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Abstract
Get a quick, expert overview of the etiology, diagnosis, and management of pulmonary and extra pulmonary sarcoidosis with this concise, practical resource. Drs. Robert B. Baughman and Dominique Valeyre fully cover the recent advances in various aspects of this disease, including new genetic studies and new diagnostic techniques. It’s an ideal resource for pulmonologists and respiratory medicine specialists, as well as primary care physicians and pulmonary/respiratory care nurses.
- Provides a comprehensive discussion of the various facets of sarcoidosis, including common manifestations of the lung, skin, and eyes, as well as other important aspects such as cardiac and neurologic disease.
- Covers newer diagnostic techniques for the lungs and elsewhere in the body, each discussed in detail and compared to older diagnostic techniques.
- Discusses treatment options including anti-inflammatory drugs, and management of other aspects of the disease, such as pulmonary hypertension, fatigue, and small fiber neuropathy.
- Consolidates today’s available information and experience in this important area into one convenient resource.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Sarcoidosis: A Clinician’s Guide | i | ||
| Sarcoidosis: A Clinician’s Guide | iii | ||
| Copyright | iv | ||
| List of Contributors | v | ||
| Preface | xi | ||
| Contents | xiii | ||
| I - EPIDEMIOLOGY AND CAUSES | 1 | ||
| 1 - Geoepidemiology of Sarcoidosis | 1 | ||
| INTRODUCTION | 1 | ||
| PREVALENCE AND INCIDENCE | 1 | ||
| AGE AND GENDER | 1 | ||
| ETHNICITY | 5 | ||
| How Ethnicity Influences the Clinical Presentation | 6 | ||
| How Ethnicity Influences the Outcome | 8 | ||
| GEOLOCATION | 9 | ||
| ENVIRONMENTAL EXPOSURES | 10 | ||
| Area of Residence | 10 | ||
| Occupational Exposure | 11 | ||
| Agriculture | 11 | ||
| Water-related jobs | 11 | ||
| Construction-related occupations | 13 | ||
| Metal-related jobs | 13 | ||
| Education and health occupations | 13 | ||
| Services and caring occupations | 13 | ||
| Household Exposures | 13 | ||
| LIFESTYLE AND SOCIOECONOMIC FACTORS | 14 | ||
| Obesity | 14 | ||
| Leisure activities | 14 | ||
| Other | 14 | ||
| Socioeconomic Factors | 14 | ||
| COMBINING GEOEPIDEMIOLOGICAL RISK FACTORS | 14 | ||
| CONCLUSIONS | 15 | ||
| REFERENCES | 16 | ||
| 2 - Environmental and Infectious Causes of Sarcoidosis | 23 | ||
| ABBREVIATION | 23 | ||
| INTRODUCTION | 23 | ||
| Environmental Triggers | 23 | ||
| Bacteria | 24 | ||
| Mycobacteria | 24 | ||
| Propionibacteria | 26 | ||
| Fungi: Indoor Air and Mold | 26 | ||
| OTHER MICROBIAL AGENTS | 27 | ||
| Occupational Hazard and Exposure to Organic/Inorganic Particulate Matter | 27 | ||
