- •Preface to the First Edition
- •Preface to the Second Edition
- •Contents
- •Diagnostic Challenges
- •Expert Centers
- •Patient Organizations
- •Clinical Trials
- •Research in Orphan Lung Diseases
- •Orphan Drugs
- •Orphanet
- •Empowerment of Patients
- •Conclusions
- •References
- •Introduction
- •Challenges to Overcome in Order to Undertake Quality Clinical Research
- •Lack of Reliable Data on Prevalence
- •Small Number of Patients
- •Identifying Causation/Disease Pathogenesis
- •Disease Complexity
- •Lack of Access to a Correct Diagnosis
- •Delay in Diagnosis
- •Challenges But Not Negativity
- •Some Success Stories
- •The Means to Overcome the Challenges of Clinical Research: Get Bigger Numbers of Well-Characterized Patients
- •The Importance of Patient Organizations
- •National and International Networks
- •End Points for Trials: Getting Them Right When Numbers Are Small and Change Is Modest
- •Orphan Drug Development
- •Importance of Referral Centers
- •Looking at the Future
- •The Arguments for Progress
- •Concluding Remarks
- •References
- •3: Chronic Bronchiolitis in Adults
- •Introduction
- •Cellular Bronchiolitis
- •Follicular Bronchiolitis
- •Respiratory Bronchiolitis
- •Airway-Centered Interstitial Fibrosis
- •Proliferative Bronchiolitis
- •Diagnosis
- •Chest Imaging Studies
- •Pulmonary Function Testing
- •Lung Biopsy
- •Mineral Dusts
- •Organic Dusts
- •Volatile Flavoring Agents
- •Infectious Causes of Bronchiolitis
- •Idiopathic Forms of Bronchiolitis
- •Connective Tissue Diseases
- •Organ Transplantation
- •Hematopoietic Stem Cell Transplantation
- •Drug-Induced Bronchiolitis
- •Treatment
- •Constrictive Bronchiolitis
- •Follicular Bronchiolitis
- •Airway-Centered Interstitial Fibrosis
- •Proliferative Bronchiolitis
- •References
- •Background and Epidemiology
- •Pathophysiology
- •Host Characteristics
- •Clinical Manifestations
- •Symptoms
- •Laboratory Evaluation
- •Skin Testing
- •Serum Precipitins
- •Eosinophil Count
- •Total Serum Immunoglobulin E Levels
- •Recombinant Antigens
- •Radiographic Imaging
- •Pulmonary Function Testing
- •Histology
- •Diagnostic Criteria
- •Historical Diagnostic Criteria
- •Rosenberg and Patterson Diagnostic Criteria
- •ISHAM Diagnostic Criteria
- •Cystic Fibrosis Foundation Diagnostic Criteria
- •General Diagnostic Recommendations
- •Allergic Aspergillus Sinusitis (AAS)
- •Natural History
- •Treatment
- •Corticosteroids
- •Antifungal Therapy
- •Monoclonal Antibodies
- •Monitoring for Treatment Response
- •Conclusions
- •References
- •5: Orphan Tracheopathies
- •Introduction
- •Anatomical Considerations
- •Clinical Presentation
- •Etiological Considerations
- •Idiopathic Subglottic Stenosis
- •Introduction
- •Clinical Features
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Introduction and Clinical Presentation
- •Clinical Features
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Tracheomalacia
- •Introduction
- •Clinical Features
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Tracheobronchomegaly
- •Introduction
- •Clinical Features
- •Pathophysiology
- •Pulmonary Function Studies
- •Imaging Studies
- •Treatment
- •Tracheopathies Associated with Systemic Diseases
- •Relapsing Polychondritis
- •Introduction
- •Clinical Features
- •Laboratory Findings
- •Pulmonary Function and Imaging Studies
- •Treatment
- •Introduction
- •Clinical Features
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Tracheobronchial Amyloidosis
- •Introduction
- •Clinical Features
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Sarcoidosis
- •Introduction
- •Pulmonary Function Studies
- •Imaging Studies
- •Bronchoscopy
- •Treatment
- •Orphan Tracheopathies: Conclusions
- •References
- •6: Amyloidosis and the Lungs and Airways
- •Introduction
- •Diagnosis and Evaluation of Amyloidosis
- •Systemic AA Amyloidosis
- •Systemic AL Amyloidosis
- •Amyloidosis Localised to the Respiratory Tract
- •Laryngeal Amyloidosis
- •Tracheobronchial Amyloidosis
- •Parenchymal Pulmonary Amyloidosis
- •Pulmonary Amyloidosis Associated with Sjögren’s Disease
- •Conclusions
- •References
- •Introduction
- •Pathophysiology
- •Genetic Predisposition
- •Immune Dysregulation
- •Epidemiology
- •Incidence and Prevalence
- •Triggering Factors
- •Clinical Manifestations
- •General Symptoms
- •Pulmonary Manifestations
- •Ear, Nose, and Throat (ENT) Manifestations
- •Neurological Manifestations
- •Skin Manifestations
- •Cardiac Manifestations
- •Gastrointestinal Involvement
- •Renal Manifestations
- •Ophthalmological Manifestations
- •Complementary Investigations
- •Diagnosis
