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Book Details
Abstract
This issue of Clinics in Chest Medicine focuses on Sarciodosis. Drs. Baughman and Culver have put together an expert roster of authors for articles concerning: Etiology of sarcoidosis, Immunology of sarcoidosis, Genetics of Sarcoidosis, Diagnosis of sarcoidosis, Chest imaging, Biomarkers and genetic profiles, Pulmonary Sarcoidosis, Neurosarcoidosis, Cardiac Sarcoidosis, Ocular Sarcoidosis, Quality of life assessments, and more!
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Sarcoidosis\r | i | ||
| Copyright\r | ii | ||
| Contributors | iii | ||
| EDITORS | iii | ||
| AUTHORS | iii | ||
| Contents | vii | ||
| Preface: Sarcoidosis\r | vii | ||
| Cellular Players in the Immunopathogenesis of Sarcoidosis\r | vii | ||
| The Etiologic Role of Infectious Antigens in Sarcoidosis Pathogenesis\r | vii | ||
| Genetics of Sarcoidosis\r | vii | ||
| The Diagnosis of Sarcoidosis\r | vii | ||
| Chest Imaging\r | viii | ||
| Identifying Novel Biomarkers in Sarcoidosis Using Genome-Based Approaches\r | viii | ||
| Pulmonary Sarcoidosis\r | viii | ||
| Neurosarcoidosis\r | viii | ||
| Cardiac Sarcoidosis\r | ix | ||
| Ocular Sarcoidosis\r | ix | ||
| Cutaneous Sarcoidosis\r | ix | ||
| Pulmonary Hypertension in Sarcoidosis\r | ix | ||
| Severe Sarcoidosis\r | x | ||
| Consequences of Sarcoidosis\r | x | ||
| Quality of Life Assessment in Sarcoidosis\r | x | ||
| Treatment of Sarcoidosis\r | x | ||
| PROGRAM OBJECTIVE | xi | ||
| TARGET AUDIENCE | xi | ||
| LEARNING OBJECTIVES | xi | ||
| ACCREDITATION | xi | ||
| DISCLOSURE OF CONFLICTS OF INTEREST | xi | ||
| UNAPPROVED/OFF-LABEL USE DISCLOSURE | xii | ||
| TO ENROLL | xii | ||
| METHOD OF PARTICIPATION | xii | ||
| CME INQUIRIES/SPECIAL NEEDS | xii | ||
| CLINICS IN CHEST MEDICINE\r | xiii | ||
| FORTHCOMING ISSUES | xiii | ||
| March 2016 | xiii | ||
| June 2016 | xiii | ||
| September 2016 | xiii | ||
| RECENT ISSUES | xiii | ||
| September 2015 | xiii | ||
| June 2015 | xiii | ||
| March 2015 | xiii | ||
| Preface: Sarcoidosis \r | xv | ||
| Cellular Players in the Immunopathogenesis of Sarcoidosis | 549 | ||
| Key points | 549 | ||
| INTRODUCTION | 549 | ||
| THE INNATE IMMUNE SYSTEM | 550 | ||
| Receptors of the Innate Immune System | 550 | ||
| Neutrophil Granulocytes | 550 | ||
| The Etiologic Role of Infectious Antigens in Sarcoidosis Pathogenesis | 561 | ||
| Key points | 561 | ||
| SARCOIDOSIS EPIDEMIOLOGY SUGGESTS EXPOSURE TO MICROBIAL BIOAEROSOLS | 561 | ||
| MOLECULAR AND IMMUNOLOGIC INVESTIGATIONS REVEAL MICROBIAL PROTEINS AND DNA | 562 | ||
| IMMUNE RESPONSES AGAINST MYCOBACTERIAL VIRULENCE FACTORS ARE PRESENT IN SYSTEMIC AND ACTIVE SARCOIDOSIS INVOLVEMENT | 563 | ||
