||
| THE STORY OF NINTEDANIB | 28 | ||
| NAC (N-ACETYLCYSTEINE) OR NOT NAC, THAT IS THE QUESTION | 31 | ||
| ANTACID TREATMENT: MORE DOUBTS THAN CERTAINTIES | 32 | ||
| CURRENT CLINICAL TRIALS: THE FUTURE IS COMING | 32 | ||
| CONCLUSION: IS PATHOBIOLOGY IMPORTANT FOR NOVEL APPROACHES TO TREATMENT? | 34 | ||
| 3 - Smoking-Related Interstitial Lung Diseases | 39 | ||
| SMOKING AND INTERSTITIAL LUNG DISEASE | 39 | ||
| MECHANISMS BY WHICH TOBACCO SMOKE MAY PROMOTE INTERSTITIAL LUNG DISEASE | 41 | ||
| RESPIRATORY BRONCHIOLITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE | 42 | ||
| DESQUAMATIVE INTERSTITIAL PNEUMONIA | 43 | ||
| PULMONARY LANGERHANS CELL HISTIOCYTOSIS | 44 | ||
| ACUTE INTERSTITIAL LUNG DISEASES ASSOCIATED WITH SMOKING | 46 | ||
| SMOKING AND PULMONARY FIBROSIS | 47 | ||
| INTERSTITIAL LUNG DISEASES THAT ARE LESS COMMON IN SMOKERS | 48 | ||
| SUMMARY | 48 | ||
| REFERENCES | 48 | ||
| 4 - Management of Idiopathic Pulmonary Fibrosis | 55 | ||
| PHARMACOLOGIC TREATMENTS | 55 | ||
| Guidelines for Treatment of Idiopathic Pulmonary Fibrosis: The 2015 Update | 55 | ||
| Pirfenidone | 57 | ||
| Nintedanib | 58 | ||
| Limitations of Pharmacologic Treatment | 59 | ||
| MANAGEMENT OF ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS | 59 | ||
| NONPHARMACOLOGIC TREATMENTS | 60 | ||
| LUNG TRANSPLANTATION | 61 | ||
| CLOSING REMARKS | 61 | ||
| REFERENCES | 61 | ||
| 5 - Nonpharmacologic Therapy for Idiopathic Pulmonary Fibrosis | 65 | ||
| PALLIATIVE CARE | 66 | ||
| DYSPNEA | 66 | ||
| COUGH | 68 | ||
| GASTROESOPHAGEAL REFLUX DISEASE | 69 | ||
| TREATING COMORBIDITIES | 70 | ||
| LUNG TRANSPLANTATION | 71 | ||
| CONCLUSION | 72 | ||
| REFERENCES | 72 | ||
| 6 - Idiopathic Pulmonary Fibrosis: Diagnosis and Epidemiology | 75 | ||
| DIAGNOSIS OF IDIOPATHIC PULMONARY FIBROSIS | 75 | ||
| Challenges in Diagnosing Idiopathic Pulmonary Fibrosis | 76 | ||
| EPIDEMIOLOGY OF IDIOPATHIC PULMONARY FIBROSIS | 77 | ||
| Background | 77 | ||
| Mortality and Mortality Trends Over Time | 78 | ||
| Prevalence and Incidence, and Trends Over Time | 80 | ||
| Risk Factors | 81 | ||
| Cigarette smoking | 82 | ||
| Environmental and Occupational Exposures | 82 | ||
| Metal dust. In several case-control studies and a metaanalysis, investigators have examined the association between a variety of... | 82 | ||
| Wood dust. In the studies mentioned earlier and other case-controlled studies, researchers have examined the association between... | 83 | ||
| Sand, stone, and silica. Three of four case-control studies have found an increased risk of IPF in patients with exposures to sa... | 83 | ||
| Farming and livestock. Agriculture/farming-related and livestock-related exposures have been found to be associated with IPF. In... | 83 | ||
| SUMMARY | 83 | ||
| REFERENCES | 83 | ||
| 7 - Approach to the Diagnosis of Interstitial Lung Disease | 87 | ||
| CLINICAL EVALUATION | 87 | ||