| REFERENCES | 29 | ||
| 3 - Immunological Manifestations in Sarcoidosis | 37 | ||
| INTRODUCTION | 37 | ||
| INNATE AND ADAPTIVE IMMUNE RESPONSES | 37 | ||
| INNATE RESPONSE IN SARCOIDOSIS | 37 | ||
| Macrophages | 37 | ||
| Monocytes | 39 | ||
| Trained Innate Immunity | 40 | ||
| BRIDGING INNATE-ADAPTIVE IN SARCOIDOSIS | 41 | ||
| Dendritic Cells | 41 | ||
| ADAPTIVE IMMUNE RESPONSE IN SARCOIDOSIS | 43 | ||
| Th-Cell Response | 43 | ||
| Th-Cell Suppressive Mechanisms in Sarcoidosis | 44 | ||
| B-Cell Responses in Sarcoidosis | 47 | ||
| TIPPING THE BALANCE TOWARD PROINFLAMMATORY IMMUNE RESPONSES IN SARCOIDOSIS | 47 | ||
| CLINICAL IMPLICATIONS | 48 | ||
| REFERENCES | 48 | ||
| 4 - Genetics of Sarcoidosis | 55 | ||
| EVIDENCE FOR A GENETIC PREDISPOSITION TO SARCOIDOSIS | 55 | ||
| Familial Clustering | 55 | ||
| GENETIC STUDIES | 55 | ||
| SCANNING THE GENOME | 56 | ||
| Genome-Wide Studies in Caucasians | 56 | ||
| Genome-Wide Studies in Non-Caucasians | 57 | ||
| HLA GENES | 58 | ||
| HLA Class I | 58 | ||
| HLA Class II | 58 | ||
| HLA Class III | 59 | ||
| Löfgren’s Syndrome | 59 | ||
| ASSOCIATIONS WITH ORGAN-SPECIFIC MANIFESTATIONS AND DISEASE BEHAVIOR | 60 | ||
| ADDITIONAL GENES ASSOCIATED WITH SA | 60 | ||
| GENE EXPRESSION STUDIES | 62 | ||
| CONCLUSIONS | 62 | ||
| REFERENCES | 62 | ||
| 5 - Sarcoidosis Models: Past, Present, and Future | 67 | ||
| INTRODUCTION | 67 | ||
| MECHANISMS | 67 | ||
| SARCOIDOSIS MODELS | 68 | ||
| In Vitro Sarcoidosis Models | 68 | ||
| In Vivo Sarcoidosis Models | 70 | ||
| Ex Vivo Tissue Models | 70 | ||
| In Silico Models | 71 | ||
| CONCLUSION | 71 | ||
| REFERENCES | 72 | ||
| 6 - Pathology of Granuloma | 75 | ||
| THE EPITHELIOID GRANULOMA IN SARCOIDOSIS | 75 | ||
| GRANULOMA IN PULMONARY SARCOIDOSIS | 75 | ||
| GRANULOMA IN SKIN SARCOIDOSIS | 78 | ||
| CONSTITUTION AND EVOLUTION OF GRANULOMA IN SARCOIDOSIS | 79 | ||
| EXPERIMENTAL MODELS OF GRANULOMAS | 80 | ||
| CONCLUSION | 81 | ||
| REFERENCES | 81 | ||
| II - DIAGNOSIS | 85 | ||
| 7 - Baughman’s Sarcoidosis: Diagnosis | 85 | ||
| INTRODUCTION | 85 | ||
| GENERAL PRINCIPLES | 85 | ||
| DIFFERENTIAL DIAGNOSIS | 86 | ||
| SYMPTOMATOLOGY | 86 | ||
| SEROLOGY | 87 | ||
| IMAGING | 88 | ||
| ENDOSCOPIC DIAGNOSTICS | 89 | ||
| Bronchoalveolar Lavage | 89 | ||
| Diagnostic Yield of Bronchoscopic Techniques | 90 | ||
| HISTOPATHOLOGY | 91 | ||
| GLOBAL PATIENT ASSESSMENT | 92 | ||
| REFERENCES | 92 | ||
| 8 - Bronchoscopic Modalities to Diagnose Sarcoidosis | 97 | ||
| INTRODUCTION | 97 | ||
| METHODS | 97 | ||
| BRONCHOALVEOLAR LAVAGE | 97 | ||
| ENDOBRONCHIAL BIOPSY | 97 | ||
| CONVENTIONAL TRANSBRONCHIAL NEEDLE ASPIRATION | 98 | ||