| NONINFECTIOUS ETIOLOGIES OF SARCOIDOSIS | 564 | ||
| MICROBIAL INDUCTION OF SARCOIDOSIS CD4+ T-CELL DYSFUNCTION | 564 | ||
| CLINICAL TRIALS OF ANTIMICROBIAL THERAPY IN SARCOIDOSIS | 565 | ||
| SUMMARY | 566 | ||
| REFERENCES | 566 | ||
| Genetics of Sarcoidosis | 569 | ||
| Key points | 569 | ||
| INTRODUCTION | 569 | ||
| SUMMARY OF APPROACHES TAKEN IN GENETIC EPIDEMIOLOGY OF SARCOIDOSIS | 569 | ||
| Familial Heritability Studies | 570 | ||
| Familial Linkage Studies | 570 | ||
| Genome-wide Association Studies | 570 | ||
| Candidate Gene Studies | 570 | ||
| Admixture Mapping Studies | 571 | ||
| GENES AND REGIONS ASSOCIATED WITH SARCOIDOSIS | 571 | ||
| Cell Surface Immune Receptors | 571 | ||
| HLA region | 571 | ||
| Butyrophilinlike 2 | 576 | ||
| Toll-like receptors | 576 | ||
| Chemokine receptor type 2 | 577 | ||
| Chemokine receptor type 5 | 577 | ||
| SLC11A1 (NRAMP1, NATURAL RESISTANCE-ASSOCIATED MACROPHAGE PROTEIN GENE) | 577 | ||
| Cytokines | 577 | ||
| Tumor necrosis factor-α and lymphotoxin-A, tumor necrosis factor-β | 577 | ||
| Transforming growth factor-β | 578 | ||
| Interleukin-23 | 578 | ||
| Signaling Molecules | 578 | ||
| Annexin A11 | 578 | ||
| Others Genes Associated with Sarcoidosis in Recent Genome-wide Association Studies | 579 | ||
| Neurogenic locus notch homolog protein 4 | 579 | ||
| Osteosarcoma amplified 9 | 579 | ||
| X-linked inhibitor of apoptosis associated factor | 579 | ||
| GENE ENVIRONMENT INTERACTIONS IN SARCOIDOSIS | 579 | ||
| SARCOIDOSIS GENETICS: A FINGERPRINT OF IMMUNE-MEDIATED DISEASE | 579 | ||
| REFERENCES | 580 | ||
| The Diagnosis of Sarcoidosis | 585 | ||
| Key points | 585 | ||
| NATURE OF THE PROBLEM | 585 | ||
| DEFINITION | 585 | ||
| APPROACH TO DIAGNOSIS | 586 | ||
| Does the Clinical Presentation Justify Consideration of Diagnosis of Sarcoidosis? | 587 | ||
| At-risk populations | 587 | ||
| The physical examination: “Footprints of sarcoidosis” | 590 | ||
| Serologic and radiographic findings | 590 | ||
| Serum angiotensin-converting enzyme | 590 | ||
| Tuberculin skin test and interferon-gamma release assay | 591 | ||
| Asymptomatic bilateral hilar adenopathy on chest radiography | 591 | ||
| Abnormalities in perilymphatic distribution on computed tomography | 591 | ||
| Other tests | 591 | ||
| When and Where to Consider a Tissue Biopsy to Support a Diagnosis of Sarcoidosis | 592 | ||
| Classic presentations where biopsy is not required | 592 | ||
| Selecting a Site for Biopsy | 593 | ||
| Option 1: Sample easily accessible sites first | 593 | ||
| Option 2: Sample intrathoracic disease | 593 | ||
| Option 3: Diagnostic dilemma—When a biopsy site is not easily accessible | 594 | ||