- •Diagnostic Criteria
- •Prognosis and Outcomes
- •Phenotypes According to the ANCA Status
- •Treatment
- •Therapeutic Strategies
- •Remission Induction
- •Maintenance Therapy
- •Other Treatments
- •Prevention of AEs
- •Conclusions
- •References
- •8: Granulomatosis with Polyangiitis
- •A Brief Historical Overview
- •Epidemiology
- •Pathogenesis
- •Clinical Manifestations
- •Constitutional Symptoms
- •Ear, Nose, and Throat (ENT) Manifestations
- •Pulmonary Manifestations
- •Kidney and Urological Manifestations
- •Kidney Manifestations
- •Urological Manifestations
- •Neurological Manifestations
- •Peripheral Nervous System (PNS) Manifestations
- •Central Nervous System (CNS) Manifestations
- •Spinal Cord and Cranial Nerve Involvement
- •Skin and Oral Mucosal Manifestations
- •Eye Manifestations
- •Cardiac Involvement
- •Gastrointestinal Manifestations
- •Gynecological and Obstetric Manifestations
- •Venous Thrombosis and Other Vascular Events
- •Other Manifestations
- •Pediatric GPA
- •Diagnosis
- •Diagnostic Approach
- •Laboratory Investigations
- •Biology
- •Immunology
- •Pathology
- •Treatment
- •Glucocorticoids
- •Cyclophosphamide
- •Rituximab
- •Other Current Induction Approaches
- •Other Treatments in GPA
- •Intravenous Immunoglobulins
- •Plasma Exchange
- •CTLA4-Ig (Abatacept)
- •Cotrimoxazole
- •Other Agents
- •Principles of Treatment for Relapsing and Refractory GPA
- •Outcomes and Prognostic Factors
- •Survival and Causes of Deaths
- •Relapse
- •Damage and Disease Burden on Quality of Life
- •Conclusions
- •References
- •9: Alveolar Hemorrhage
- •Introduction
- •Clinical Presentation
- •Diagnosis (Table 9.1, Fig. 9.3)
- •Pulmonary Capillaritis
- •Histology (Fig. 9.4)
- •Etiologies
- •ANCA-Associated Small Vessel Vasculitis: Granulomatosis with Polyangiitis (GPA)
- •ANCA-Associated Small Vessel Vasculitis: Microscopic Polyangiitis
- •Isolated Pulmonary Capillaritis
- •Systemic Lupus Erythematosus
- •Antiphospholipid Antibody Syndrome
- •Anti-Basement Membrane Antibody Disease (Goodpasture Syndrome)
- •Lung Allograft Rejection
- •Others
- •Bland Pulmonary Hemorrhage (Fig. 9.5)
- •Histology
- •Etiologies
- •Idiopathic Pulmonary Hemosiderosis
- •Drugs and Medications
- •Coagulopathy
- •Valvular Heart Disease and Left Ventricular Dysfunction
- •Other
- •Histology
- •Etiologies
- •Hematopoietic Stem Cell Transplantation (HSCT)
- •Cocaine Inhalation
- •Acute Exacerbation of Interstitial Lung Disease
- •Acute Interstitial Pneumonia
- •Acute Respiratory Distress Syndrome
- •Miscellaneous Causes
- •Etiologies
- •Pulmonary Capillary Hemangiomatosis
- •Treatment
- •Conclusions
- •References
- •Takayasu Arteritis
- •Epidemiology
- •Pathologic Features
- •Pathogenesis
- •Clinical Features
- •Laboratory Findings
- •Imaging Studies
- •Therapeutic Management
- •Prognosis
- •Behçet’s Disease
- •Epidemiology
- •Pathologic Features
- •Pathogenesis
- •Diagnostic Criteria
- •Clinical Features
- •Pulmonary Artery Aneurysm
- •Pulmonary Artery Thrombosis
- •Pulmonary Parenchymal Involvement
- •Laboratory Findings
- •Imaging Studies
- •Therapeutic Management
- •Treatment of PAA
- •Treatment of PAT
- •Prognosis
- •References
- •Introduction
- •Portopulmonary Hypertension (PoPH)
- •Epidemiology and Risk Factors
- •Molecular Pathogenesis
- •PoPH Treatment
- •Hepatopulmonary Syndrome (HPS)
- •Epidemiology and Risk Factors
- •Molecular Pathogenesis
- •HPS Treatment
- •Conclusion
- •References
- •12: Systemic Sclerosis and the Lung
- •Introduction
- •Risk factors for SSc-ILD
- •Genetic Associations
- •Clinical Presentation of SSc-ILD
- •Pulmonary Function Tests (PFTs)
- •Imaging
- •Management
- •References
- •13: Rheumatoid Arthritis and the Lungs
- •Introduction
- •Epidemiology
- •Risk Factors for ILD (Table 13.3)
- •Pathogenesis
- •Clinical Features and Diagnosis
- •Treatments
- •Prognosis
- •Epidemiology
- •Risk Factors
- •Clinical Features, Diagnosis, and Outcome
- •Subtypes or RA-AD
- •Obliterative Bronchiolitis
- •Bronchiectasis
- •COPD
- •Cricoarytenoid Involvement
- •Pleural Disease
- •Conclusion
- •References
- •Introduction
- •Systemic Lupus Erythematosus
- •Epidemiology
- •Pathophysiology
- •Pulmonary Manifestations
- •Pleural Disease
- •Shrinking Lung Syndrome
- •Thrombotic Manifestations
- •Interstitial Lung Disease
- •Other Pulmonary Manifestations
- •Prognosis
- •Sjögren’s Syndrome
- •Epidemiology
- •Pathophysiology
- •Pulmonary Manifestations
- •Airway Disorders
- •Lymphoproliferative Disease
- •Interstitial Lung Disease
- •Prognosis
- •Mixed Connective Tissue Disease
- •Epidemiology
- •Pathophysiology