| ENDOBRONCHIAL ULTRASOUND-GUIDED TRANSBRONCHIAL NEEDLE ASPIRATION | 98 | ||
| ESOPHAGEAL ULTRASOUND-GUIDED FINE NEEDLE ASPIRATION | 99 | ||
| TRANSBRONCHIAL LUNG BIOPSY | 99 | ||
| TRANSBRONCHIAL LUNG CRYOBIOPSY | 99 | ||
| ENDOBRONCHIAL ULTRASOUND LYMPH NODE MINIFORCEPS BIOPSY | 100 | ||
| DISCUSSION | 100 | ||
| CONCLUSION | 102 | ||
| REFERENCES | 103 | ||
| 9 - Cardiac Sarcoidosis: Questions for the Clinician | 105 | ||
| INTRODUCTION | 105 | ||
| Who Do You Screen for CS? | 105 | ||
| How Do You Diagnose CS? | 106 | ||
| Who Should Be Treated for CS and How Should Treatment Be Monitored? | 109 | ||
| Immunosuppression | 109 | ||
| Treatment monitoring with FDG-PET | 110 | ||
| ICD placement | 111 | ||
| What Constitutes “Complicated CS” and Who/When Do You Refer to a CS Specialist? | 111 | ||
| SUMMARY AND CONCLUSIONS | 112 | ||
| REFERENCES | 112 | ||
| 10 - Neurosarcoidosis | 115 | ||
| CLINICAL PRESENTATIONS OF NEUROSARCOIDOSIS | 115 | ||
| CENTRAL NERVOUS SYSTEM LOCALIZATIONS OF SARCOIDOSIS | 115 | ||
| PNS LOCALIZATIONS OF SARCOIDOSIS | 118 | ||
| EXTRANEUROLOGICAL INVOLVEMENT DURING NEUROSARCOIDOSIS | 118 | ||
| BIOLOGICAL CHARACTERISTICS OF NEUROSARCOIDOSIS | 118 | ||
| IMAGING IN NEUROSARCOIDOIS | 118 | ||
| DIAGNOSIS CRITERIA | 119 | ||
| MANAGEMENT OF NEUROLOGICAL SIGNS OCCURRING AT ONSET OR DURING THE COURSE OF SARCOIDOSIS | 121 | ||
| TREATMENTS OF NEUROSARCOIDOSIS | 122 | ||
| OUTCOMES | 122 | ||
| CONCLUSIONS | 123 | ||
| REFERENCES | 123 | ||
| 11 - Cutaneous Sarcoidosis | 127 | ||
| HISTORY | 127 | ||
| EPIDEMIOLOGY | 127 | ||
| PATHOPHYSIOLOGY | 127 | ||
| CLINICAL MANIFESTATIONS | 128 | ||
| Specific Cutaneous Sarcoidosis Findings | 128 | ||
| Papules and papulonodules | 128 | ||
| Plaques | 129 | ||
| Scar sarcoidosis | 130 | ||
| Lupus pernio | 131 | ||
| Subcutaneous nodules | 132 | ||
| Atrophic and ulcerative | 132 | ||
| Genital | 133 | ||
| Mucosal | 133 | ||
| Erythroderma | 133 | ||
| Alopecic | 133 | ||
| Nail sarcoidosis | 133 | ||
| Macules and patches | 134 | ||
| DIAGNOSIS AND EVALUATION | 134 | ||
| HISTOPATHOLOGY | 135 | ||
| TREATMENT | 136 | ||
| First line | 136 | ||
| Second line | 136 | ||
| Antimalarials | 138 | ||
| Phosphodiesterase inhibitors | 138 | ||
| Acthar | 139 | ||
| Methotrexate | 139 | ||
| Thalidomide and its derivatives | 139 | ||
| TNF inhibitors | 139 | ||
| Mycophenolate mofetil and azathioprine | 140 | ||
| Future Therapies | 140 | ||
| REFERENCES | 140 | ||
| 12 - Ocular Sarcoidosis | 145 | ||
| INTRODUCTION | 145 | ||
| Pathogenesis | 145 | ||
| Epidemiology | 145 | ||
| CLINICAL PRESENTATION | 146 | ||
| Nonuveitis Ocular Manifestations | 149 | ||
| Neuro-Ophthalmic Sarcoidosis | 149 | ||
| DIAGNOSTIC APPROACH | 150 | ||