| Have Alternative Explanations for the Clinical Presentation Been Excluded? | 594 | ||
| Pathology of sarcoidal granulomas | 594 | ||
| Atypical pathologic presentations | 594 | ||
| Is There Any Evidence of Additional Organ Involvement? | 600 | ||
| SUMMARY | 600 | ||
| REFERENCES | 600 | ||
| Chest Imaging | 603 | ||
| Key points | 603 | ||
| INTRODUCTION | 603 | ||
| RADIOGRAPHIC SCORING SYSTEMS: SCADDING STAGING | 603 | ||
| LARGE AIRWAY INVOLVEMENT | 604 | ||
| MEDIASTINAL AND HILAR LYMPHADENOPATHY | 604 | ||
| PARENCHYMAL INVOLVEMENT | 606 | ||
| Nodular and Reticulonodular Pattern | 606 | ||
| Large Nodules and Alveolar Sarcoidosis | 606 | ||
| Galaxy Sign, Cluster Sign, and (Reversed) Halo Sign | 606 | ||
| Ground Glass Opacities | 607 | ||
| Fibrotic Sarcoidosis | 608 | ||
| Mosaic Attenuation Pattern and Air Trapping | 608 | ||
| MYCETOMAS | 609 | ||
| PLEURAL INVOLVEMENT | 609 | ||
| Pleural Effusion | 609 | ||
| Chylothorax | 609 | ||
| Pneumothorax | 609 | ||
| NECROTIZING SARCOID GRANULOMATOSIS | 609 | ||
| PULMONARY HYPERTENSION | 610 | ||
| FLUORODEOXYGLUCOSE F 18 PET/COMPUTED TOMOGRAPHY | 610 | ||
| Fluorodeoxyglucose F 18 Uptake in Sarcoidosis | 610 | ||
| Patient Preparation and Fluorodeoxyglucose F 18 PET/Computed Tomography Acquisition in Sarcoidosis | 610 | ||
| Radiation Dose of Fluorodeoxyglucose F 18 PET/Computed Tomography | 611 | ||
| CHEST RADIOGRAPH AND FLUORODEOXYGLUCOSE F 18 PET/COMPUTED TOMOGRAPHY | 611 | ||
| HIGH-RESOLUTION COMPUTED TOMOGRAPHY AND FLUORODEOXYGLUCOSE F 18 PET/COMPUTED TOMOGRAPHY | 611 | ||
| Pulmonary Function and Fluorodeoxyglucose F 18 PET/Computed Tomography | 612 | ||
| Fluorodeoxyglucose F 18 PET/Computed Tomography in Cardiac Sarcoidosis | 612 | ||
| When Should Fluorodeoxyglucose F 18 PET/Computed Tomography Be Used in Thoracic Sarcoidosis? | 615 | ||
| SUMMARY | 615 | ||
| REFERENCES | 615 | ||
| Identifying Novel Biomarkers in Sarcoidosis Using Genome-Based Approaches | 621 | ||
| Key points | 621 | ||
| SARCOIDOSIS: OVERVIEW AND NEED FOR BIOMARKERS | 621 | ||
| Traditional Biomarkers in Sarcoidosis | 622 | ||
| SOLUBLE BIOMARKERS ASSOCIATED WITH MONOCYTE-MACROPHAGE ACTIVATION | 622 | ||
| Serum Amyloid A | 623 | ||
| Lysozyme | 623 | ||
| SOLUBLE BIOMARKERS ASSOCIATED WITH LYMPHOCYTE ORIGIN | 623 | ||
| Soluble Interleukin-2 Receptor | 623 | ||
| BIOMARKERS ASSOCIATED WITH FIBROSIS AND EXTRACELLULAR MATRIX | 623 | ||
| Tenascin-C | 623 | ||
| EMERGING STRATEGIES FOR BIOMARKER DEVELOPMENT | 623 | ||
| SARCOIDOSIS BIOMARKERS IDENTIFIED BY EXPRESSION PROFILING IN LUNG TISSUES | 623 | ||
| GENE SIGNATURES IN BLOOD SERVE AS POTENTIAL DIAGNOSTIC TOOL FOR SARCOIDOSIS | 624 | ||