- •Pulmonary Manifestations
- •Pulmonary Hypertension
- •Interstitial Lung Disease
- •Prognosis
- •Myositis
- •Epidemiology
- •Pathophysiology
- •Pulmonary Manifestations and Treatments
- •Interstitial Lung Disease
- •Respiratory Muscle Weakness
- •Other Pulmonary Manifestations
- •Prognosis
- •Other Therapeutic Options in CTD-ILD
- •Lung Transplantation
- •Conclusion
- •References
- •Introduction
- •Diagnostic Criteria
- •Controversies in the Diagnostic Criteria
- •Typical Clinical Features
- •Disease Progression and Prognosis
- •Summary
- •References
- •Introduction
- •Histiocytes and Dendritic Cells
- •Introduction
- •Cellular and Molecular Pathogenesis
- •Pathology
- •Clinical Presentation
- •Treatment and Prognosis
- •Erdheim-Chester Disease
- •Epidemiology
- •Cellular and Molecular Pathogenesis
- •Histopathology and Immunohistochemistry
- •Clinical Presentation
- •Investigation/Diagnosis
- •Chest Studies
- •Cardiovascular Imaging
- •CNS Imaging
- •Bone Radiography
- •Other Imaging Findings and Considerations
- •Disease Monitoring
- •Pathology
- •Management/Treatment
- •Prognosis
- •Rosai-Dorfman Destombes Disease
- •Epidemiology
- •Etiology/Pathophysiology
- •Histopathology and Immunohistochemistry
- •Clinical Presentation
- •Investigation/Diagnosis
- •Management/Treatment
- •Prognosis
- •Conclusions
- •Diagnostic Criteria for Primary Histiocytic Disorders of the Lung
- •References
- •17: Eosinophilic Pneumonia
- •Introduction
- •Eosinophil Biology
- •Physiologic and Immunologic Role of Eosinophils
- •Release of Mediators
- •Targeting the Eosinophil Cell Lineage
- •Historical Perspective
- •Clinical Presentation
- •Pathology
- •Diagnosis
- •Eosinophilic Lung Disease of Undetermined Cause
- •Idiopathic Chronic Eosinophilic Pneumonia
- •Clinical Features
- •Imaging
- •Laboratory Studies
- •Bronchoalveolar Lavage
- •Lung Function Tests
- •Treatment
- •Outcome and Perspectives
- •Clinical Features
- •Imaging
- •Laboratory Studies
- •Bronchoalveolar Lavage
- •Lung Function Tests
- •Lung Biopsy
- •Treatment and Prognosis
- •Eosinophilic Granulomatosis with Polyangiitis
- •History and Nomenclature
- •Pathology
- •Clinical Features
- •Imaging
- •Laboratory Studies
- •Pathogenesis
- •Diagnosis
- •Treatment and Prognosis
- •Long-Term Outcome
- •Hypereosinophilic Syndrome
- •Pathogenesis
- •Clinical and Imaging Features
- •Laboratory Studies
- •Treatment and Prognosis
- •Eosinophilic Pneumonias of Parasitic Origin
- •Tropical Eosinophilia [191]
- •Ascaris Pneumonia
- •Eosinophilic Pneumonia in Larva Migrans Syndrome
- •Strongyloides Stercoralis Infection
- •Eosinophilic Pneumonias in Other Infections
- •Allergic Bronchopulmonary Aspergillosis
- •Pathogenesis
- •Diagnostic Criteria
- •Biology
- •Imaging
- •Treatment
- •Bronchocentric Granulomatosis
- •Miscellaneous Lung Diseases with Associated Eosinophilia
- •References
- •Introduction
- •Pulmonary Langerhans’ Cell Histiocytosis
- •Epidemiology
- •Pathogenesis
- •Diagnosis
- •Clinical Features
- •Extrathoracic Lesions
- •Pulmonary Function Tests
- •Chest Radiography
- •High-Resolution Computed Tomography (HRCT)
- •Bronchoscopy and Bronchoalveolar Lavage (BAL)
- •Lung Biopsy
- •Pathology
- •Treatment
- •Course and Prognosis
- •Case Report I
- •Introduction
- •Epidemiology
- •Clinical Features
- •Histopathological Findings
- •Radiologic Findings
- •Prognosis and Therapy
- •Desquamative Interstitial Pneumonia
- •Epidemiologic and Clinical Features
- •Histopathological Findings
- •Radiological Findings
- •Prognosis and Therapy
- •Conclusion
- •References
- •19: Lymphangioleiomyomatosis
- •Introduction
- •Pathogenesis
- •Presentation
- •Prognosis
- •Management
- •General Measures
- •Parenchymal Lung Disease
- •Pleural Disease
- •Renal Angiomyolipoma
- •Abdominopelvic Lymphatic Disease
- •Pregnancy
- •Tuberous Sclerosis
- •Drug Treatment
- •Bronchodilators
- •mTOR Inhibitors
- •Anti-Oestrogen Therapy
- •Experimental Therapies
- •Interventions for Advanced Disease
- •Oxygen Therapy
- •Pulmonary Hypertension
- •References
- •20: Diffuse Cystic Lung Disease
- •Introduction
- •Lymphangioleiomyomatosis
- •Pathogenesis
- •Pathologic and Radiographic Characteristics
- •Diagnostic Approach
- •Pulmonary Langerhans Cell Histiocytosis (PLCH)
- •Pathogenesis
- •Pathological and Radiographic Characteristics
- •Diagnostic Approach
- •Birt-Hogg-Dubé Syndrome (BHD)
- •Pathogenesis
- •Pathological and Radiographic Characteristics
- •Diagnostic Approach
- •Lymphoproliferative Disorders
- •Pathogenesis
- •Pathological and Radiographic Characteristics
- •Diagnostic Approach