| Diagnostic Criteria | 150 | ||
| Diagnostic Evaluation | 150 | ||
| Treatment | 151 | ||
| CONCLUSION | 153 | ||
| REFERENCES | 153 | ||
| 13 - Other Extra-pulmonary Visceral Involvement | 157 | ||
| HEPATIC AND SPLENIC SARCOIDOSIS | 157 | ||
| RENAL SARCOIDOSIS | 158 | ||
| PAROTID GLAND SARCOIDOSIS | 158 | ||
| SINONASAL AND LARYNGEAL SARCOIDOSIS | 160 | ||
| OSSEOUS SARCOIDOSIS | 161 | ||
| OTHERS (UTERUS, BREAST, TESTICULAR, AND DIGESTIVE TRACT SARCOIDOSIS) | 163 | ||
| REFERENCES | 163 | ||
| 14 - Parasarcoidosis Syndromes | 167 | ||
| INTRODUCTION | 167 | ||
| SMALL-FIBER NEUROPATHY | 167 | ||
| FATIGUE | 169 | ||
| VITAMIN D DYSREGULATION | 170 | ||
| ERYTHEMA NODOSUM | 171 | ||
| PAIN SYNDROMES | 172 | ||
| DEPRESSION | 172 | ||
| COGNITIVE IMPAIRMENT | 173 | ||
| SUMMARY | 173 | ||
| REFERENCES | 173 | ||
| III - MONITORING | 179 | ||
| 15 - Evaluation of Pulmonary Sarcoidosis | 179 | ||
| INTRODUCTION | 179 | ||
| WHAT IS THE IMPACT OF PULMONARY SARCOIDOSIS AND HOW TO ASSESS IT AT DIAGNOSIS WORKUP? | 179 | ||
| HOW TO MONITOR PULMONARY DISEASE OUTCOME TO FULFILL THE GOALS DESIGNED FOR THE PATIENT’S CARE, AT WHAT FREQUENCY, WHICH INVESTIG... | 181 | ||
| Goals | 181 | ||
| Visits, Agenda, and Modalities | 181 | ||
| OUTCOME OF PULMONARY SARCOIDOSIS | 183 | ||
| The Different Patterns of Pulmonary Sarcoidosis Evolution | 183 | ||
| Survey of treated patients and response assessment | 183 | ||
| How to diagnose exacerbations or relapses | 184 | ||
| Recurrence of sarcoidosis and recovery | 184 | ||
| SPECIAL SITUATIONS | 184 | ||
| Pulmonary Fibrosis | 184 | ||
| Chronic Pulmonary Aspergillosis | 185 | ||
| Pneumothorax | 185 | ||
| Pulmonary Hypertension | 185 | ||
| Comorbidities | 185 | ||
| Pregnancy | 185 | ||
| CONCLUSION | 185 | ||
| REFERENCES | 185 | ||
| 16 - Roentgenogram, CT, and MRI | 189 | ||
| INTRODUCTION | 189 | ||
| CHEST RADIOGRAPHY | 189 | ||
| Staging Systems | 189 | ||
| Complications | 190 | ||
| COMPUTED TOMOGRAPHY | 190 | ||
| Airway Involvement | 195 | ||
| Complications | 195 | ||
| Functional and Prognostic Evaluation | 195 | ||
| MAGNETIC RESONANCE IMAGING | 196 | ||
| Lung involvement | 197 | ||
| Future directions | 197 | ||
| REFERENCES | 197 | ||
| 17 - Nuclear Imaging | 201 | ||
| INTRODUCTION | 201 | ||
| FLUDEOXYGLUCOSE–POSITRON EMISSION TOMOGRAPHY/COMPUTED TOMOGRAPHY | 201 | ||
| FDG-PET/CT Preparation and Protocol | 201 | ||
| FDG-PET/CT Compared to Biomarkers, Symptoms, and Pulmonary Function Tests | 201 | ||
| FDG-PET/CT and Chest Radiography | 202 | ||
| FDG-PET/CT in Treatment Monitoring | 202 | ||
| MOLECULAR IMAGING IN CARDIAC SARCOIDOSIS | 202 | ||
| FDG-PET/CT in CS | 203 | ||
| FDG-PET/CT and Myocardial Blood Flow | 203 | ||