| History Taking | 87 | ||
| ASSESSMENT OF SYMPTOMS | 89 | ||
| PHYSICAL EXAMINATION | 89 | ||
| LABORATORY TESTING | 90 | ||
| PULMONARY FUNCTION TESTING | 90 | ||
| RADIOLOGIC ASSESSMENT | 91 | ||
| BRONCHOSCOPY | 92 | ||
| SURGICAL LUNG BIOPSY | 93 | ||
| ASSESSMENT OF PULMONARY HYPERTENSION | 93 | ||
| DIAGNOSTIC ALGORITHM | 93 | ||
| CLASSIFICATION OF DISEASE BEHAVIOR | 95 | ||
| REFERENCES | 95 | ||
| 8 - Palliative and End-of-Life Care in Idiopathic Pulmonary Fibrosis | 97 | ||
| INTERSTITIAL LUNG DISEASE | 97 | ||
| Chronic Illness | 97 | ||
| Idiopathic Pulmonary Fibrosis | 97 | ||
| Palliative Care—Goals and Timing | 97 | ||
| Palliative Care Versus Hospice | 98 | ||
| Palliative Care in Patients With Malignant Disease | 99 | ||
| Palliative Care in Patients With Advanced Lung Disease | 99 | ||
| PALLIATIVE CARE IN IDIOPATHIC PULMONARY FIBROSIS | 99 | ||
| Supporting Research | 99 | ||
| Caregiver Perspective | 100 | ||
| Critical Care Perspective | 101 | ||
| Lung Transplant Perspective | 101 | ||
| OVERCOMING BARRIERS | 101 | ||
| Who Should Deliver Palliative Care? | 101 | ||
| How to Deliver Palliative Care | 102 | ||
| FUTURE DIRECTIONS | 103 | ||
| SUMMARY/DISCUSSION | 103 | ||
| REFERENCES | 104 | ||
| 9 - Lung Transplantation in Interstitial Lung Disease | 107 | ||
| WHO SHOULD BE REFERRED AND BE LISTED FOR TRANSPLANT? | 108 | ||
| Choice of Procedure | 108 | ||
| OUTCOMES AFTER TRANSPLANTATION FOR INTERSTITIAL LUNG DISEASE | 110 | ||
| DISEASE-SPECIFIC CONSIDERATIONS | 112 | ||
| Idiopathic Pulmonary Fibrosis | 112 | ||
| Sarcoidosis | 114 | ||
| Lymphangioleiomyomatosis | 115 | ||
| Pulmonary Langerhans Cell Histiocytosis | 115 | ||
| SUMMARY | 115 | ||
| REFERENCES | 116 | ||
| 10 - Idiopathic Pulmonary Fibrosis: Phenotypes and Comorbidities | 121 | ||
| ARE THERE DISTINCT PHENOTYPES IN IDIOPATHIC PULMONARY FIBROSIS? | 121 | ||
| Combined Pulmonary Fibrosis and Emphysema | 121 | ||
| Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis | 122 | ||
| Rapidly Progressive Idiopathic Pulmonary Fibrosis | 123 | ||
| Gastroesophageal Reflux Disease | 124 | ||
| Cardiovascular Disease and Venous Thromboembolic Disease | 124 | ||
| Lung Cancer | 125 | ||
| Depression | 125 | ||
| Sleep Apnea and Idiopathic Pulmonary Fibrosis | 125 | ||
| Diabetes Mellitus | 126 | ||
| SUMMARY | 126 | ||
| REFERENCES | 126 | ||
| 11 - Acute Exacerbations in Patients With Idiopathic Pulmonary Fibrosis | 131 | ||
| ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS: UNRECOGNIZED OTHER CONDITIONS? | 132 | ||
| An Occult Infection? | 132 | ||
| Silent Aspiration of Gastric Contents? | 132 | ||
| ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS: AN ACCELERATION OF IDIOPATHIC PULMONARY FIBROSIS PROCESS? | 132 | ||
| TRIGGERING FACTORS | 133 | ||
| RISK FACTORS | 133 | ||
| BIOMARKERS | 134 | ||
| NEW DEFINITION FOR ACUTE RESPIRATORY DETERIORATION IN IDIOPATHIC PULMONARY FIBROSIS | 134 | ||