- •Amyloidosis
- •Light Chain Deposition Disease (LCDD)
- •Conclusion
- •References
- •Introduction
- •Lymphatic Development
- •Clinical Presentation of Lymphatic Disorders
- •Approaches to Diagnosis and Management of Congenital Lymphatic Anomalies
- •Generalized Lymphatic Anomaly
- •Etiopathogenesis
- •Clinical Presentation and Diagnosis
- •Course/Prognosis
- •Management
- •Kaposiform Lymphangiomatosis
- •Etiopathogenesis
- •Clinical Presentation and Diagnosis
- •Management
- •Course/Prognosis
- •Gorham Stout Disease
- •Etiopathogenesis
- •Clinical Presentation and Diagnosis
- •Management
- •Course/Prognosis
- •Channel-Type LM/Central Conducting LM
- •Etiopathogenesis
- •Clinical Presentation and Diagnosis
- •Management
- •Course/Prognosis
- •Yellow Nail Syndrome
- •Etiopathogenesis
- •Clinical Presentation and Diagnosis
- •Management
- •Course/Prognosis
- •Summary
- •References
- •Introduction
- •Historical Note
- •Epidemiology
- •Pathogenesis
- •Surfactant Homeostasis in PAP
- •GM-CSF Signaling Disruption
- •Myeloid Cell Dysfunction
- •GM-CSF Autoantibodies
- •Lymphocytosis
- •Clinical Manifestations
- •Clinical Presentation
- •Secondary Infections
- •Pulmonary Fibrosis
- •Diagnosis
- •Pulmonary Function Testing
- •Radiographic Assessment
- •Bronchoscopy and Bronchoalveolar Lavage
- •Laboratory Studies and Biomarkers
- •GM-CSF Autoantibodies
- •Genetic Testing
- •Lung Pathology
- •Diagnostic Approach to the Patient with PAP
- •Natural History and Prognosis
- •Treatment
- •Whole-Lung Lavage
- •Subcutaneous GM-CSF
- •Inhaled GM-CSF
- •Other Approaches
- •Conclusions and Future Directions
- •References
- •Introduction
- •Epidemiology
- •Gastric Contents
- •Pathobiology of GER/Microaspirate in the Lungs of Patients with IPF
- •GER and the Microbiome
- •Diagnosis
- •Clinical History/Physical Exam
- •Investigations
- •Esophageal Physiology
- •Upper Esophageal Sphincter
- •Esophagus and Peristalsis
- •Lower Esophageal Sphincter and Diaphragm
- •Esophageal pH and Impedance Testing
- •High Resolution Esophageal Manometry
- •Esophagram/Barium Swallow
- •Bronchoalveolar Lavage/Sputum: Biomarkers
- •Treatment
- •Anti-Acid Therapy (PPI/H2 Blocker)
- •GER and Acute Exacerbations of IPF
- •Suggested Approach
- •Summary and Future Directions
- •References
- •Introduction
- •Familial Interstitial Pneumonia
- •Telomere Related Genes
- •Genetic
- •Telomere Length
- •Pulmonary Involvement
- •Interstitial Lung Disease
- •Other Lung Disease
- •Hepatopulmonary Syndrome
- •Emphysema
- •Extrapulmonary Manifestations
- •Mucocutaneous Involvement
- •Hematological Involvement
- •Liver Involvement
- •Other Manifestations
- •Treatment
- •Telomerase Complex Agonists
- •Lung Transplantation
- •Surfactant Pathway
- •Surfactant Protein Genes
- •Pulmonary Involvement
- •Treatment
- •Heritable Forms of Pulmonary Fibrosis with Autoimmune Features
- •TMEM173
- •COPA
- •Pulmonary Alveolar Proteinosis
- •GMCSF Receptor Mutations
- •GATA2
- •MARS
- •Lysinuric Protein Intolerance
- •Lysosomal Diseases
- •Hermansky-Pudlak Syndrome
- •Lysosomal Storage Disorders
- •FAM111B, NDUFAF6, PEPD
- •Conclusion
- •References
- •Introduction
- •Pathophysiology
- •Clinical Presentation
- •Epidemiology
- •Genetic Causes of Bronchiectasis
- •Disorders of Mucociliary Clearance
- •Cystic Fibrosis
- •Primary Ciliary Dyskinesia
- •Other Ciliopathies
- •X-Linked Agammaglobulinemia
- •Chronic Granulomatous Disease and Other Disorders of Neutrophil Function
- •Other Genetic Disorders Predisposing to Bronchiectasis
- •Idiopathic Bronchiectasis
- •Diagnosis of Bronchiectasis
- •Management of Patients with Bronchiectasis
- •Airway Clearance Therapy (ACT)
- •Management of Infections
- •Immune Therapy
- •Surgery
- •Novel Therapies for Managing Cystic Fibrosis
- •Summary
- •References
- •Pulmonary Arteriovenous Malformations
- •Background Pulmonary AVMs
- •Anatomy Pulmonary AVMs
- •Clinical Presentation of Pulmonary AVMs
- •Screening Pulmonary AVMs
- •Treatment Pulmonary AVMs
- •Children with Hereditary Hemorrhagic Telangiectasia
- •Pulmonary Hypertension
- •Pulmonary Hypertension Secondary to Liver Vascular Malformations
- •Pulmonary Arterial Hypertension
- •Background HHT
- •Pathogenesis
- •References
- •27: Pulmonary Alveolar Microlithiasis
- •Introduction
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Diagnosis
- •Management
- •Summary
- •References
- •Introduction
- •Hermansky-Pudlak Syndrome
- •Telomerase-Associated Pulmonary Fibrosis
- •Lysosomal Storage Diseases
- •Lysinuric Protein Intolerance
- •Familial Hypocalciuric Hypercalcemia