| FDG-PET/CT in Monitoring | 203 | ||
| Hybrid CMR/PET in CS | 203 | ||
| FUTURE RADIONUCLIDE IMAGING IN SARCOIDOSIS ACTIVITY | 204 | ||
| CONCLUSIONS | 204 | ||
| REFERENCES | 207 | ||
| 18 - Quality of Life | 209 | ||
| INTRODUCTION TO HEALTH-RELATED QUALITY OF LIFE | 209 | ||
| PATIENT-REPORTED OUTCOME (PRO) | 211 | ||
| VALIDATION OF HRQOL ASSESSMENT TOOLS | 211 | ||
| GENERIC HRQOL ASSESSMENT TOOLS USED IN SARCOIDOSIS | 211 | ||
| Short Form-36 | 212 | ||
| World Health Organization Quality of Life Assessment-100 | 212 | ||
| St George’s Respiratory Questionnaire | 213 | ||
| Fatigue Questionnaires | 213 | ||
| Dyspnea, Cough, and Depression | 213 | ||
| SARCOIDOSIS-SPECIFIC HRQOL ASSESSMENT TOOLS | 214 | ||
| Sarcoidosis Health Questionnaire | 214 | ||
| The Kings Sarcoidosis Questionnaire | 214 | ||
| The Sarcoidosis Assessment Tool | 215 | ||
| CONCLUSIONS AND FUTURE WORK | 215 | ||
| REFERENCES | 215 | ||
| 19 - Biomarkers in Sarcoidosis | 219 | ||
| SERUM AND BAL BIOMARKERS IN PULMONARY AND EXTRAPULMONARY SARCOIDOSIS | 219 | ||
| CONVENTIONAL BIOMARKERS | 219 | ||
| Serum Angiotensin-1–Converting Enzyme | 219 | ||
| Serum Soluble Interleukin-2 Receptor | 222 | ||
| Serum Calcium and Urinary Calcium | 222 | ||
| Lysozyme | 226 | ||
| BAL Fluid Characteristics | 226 | ||
| Percentage of Lymphocytes | 226 | ||
| CD4+/CD8+ Ratio | 226 | ||
| CD103+CD4+/CD4+ Ratio | 232 | ||
| POTENTIAL BIOMARKERS | 232 | ||
| Chitotriosidase | 232 | ||
| Th17-Cells and Tregs | 232 | ||
| Serum Amyloid A | 232 | ||
| Chemokines | 232 | ||
| Future Purposes: Omics | 233 | ||
| CONCLUSION | 233 | ||
| REFERENCES | 233 | ||
| IV - TREATMENT AND COMPLICATIONS | 239 | ||
| 20 - Antiinflammatory Therapy | 239 | ||
| INTRODUCTION | 239 | ||
| INDIVIDUAL TREATMENTS OF SARCOIDOSIS | 239 | ||
| FIRST LINE | 240 | ||
| SECOND LINE | 242 | ||
| STEP THREE | 245 | ||
| FOURTH LINE | 248 | ||
| CONCLUSION | 250 | ||
| 21 - Sarcoidosis-Associated Disability | 257 | ||
| INTRODUCTION | 257 | ||
| Assessment of Symptom Burden and Disability | 257 | ||
| Fatigue | 258 | ||
| Cause, risk factors, and diagnosis of fatigue | 258 | ||
| Predictors of sarcoidosis-associated fatigue | 258 | ||
| Treatment options of fatigue | 258 | ||
| Assessment of fatigue | 259 | ||
| Everyday Cognitive Failure | 259 | ||
| Depressive Symptoms and Anxiety | 259 | ||
| Neurobiological Abnormalities | 259 | ||
| SFN-Associated Symptoms | 260 | ||
| Overall Impact on QoL | 260 | ||
| Disability Due to Sarcoidosis | 262 | ||
| Role of Self-Management? | 262 | ||
| CONCLUSION/SUMMARY | 262 | ||
| ACKNOWLEDGMENTS | 263 | ||
| REFERENCES | 263 | ||
| 22 - Calcium Metabolism and Bone Health in Sarcoidosis | 265 | ||