| DISTINGUISHING PULMONARY SARCOIDOSIS FROM TUBERCULOSIS BY BLOOD GENE EXPRESSION PROFILING | 625 | ||
| HIGH-THROUGHPUT GENETIC STUDIES TO IDENTIFY NOVEL CANDIDATE GENES OF SARCOIDOSIS | 627 | ||
| SUMMARY | 628 | ||
| REFERENCES | 628 | ||
| Pulmonary Sarcoidosis | 631 | ||
| Key points | 631 | ||
| INTRODUCTION | 631 | ||
| PATHOLOGY | 631 | ||
| EPIDEMIOLOGY | 633 | ||
| MODES OF ONSET | 634 | ||
| PULMONARY CLINICAL SIGNS AND INVESTIGATIONS | 634 | ||
| Physical Examination | 634 | ||
| Bronchoalveolar Lavage | 635 | ||
| Pulmonary Function | 636 | ||
| Six-minute Walk Test | 636 | ||
| Cardiopulmonary Exercise Testing | 636 | ||
| PROGNOSTIC FACTORS | 636 | ||
| EVOLUTION | 636 | ||
| Modes of Evolution | 636 | ||
| Follow-up and Events | 637 | ||
| Mortality | 638 | ||
| SUMMARY | 638 | ||
| REFERENCES | 638 | ||
| Neurosarcoidosis | 643 | ||
| Key points | 643 | ||
| INTRODUCTION | 643 | ||
| EPIDEMIOLOGY AND PATHOLOGY | 643 | ||
| CLINICAL PRESENTATION | 644 | ||
| Cranial Nerve | 644 | ||
| Meningeal | 644 | ||
| Intracranial Parenchyma | 645 | ||
| Spinal Cord | 645 | ||
| Neurovascular | 645 | ||
| Neuromuscular | 645 | ||
| Other | 646 | ||
| DIAGNOSTIC TESTING | 646 | ||
| Tissue Biopsy | 646 | ||
| MRI | 647 | ||
| Cerebrospinal Fluid | 648 | ||
| Peripheral Nervous System Testing | 649 | ||
| Other Testing | 649 | ||
| Systemic Evaluation | 650 | ||
| Conjunctival Biopsy | 650 | ||
| Isolated Neurosarcoidosis | 651 | ||
| TREATMENT | 651 | ||
| Corticosteroids | 651 | ||
| Tumor Necrosis Factor-α Antagonists | 652 | ||
| Other Corticosteroid-Sparing Agents | 654 | ||
| SUMMARY | 654 | ||
| REFERENCES | 654 | ||
| Cardiac Sarcoidosis | 657 | ||
| Key points | 657 | ||
| EPIDEMIOLOGY AND CLINICAL MANIFESTATIONS | 657 | ||
| DIAGNOSIS | 657 | ||
| Role of Endomyocardial Biopsy in the Diagnosis of Cardiac Sarcoidosis | 658 | ||
| Screening for Cardiac Involvement in Patients with Biopsy-Proven Extracardiac Sarcoidosis | 658 | ||
| PROGNOSIS | 659 | ||
| Class I recommendations | 659 | ||
| Class IIa recommendations | 659 | ||
| Class III recommendation | 659 | ||
| CLINICAL MANAGEMENT: ROLE OF IMMUNOSUPPRESSION | 660 | ||
| Role of FDG-PET Imaging to Guide Immunosuppression | 660 | ||
| MANAGEMENT OF LEFT VENTRICULAR DYSFUNCTION | 661 | ||
| MANAGEMENT OF CONDUCTION ABNORMALITIES | 662 | ||
| MANAGEMENT OF VENTRICULAR ARRHYTHMIAS | 663 | ||
| Mechanisms of Ventricular Arrhythmias | 663 | ||
| The Role of Immunosuppression | 663 | ||
| Ablation for Ventricular Arrhythmias | 663 | ||
| RISK STRATIFICATION FOR SUDDEN CARDIAC DEATH | 664 | ||
| Left Ventricular Function | 664 | ||
| Class IIa recommendations | 664 | ||
| Cardiac Magnetic Resonance | 665 | ||