- •Surfactant Dysfunction Disorders
- •Concluding Remarks
- •References
- •Introduction
- •Background
- •Image Acquisition
- •Key Features of Fibrosis
- •Ancillary Features of Fibrosis
- •Other Imaging Findings in FLD
- •Probable UIP-IPF
- •Indeterminate
- •Alternative Diagnosis
- •UIP in Other Fibrosing Lung Diseases
- •Pleuroparenchymal Fibroelastosis (PPFE)
- •Combined Pulmonary Fibrosis and Emphysema
- •Chronic Hypersensitivity Pneumonitis
- •Other Fibrosing Lung Diseases
- •Fibrosing Sarcoidosis
- •CTD-ILD and Drug-Induced FLD
- •Complications
- •Prognosis
- •Computer Analysis of CT Imaging
- •The Progressive Fibrotic Phenotype
- •Other Imaging Techniques
- •Conclusion
- •References
- •Introduction
- •Bronchoalveolar Lavage (BAL)
- •Technique
- •Interpretation
- •Transbronchial Biopsy (TBB)
- •Transbronchial Lung Cryobiopsy (TLCB)
- •References
- •Introduction
- •Overview of ILD Diagnosis
- •Clinical Assessment
- •Radiological Assessment
- •Laboratory Assessment
- •Integration of Individual Features
- •Multidisciplinary Discussion
- •Diagnostic Ontology
- •Conclusions
- •References
- •Introduction
- •Idiopathic Pulmonary Fibrosis
- •Chronic Hypersensitivity Pneumonitis
- •Connective Tissue Disease
- •Drug-Induced Lung Diseases
- •Radiation Pneumonitis
- •Asbestosis
- •Hermansky-Pudlak Syndrome
- •Risk Factors for Progression
- •Diagnosis
- •Pharmacological Management
- •Conclusions
- •References
- •Historical Perspective
- •Epidemiology and Etiologies
- •Tobacco Smoking and Male Sex
- •Genetic Predisposition
- •Systemic Diseases
- •Other Etiological Contexts
- •Clinical Manifestations
- •Pulmonary Function and Physiology
- •Imaging
- •Computed Tomography Characteristics and Patterns
- •Thick-Walled Large Cysts
- •Imaging Phenotypes
- •Pitfalls
- •Pathology
- •Diagnosis
- •CPFE Is a Syndrome
- •Biology
- •Complications and Outcome
- •Mortality
- •Pulmonary Hypertension
- •Lung Cancer
- •Acute Exacerbation of Pulmonary Fibrosis
- •Other Comorbidities and Complications
- •Management
- •General Measures and Treatment of Emphysema
- •Treatment of Pulmonary Fibrosis
- •Management of Pulmonary Hypertension
- •References
- •Acute Interstitial Pneumonia (AIP)
- •Epidemiology
- •Presentation
- •Diagnostic Evaluation
- •Radiology
- •Histopathology
- •Clinical Course
- •Treatment
- •Epidemiology
- •Presentation
- •Diagnostic Evaluation
- •Radiology
- •Histopathology
- •Clinical Course
- •Desquamative Interstitial Pneumonia (DIP)
- •Presentation
- •Diagnostic Evaluation
- •Radiology
- •Histopathology
- •Clinical Course
- •Treatment
- •Epidemiology
- •Presentation
- •Diagnostic Evaluation
- •Radiology
- •Histopathology
- •Clinical Course
- •Treatment
- •References
- •Organizing Pneumonias
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Imaging
- •Multifocal Form
- •Isolated Nodular Form
- •Other Imaging Patterns
- •Histopathological Diagnosis of OP Pattern
- •Etiological Diagnosis of OP
- •Treatment
- •Clinical Course and Outcome
- •Severe Forms of OP with Respiratory Failure
- •Acute Fibrinous and Organizing Pneumonia
- •Granulomatous Organizing Pneumonia
- •Acute Interstitial Pneumonia
- •Epidemiology
- •Clinical Picture
- •Imaging
- •Histopathology
- •Diagnosis
- •Treatment
- •Outcome
- •References
- •36: Pleuroparenchymal Fibroelastosis
- •Introduction
- •Epidemiology
- •Clinical Manifestations
- •Laboratory Findings
- •Respiratory Function
- •Radiologic Features
- •Pathologic Features
- •Diagnosis
- •Treatment
- •Prognosis
- •Conclusions
- •References
- •Introduction
- •Acute Berylliosis
- •Chronic Beryllium Disease
- •Exposure
- •Epidemiology
- •Immunopathogenesis and Pathology
- •Genetics
- •Clinical Description and Natural History
- •Treatment and Monitoring
- •Indium–Tin Oxide-Lung Disease
- •Hard Metal Lung
- •Flock Worker’s Disease
- •Asbestosis
- •Nanoparticle Induced ILD
- •Flavoring-Induced Lung Disease
- •Silica-Induced Interstitial Lung Disease
- •Chronic Silicosis
- •Acute and Accelerated Silicosis
- •Chronic Obstructive Disease in CMDLD
- •Simple CMDLD
- •Complicated CMDLD
- •Conclusion
- •References
- •38: Unclassifiable Interstitial Lung Disease
- •Introduction
- •Diagnostic Scenarios
- •Epidemiology
- •Clinical Presentation
- •Diagnosis
- •Clinical Features
- •Radiology
- •Laboratory Investigations
- •Pathology
- •Conclusion
- •References
- •39: Lymphoproliferative Lung Disorders
- •Introduction
- •Nodular Lymphoid Hyperplasia
- •Lymphocytic Interstitial Pneumonia (LIP)
- •Follicular Bronchitis/Bronchiolitis
- •Castleman Disease
- •Primary Pulmonary Lymphomas
- •Primary Pulmonary MALT B Cell Lymphoma