| INTRODUCTION | 265 | ||
| Epidemiology | 265 | ||
| Perplexing Problem of Calcium and Vitamin D in Sarcoidosis | 266 | ||
| Calcium Metabolism in Sarcoidosis Patients | 266 | ||
| Diagnosis and Treatment of Hypercalcemia | 268 | ||
| Bone Health in Sarcoidosis | 268 | ||
| Bone Mineral Density and Sarcoidosis | 269 | ||
| Bone Fracture Risk in Sarcoidosis | 269 | ||
| BONE FRAGILITY TREATMENT | 270 | ||
| Efficacy and Safety of Vitamin D Supplementation in Sarcoidosis | 271 | ||
| Efficacy | 271 | ||
| Safety | 271 | ||
| PREVENTION OF CORTICOSTEROID-INDUCED BONE LOSS | 272 | ||
| SUMMARY AND CONCLUSION | 273 | ||
| REFERENCES | 273 | ||
| 23 - Nonpharmacological Therapy for Pulmonary Sarcoidosis | 277 | ||
| INTRODUCTION | 277 | ||
| Proximal Airway Stenosis | 277 | ||
| Aspergilloma | 277 | ||
| Airway/cavity instillation of antifungals | 278 | ||
| Bronchial artery embolization | 278 | ||
| Surgical resection | 279 | ||
| Pulmonary Hypertension | 280 | ||
| Lung Transplant | 280 | ||
| PULMONARY REHABILITATION | 282 | ||
| REFERENCES | 282 | ||
| 24 - Pulmonary Hypertension Associated With Sarcoidosis | 285 | ||
| CLASSIFICATION OF PH | 285 | ||
| FREQUENCY OF SARCOIDOSIS-ASSOCIATED PH | 285 | ||
| MECHANISMS OF SARCOIDOSIS PH | 287 | ||
| Specific pulmonary vasculopathy | 288 | ||
| Extrinsic compression of pulmonary vessels | 289 | ||
| Portal hypertension | 290 | ||
| Postcapillary PH | 291 | ||
| Comorbidities | 291 | ||
| DIAGNOSIS OF SARCOIDOSIS PH | 291 | ||
| Clinical Presentation | 291 | ||
| Transthoracic Doppler Echocardiography | 291 | ||
| Right Heart Catheterization | 292 | ||
| Pulmonary Function Tests and Six-Minute Walk Test | 292 | ||
| Imaging | 292 | ||
| Natriuretic Peptides | 294 | ||
| SCREENING OF SARCOIDOSIS PH | 294 | ||
| CLINICAL BURDEN AND PROGNOSIS OF SARCOIDOSIS PH | 295 | ||
| TREATMENT OF SARCOIDOSIS PH | 296 | ||
| CONCLUSION | 299 | ||
| REFERENCES | 300 | ||
| 25 - Mortality in Sarcoidosis | 305 | ||
| INTRODUCTION | 305 | ||
| PREVALENCE AND CAUSES OF SARCOIDOSIS-RELATED DEATHS | 305 | ||
| IS SARCOIDOSIS-RELATED MORTALITY SIGNIFICANTLY UNDERRECOGNIZED? | 306 | ||
| MORTALITY DATA AND THE PERCEPTION THAT SARCOIDOSIS IS A BENIGN DISEASE | 307 | ||
| ORGAN-BASED MORTALITY RISK STRATIFICATION | 308 | ||
| CONCLUSION | 310 | ||
| REFERENCES | 311 | ||
| Index | 313 | ||
| A | 313 | ||
| B | 313 | ||
| C | 314 | ||
| D | 315 | ||
| E | 315 | ||
| F | 315 | ||
| G | 316 | ||
| H | 316 | ||
| I | 317 | ||
| J | 317 | ||
| K | 317 | ||
| L | 317 | ||
| M | 318 | ||
| N | 318 | ||
| O | 318 | ||
| P | 319 | ||
| Q | 320 | ||
| R | 320 | ||
| S | 320 | ||
| T | 320 | ||
| U | 321 | ||
| V | 321 | ||
| W | 321 | ||
| Z | 321 |