| MANAGEMENT OF ATRIAL ARRHYTHMIAS | 665 | ||
| SUMMARY AND FUTURE DIRECTIONS | 665 | ||
| REFERENCES | 666 | ||
| Ocular Sarcoidosis | 669 | ||
| Key points | 669 | ||
| EPIDEMIOLOGY | 669 | ||
| CLINICAL MANIFESTATIONS OF OCULAR SARCOIDOSIS | 670 | ||
| Uveitis and Fundoscopic Abnormalities | 670 | ||
| Optic Nerve Involvement and Neuroophthalmic Manifestations | 672 | ||
| Eyelid and Ocular Surface Disease | 673 | ||
| Lacrimal System and Keratoconjunctivitis Sicca | 673 | ||
| Orbital Involvement | 674 | ||
| Glaucoma and Cataract | 674 | ||
| DIAGNOSIS OF OCULAR SARCOIDOSIS | 674 | ||
| TREATMENT OF OCULAR SARCOIDOSIS | 676 | ||
| Treatment of Sarcoid Uveitis | 676 | ||
| Topical corticosteroids | 676 | ||
| Topical cycloplegics | 676 | ||
| Regional corticosteroid injections and implants | 676 | ||
| Systemic corticosteroids | 677 | ||
| Systemic immunosuppressive agents | 677 | ||
| Biologic agents | 677 | ||
| Treatment of Scleritis and External Eye Diseases | 678 | ||
| Treatment of Orbital Disease | 678 | ||
| Treatment of Ocular Complications | 679 | ||
| PROGNOSIS OF OCULAR SARCOIDOSIS | 679 | ||
| REFERENCES | 679 | ||
| Cutaneous Sarcoidosis | 685 | ||
| Key points | 685 | ||
| INTRODUCTION | 685 | ||
| SKIN PATHOPHYSIOLOGY | 685 | ||
| CLINICAL PRESENTATION | 686 | ||
| Common Morphologies | 686 | ||
| Macules/papules | 686 | ||
| Plaque | 686 | ||
| Lupus pernio | 687 | ||
| Subcutaneous sarcoidosis | 687 | ||
| Scar/tattoo | 688 | ||
| Less Common Morphologies | 689 | ||
| Special sites | 691 | ||
| Scalp | 691 | ||
| Nails | 691 | ||
| Mucosal disease | 691 | ||
| Nonspecific | 692 | ||
| DIAGNOSIS AND WORK-UP | 692 | ||
| DIFFERENTIAL DIAGNOSIS | 693 | ||
| TREATMENT | 693 | ||
| SUMMARY | 697 | ||
| REFERENCES | 697 | ||
| Pulmonary Hypertension in Sarcoidosis | 703 | ||
| Key points | 703 | ||
| INTRODUCTION | 703 | ||
| CAUSE OF SARCOIDOSIS-ASSOCIATED PULMONARY HYPERTENSION | 703 | ||
| EPIDEMIOLOGY | 705 | ||
| DIAGNOSIS OF SARCOIDOSIS-ASSOCIATED PULMONARY HYPERTENSION | 706 | ||
| TREATMENT | 709 | ||
| SURVIVAL | 711 | ||
| SUMMARY | 711 | ||
| REFERENCES | 711 | ||
| Severe Sarcoidosis | 715 | ||
| Key points | 715 | ||
| INTRODUCTION | 715 | ||
| SEVERE PULMONARY INVOLVEMENT | 716 | ||
| The Definition of Severe Pulmonary Fibrosis | 716 | ||
| Pulmonary Risk Factors for Mortality and Future Severe/Dangerous Disease | 719 | ||
| Pulmonary Hypertension in Sarcoidosis | 720 | ||
| SEVERE CARDIAC INVOLVEMENT AND RISK STRATIFICATION | 720 | ||
| TREATMENT OF SEVERE PULMONARY AND CARDIAC SARCOIDOSIS | 721 | ||
| Use High-Dose Therapy Initially | 721 | ||
| Consider the Early Introduction of Second Line Agents | 721 | ||
| Re-evaluate Treatment Strategies When There Is Major Responsiveness | 721 | ||