- •Pulmonary Plasmacytoma
- •Follicular Lymphoma
- •Lymphomatoid Granulomatosis
- •Primary Pulmonary Hodgkin Lymphoma (PPHL)
- •Treatment
- •References
- •Introduction
- •Late-Onset Pulmonary Complications
- •Bronchiolitis Obliterans (BO)
- •Pathophysiology
- •Diagnosis
- •Management of BOS
- •Post-HSCT Organizing Pneumonia
- •Other Late-Onset NonInfectious Pulmonary Complications (LONIPCs)
- •Conclusion
- •References
- •Introduction
- •Pulmonary Hypertension Associated with Sarcoidosis (Group 5.2)
- •PH Associated with Pulmonary Langerhans Cell Histiocytosis (Group 5.2)
- •PH in Combined Pulmonary Fibrosis and Emphysema (Group 3.3)
- •PH Associated with Lymphangioleiomyomatosis (Group 3)
- •Hereditary Hemorrhagic Telangiectasia (Group 1.2)
- •Pulmonary Veno-Occlusive Disease (Group 1.5)
- •Small Patella Syndrome (Group 1.2)
- •Conclusion
- •References
- •Introduction
- •Epidemiology
- •Timing, Chronology, Delay Time
- •Route of Administration
- •Patterns of Involvement [3, 4]
- •Drugs and Agents Fallen Out of Favor
- •Drug-Induced Noncardiac Pulmonary Edema
- •Drug-Induced Cardiogenic Pulmonary Edema
- •The “Chemotherapy Lung”
- •Drug-Induced/Iatrogenic Alveolar Hemorrhage
- •Drugs
- •Superwarfarin Rodenticides
- •Transfusion Reactions: TACO–TRALI
- •Acute Eosinophilic Pneumonia
- •Acute Granulomatous Interstitial Lung Disease
- •Acute Organizing Pneumonia (OP), Bronchiolitis Obliterans Organizing Pneumonia (BOOP), or Acute Fibrinous Organizing Pneumonia (AFOP) Patterns
- •Acute Amiodarone-Induced Pulmonary Toxicity (AIPT)
- •Accelerated Pulmonary Fibrosis
- •Acute Exacerbation of Previously Known (Idiopathic) Pulmonary Fibrosis
- •Anaphylaxis
- •Acute Vasculopathy
- •Drug-Induced/Iatrogenic Airway Emergencies
- •Airway Obstruction as a Manifestation of Anaphylaxis
- •Drug-Induced Angioedema
- •Hematoma Around the Upper Airway
- •The “Pill Aspiration Syndrome”
- •Catastrophic Drug-Induced Bronchospasm
- •Peri-operative Emergencies (Table 42.8)
- •Other Rare Presentations
- •Pulmonary Nodules and Masses
- •Pleuroparenchymal Fibroelastosis
- •Late Radiation-Induced Injury
- •Chest Pain
- •Rebound Phenomenon
- •Recall Pneumonitis
- •Thoracic Bezoars: Gossipybomas
- •Respiratory Diseases Considered Idiopathic That May Be Drug-Induced (Table 42.4)
- •Eye Catchers
- •Conclusion
- •References
- •Cancer Mimics of Organizing Pneumonia
- •Lung Adenocarcinoma/Bronchioloalveolar Carcinoma
- •Primary Pulmonary Lymphoma
- •Cancer Mimics of Interstitial Lung Diseases
- •Lymphangitic Carcinomatosis
- •Epithelioid Hemangio-Endothelioma
- •Lymphomatoid Granulomatosis
- •Cystic Tumors
- •Cavitating Tumors
- •Intrathoracic Pseudotumors
- •Respiratory Papillomatosis
- •Pulmonary Langerhans Cell Histiocytosis
- •References
- •Index
36 Pleuroparenchymal Fibroelastosis |
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Pathologic Features
Originally, Frankel et al. demonstrated markedly dense brosis of the pleura and subpleural parenchyma in PPFE [1]. Fibrosis of the subpleural parenchyma is characterized by intra-alveolar brosis with prominent deposition of elasticbers ( broelastosis) (Fig. 36.2) (Table 36.4). An abrupt border between the brotic parenchymal areas and adjacent normal parenchyma is noted. A few broblastic foci at the interface of the brotic lesions and mild lymphocytic interstitial infammation are also present [31]. In addition, bronchocentric intra-alveolar brosis is occasionally seen [3]. Fibrotic parenchymal lesions in PPFE are very similar to those of the pulmonary apical cap, and distinguishing between both has been histologically dif cult. As described previously, although patients with PPFE often have the lower-lobe ILD, a few studies have focused on histologicndings of lower-lobe ILD in PPFE [27, 53, 54].Accordingly, Nunes et al. reported that among four biopsy-con rmed patients with PPFE who had lower-lobe ILD, three exhibited a UIP pattern in the lower lobes, suggesting that a UIP pattern is a common histologic nding in lower-lobe ILD of PPFE [27].
a
One study showed that patients with PPFE have more lymphatic vessels in the lung of compared to those with an apical cap and IPF [55]. Notably, Enomoto et al. tested immunostaining markers that could distinguish PPFE from IPF or apical cap and found that podoplanin-positive myo - broblasts could be a pathologic hallmark of PPFE [56]. They hypothesized that “pleural” mesothelial-to-mesenchymal transition is associated with the brosis in PPFE given that podoplanin is usually expressed in mesothelial cells but not myo broblasts.