| NEW DIAGNOSTIC CRITERIA | 134 | ||
| MANAGEMENT | 134 | ||
| PROGNOSIS | 135 | ||
| PREVENTION | 135 | ||
| CONCLUSION | 135 | ||
| REFERENCES | 136 | ||
| 12 - Histopathologic Approach to the Surgical Lung Biopsy in Interstitial Lung Disease | 141 | ||
| WHAT DOES NORMAL LOOK LIKE? | 141 | ||
| IS THIS ACUTE LUNG INJURY? | 142 | ||
| IS THERE FIBROSIS? | 144 | ||
| IS THIS USUAL INTERSTITIAL PNEUMONIA? | 144 | ||
| IS THIS NONSPECIFIC INTERSTITIAL PNEUMONIA? | 147 | ||
| IS THE FIBROSIS BRONCHIOLOCENTRIC? | 148 | ||
| IS THE BIOPSY TOO CELLULAR OR INFLAMED? | 148 | ||
| IS THIS CELLULAR NONSPECIFIC INTERSTITIAL PNEUMONIA? | 148 | ||
| ARE THERE GRANULOMAS? | 149 | ||
| HOW CAN THE GRANULOMAS BE FURTHER CLASSIFIED? | 149 | ||
| Sarcoidal Granulomas | 149 | ||
| Necrotizing Granulomas | 150 | ||
| Interstitial Lung Disease With Associated Small Granulomas | 151 | ||
| OTHER CAUSES OF CELLULAR INTERSTITIAL PNEUMONIA WITH GRANULOMAS | 152 | ||
| THIS LOOKS NORMAL, WHAT AM I MISSING? | 152 | ||
| Obliterative Bronchiolitis | 152 | ||
| Pulmonary Hypertension | 153 | ||
| IS THERE A DIAGNOSIS THAT I AM FORGETTING? | 153 | ||
| REFERENCES | 154 | ||
| 13 - Interstitial Lung Disease in the Connective Tissue Diseases | 157 | ||
| GENERAL APPROACH | 158 | ||
| Respiratory Symptoms | 158 | ||
| Other Review of Systems | 158 | ||
| Physical Examination | 158 | ||
| Serologic Testing | 158 | ||
| Pulmonary Function Tests | 159 | ||
| Chest Imaging | 159 | ||
| Bronchoalveolar Lavage | 162 | ||
| Pathology | 162 | ||
| Supportive therapy | 164 | ||
| Treatment of comorbidities | 164 | ||
| Lung transplantation | 164 | ||
| RHEUMATOID ARTHRITIS | 165 | ||
| Clinical Features | 165 | ||
| Radiographic Features | 166 | ||
| Pathologic Features | 166 | ||
| Treatment | 167 | ||
| SYSTEMIC SCLEROSIS (SCLERODERMA) | 167 | ||
| Pulmonary Function Tests | 168 | ||
| Radiographic Features | 168 | ||
| Pathologic Features | 168 | ||
| Treatment | 169 | ||
| IDIOPATHIC INFLAMMATORY MYOPATHIES | 170 | ||
| Clinical Features | 170 | ||
| Pulmonary Function Testing | 171 | ||
| Radiographic Features | 171 | ||
| Pathologic Features | 171 | ||
| Treatment | 172 | ||
| SJÖGREN SYNDROME | 172 | ||
| Clinical Features | 173 | ||
| Radiographic Features | 173 | ||
| Pathologic Features | 173 | ||
| Treatment | 174 | ||
| SYSTEMIC LUPUS ERYTHEMATOSUS | 174 | ||
| Acute Lupus Pneumonitis | 174 | ||
| Diffuse Alveolar Hemorrhage | 174 | ||
| Chronic Interstitial Lung Disease in Systemic Lupus Erythematosus | 175 | ||
| SUMMARY | 175 | ||
| REFERENCES | 175 | ||
| Index | 187 | ||
| A | 187 | ||
| B | 187 | ||
| C | 187 | ||
| D | 188 | ||
| E | 188 | ||
| F | 188 | ||
| G | 188 | ||
| H | 188 | ||
| I | 188 | ||
| J | 189 | ||
| K | 189 | ||
| L | 189 | ||
| M | 189 | ||
| N | 189 | ||
| O | 189 | ||
| P | 189 | ||
| R | 190 | ||
| S | 190 | ||
| T | 190 | ||
| U | 190 | ||
| V | 190 | ||
| W | 190 |