| Do Not Accept Therapeutic Nihilism in Irreversible Disease | 721 | ||
| Be Proactive in Identifying and Treating Complications | 722 | ||
| SUMMARY | 723 | ||
| REFERENCES | 723 | ||
| Consequences of Sarcoidosis | 727 | ||
| Key points | 727 | ||
| INTRODUCTION | 727 | ||
| SYMPTOMS | 728 | ||
| Fatigue | 728 | ||
| Dyspnea | 729 | ||
| Small Fiber Neuropathy and Autonomic Dysfunction | 729 | ||
| PSYCHOLOGICAL BURDEN | 730 | ||
| Depressive Symptoms | 730 | ||
| Anxiety and Stress | 730 | ||
| Cognitive Impairment and Memory Loss | 731 | ||
| Physical Impairment | 731 | ||
| Overall Impact on Patients’ Lives: Quality of Life | 732 | ||
| TREATMENT OPTIONS | 733 | ||
| Pharmacologic Treatment | 733 | ||
| Additional Alternatives to Pharmacologic Treatment | 733 | ||
| REHABILITATION | 733 | ||
| COMMUNICATION AND PATIENT PARTICIPATION | 734 | ||
| SUMMARY | 734 | ||
| REFERENCES | 735 | ||
| Quality of Life Assessment in Sarcoidosis | 739 | ||
| Key points | 739 | ||
| INTRODUCTION | 739 | ||
| THE IMPORTANCE OF QUALITY OF LIFE ASSESSMENT IN SARCOIDOSIS | 739 | ||
| THE ASSESSMENT OF HEALTH-RELATED QUALITY OF LIFE | 740 | ||
| PROBLEMS IN THE ASSESSMENT OF HEALTH-RELATED QUALITY OF LIFE IN SARCOIDOSIS | 741 | ||
| General Resistance to the Use of Patient-Reported Outcome Measures to Assess Health-Related Quality Of Life | 741 | ||
| Sarcoidosis-specific Resistance to the Use of Patient-Reported Outcome Measures to Assess Health-Related Quality of Life | 741 | ||
| CHARACTERISTICS OF PATIENT-REPORTED OUTCOME MEASURES | 741 | ||
| SPECIFIC HEALTH-RELATED QUALITY OF LIFE MEASURES USED IN SARCOIDOSIS | 745 | ||
| SUMMARY | 748 | ||
| REFERENCES | 749 | ||
| Treatment of Sarcoidosis | 751 | ||
| Key points | 751 | ||
| INTRODUCTION | 751 | ||
| WHOM TO TREAT | 751 | ||
| Prognosis | 751 | ||
| Effect of Treatment on Natural History | 752 | ||
| Approach to Treatment Decisions | 753 | ||
| Extent of disease | 753 | ||
| Severity of disease | 754 | ||
| Activity of disease | 754 | ||
| Impact of disease on patients’ lives | 755 | ||
| Patient Involvement | 755 | ||
| HOW TO TREAT | 755 | ||
| Corticosteroids | 756 | ||
| Inhaled corticosteroids and other topical therapy | 757 | ||
| Cytotoxic Medications | 757 | ||
| Methotrexate | 757 | ||
| Azathioprine | 758 | ||
| Leflunomide | 758 | ||
| Mycophenolate mofetil | 758 | ||
| Biologic Tumor Necrosis Factor Antagonists | 759 | ||
| Infliximab | 759 | ||
| Adalimumab and other anti–tumor necrosis factor biologics | 759 | ||
| Antimalarial Medications | 760 | ||
| Adrenocorticotropic Hormone Gel | 760 | ||
| Other Agents | 761 | ||
| SUMMARY | 761 | ||
| REFERENCES | 761 | ||
| Index | 769 |