Recent studies have shown that histologic PPFE patterns can also be found in other ILDs, such as IPF [17, 18, 52, 54, 57, 58]. Indeed, Oda et al. observed a histologic PPFE pattern in 9 (8.2%) of 110 consecutive patients with biopsy-
Table 36.4 Pathologic ndings of pleuroparenchymal broelastosis
• Dense subpleural intra-alveolar brosis with elastic ber deposition (sharp transition between brotic lesions and the normal lung)
• Fibrous visceral pleural thickening (apical portions or upper lobes)
• Mild lymphocytic infammation
• Fibroblastic foci (rare, at the brotic lesion interface)
b
Fig. 36.2 (a) Surgical lung biopsy specimens from a patient with idiopathic pleuroparenchymal broelastosis (iPPFE). A lung section of the left upper lobe stained with hematoxylin and eosin showing subpleuralbrosis with an abrupt transition to normal lung parenchyma and broblastic foci. (b) A lung specimen with Elastica van Gieson (EVG) stain-
ing demonstrating depositions of dense elastic bers (elastosis) and intra-alveolar collagen bers in the subpleural brotic lung lesion (b). These features of PPFE on EVG staining are different from those of UIP, which show architectural destruction of normal alveoli. Pleuralbrosis is also observed
632 |
T. Suda |
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con rmed IPF [17]. In addition, Kinoshita et al. reported that 11 (22.9%) of 48 patients with biopsy-con rmed IPF exhibited a histologic PPFE pattern [58]. However, the extent of the subpleural parenchymal broelastosis in patients with IPF was much smaller than that in patients with PPFE. Moreover, histologic PPFE pattern was identi ed in 50% of 24 patients with CTD-associated ILD [52]. These observations suggest that the histologic PPFE patterns may indicate chronic lung injury, similar to UIP pattern, and are focally observed in association with a variety of conditions [54].
with identi able etiologies [5]. More recently, the Study Group on Diffuse Pulmonary Disorders in Japan has proposed a more comprehensive criteria for iPPFE, which consist of the following four categories: “de nite iPPFE,” “radiologically and physiologically probable iPPFE,” “radiologically probable iPPFE,” and “radiologically possible iPPFE” (Box 36.1 and Table 36.5) [61]. “De nite iPPFE” requires surgical lung biopsy, whereas “radiologically and physiologically probable iPPFE,” “radiologically probable iPPFE,” or “radiologically possible iPPFE” do not. Currently, validation studies of the aforementioned criteria are being undertaken.
Diagnosis
Although several radiological and histological criteria for PPFE or PPFE pattern have been proposed [3, 31], no consensus regarding the diagnosis of iPPFE has yet been established. A de nitive diagnosis of iPPFE ideally requires histologic con rmation of PPFE features following surgical lung biopsy. However, surgical lung biopsy is usually not feasible considering that patients with iPPFE, especially those with advanced diseases, have severe pulmonary function impairment and often develop persistent post-operative pneumothorax. Recent studies have demonstrated the diagnostic utility and safety of transbronchial lung biopsy (TBLB) and transbronchial lung cryobiopsy (TBLC) for iPPFE [59, 60]. However, given that these studies included only a small number of patients, a larger cohort of patients with iPPFE is needed to validate their ndings. Therefore, clinical criteria without surgical lung biopsy have been desired for the diagnosis of iPPFE. Enomoto et al. recently proposed the following clinical criteria for iPPFE: (1) a radiologic PPFE pattern on chest CT (de ned as bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes, (2) radiologic con rmation of disease progression, and (3) exclusion of other lung diseases
Box 36.1 Diagnostic Criteria for Idiopathic Pleuroparenchymal Fibroelastosis (iPPFE) Proposed by the Study Group on Difuse Pulmonary Disorders in Japan [62]
•\ Defnite iPPFE (with surgical lung biopsy)
\1.\ Multiple subpleural foci of airspace consolidation with traction bronchiectasis located predominantly in the bilateral upper lobes on high-resolution computed tomography (HRCT) scans.
\2.\ Subpleural zonal or wedge-shaped dense brosis consisting of collapsed alveoli and collagen-lled alveoli with septal elastosis, with or without collagenous thickening of visceral pleura in surgical lung biopsy specimens.
\3.\ Exclusion of other diseases with known causes or conditions showing radiological and/or histological PPFE patterns, such as chronic hypersensitivity pneumonia, connective tissue diseases, occupational diseases, and hematopoietic stem cell or lung transplantation-related lung diseases.
Table 36.5 Summary of the diagnostic criteria for idiopathic pleuroparenchymal broelastosis [61]
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Radiology |
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|
Category of criteriaa |
Symptoms |
Histology |
1 |
2 |
Physiology |
De nite iPPFE |
|
○ |
○ |
|
|
Radiologically and physiologically probable iPPFE |
○ |
|
○ |
○ |
○ |
Radiologically probable iPPFE |
○ |
|
○ |
○ |
|
Radiologically possible iPPFE |
|
|
○ |
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|
Symptoms: dry cough or exertional dyspnea with insidious onset
Histology: subpleural zonal or wedge-shaped dense brosis consisting of collapsed alveoli and collagenlled alveoli with septal elastosis Radiology 1: subpleural airspace consolidation with traction bronchiectasis in the upper lobes
Radiology 2: bilateral upward shift of hilar structures and/or volume loss in the upper lobes. Physiology: “RV/TLC% pred. ≥115%” and/or “BMI ≤ 20 plus RV/TLC% pred. ≥80%”
a All categories need exclusion of other diseases with known causes or conditions showing radiological and/or histological PPFE patterns, such as chronic hypersensitivity pneumonitis, connective tissue diseases, occupational diseases, and hematopoietic stem cell or lung transplantation- related lung diseases
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36 Pleuroparenchymal Fibroelastosis |
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If all the above three criteria are met, de nite iPPFE is diagnosed. If the lower lobes are involved by brosis, multidisciplinary discussion is necessary for thenal diagnosis.
•\ Radiologically and physiologically probable iPPFE (without surgical lung biopsy)
\1.\ Dry cough or exertional dyspnea with insidious onset.
\2.\ Multiple subpleural foci of airspace consolidation with traction bronchiectasis located predominantly in the bilateral upper lobes on HRCT scans.
\3.\ Upward shift of the bilateral hilar structures on chest radiographs and/or volume loss of the upper lobes on HRCT scans.
\4.\ Exclusion of other diseases with known causes or conditions showing radiological and/or histological PPFE patterns, such as chronic hypersensitivity pneumonia, connective tissue diseases, occupational diseases, and hematopoietic stem cell or lung transplantation-related lung diseases.
\5.\ Percentage of predicted values of the ratio between residual volume and total lung capacity (RV/TLC %pred.) ≥ 115%.
\6.\ Body mass index ≤20 kg/m [2] and RV/TLC %pred. ≥ 80%.
If criteria 1, 2, 3, 4, and 5 or 6 are satis ed, radiologically and physiologically probable iPPFE is diagnosed.
•\ Radiologically probable iPPFE (without surgical lung biopsy)
\1.\ Dry cough or exertional dyspnea with insidious onset.
\2.\ Multiple subpleural foci of airspace consolidation with traction bronchiectasis located predominantly in the bilateral upper lobes on HRCT scans.
\3.\ Upward shift of the bilateral hilar structures on chest radiographs and/or volume loss of the upper lobes on HRCT scans.
\4.\ Exclusion of other diseases with known causes or conditions showing radiological and/or histological PPFE patterns, such as chronic hypersensitivity pneumonia, connective diseases, occupational diseases, and hematopoietic stem cell or lung transplantation-related lung diseases.
If all aforementioned criteria are satis ed, radiologically probable iPPFE is diagnosed.
•\ Radiologically possible iPPFE (without surgical lung biopsy).
\1.\ Multiple subpleural foci of airspace consolidation with traction bronchiectasis located predominantly in the bilateral upper lobes on HRCT scans.
\2.\ Exclusion of other diseases with known causes or conditions showing radiological and/or histological PPFE patterns, such as chronic hypersensitivity pneumonia, connective diseases, occupational diseases, and hematopoietic stem cell or lung transplantation-related lung diseases.
If both criteria are satis ed, radiologically possible iPPFE is diagnosed. Radiologically possible IPPFE includes an apical cap with neither symptoms nor long-term progression in addition to the early and localized stages of iPPFE.
Differential diagnoses include a variety of diseases with upper lobe pleural thickening and/or brosis (Table 36.6), with pulmonary apical cap being one of the dif cult conditions to differentiate. Both radiologic and histologic features of the apical cap are very similar to those of iPPFE. However, the apical cap usually occurs in older males with a history of smoking, whereas iPPFE affects relatively younger non- smokers with no gender predisposition. Moreover, the apical cap commonly shows a localized upper lobe lesion, whereas iPPFE, despite having upper lobe predominance, often exhibits a more extended distribution beyond the upper lobes. Most importantly, patients with an apical cap usually do not show disease progression over time, unlike those with iPPFE. Other differential diagnoses include chronic hypersensitivity pneumonitis and radiation-induced pneumonitis. Furthermore, to diagnose iPPFE, secondary PPFE, such as drug-induced and post-transplant PPFE (Table 36.1), must be excluded.
Another dif culty encountered when diagnosing iPPFE is distinguishing between this disease and other types of ILDs
Table 36.6 Differential diagnoses of idiopathic pleuroparenchymalbroelastosis
• Pulmonary apical cap
• Chronic hypersensitivity pneumonitis
•Advanced brosing sarcoidosis
•Radiation-induced pneumonitis
• Other ILDs (e.g., IIPs) with PPFE-like lesions in the upper lobes
• Secondary PPFE (see Table 36.1)
PPFE pleuroparenchymal broelastosis, ILD interstitial lung disease, IIPs idiopathic interstitial pneumonias
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with radiologic PPFE-like lesions or a histologic PPFE pattern in the upper lobes given that certain patients with other ILDs, such as IPF and CTD-ILD, show radiologic PPFE-like lesions or a histologic PPFE pattern [17–19, 51, 52, 55, 58, 59]. In addition, a majority of patients with iPPFE often have lower-lobe ILD [3, 5–9, 24]. Thus, for patients with both upperand lower-lobe brosis, determining upper lobe predominance, which is essential for the diagnosis of iPPFE, is occasionally challenging. Under such circumstances, MDD is necessary.
Treatment
To date, no effective treatments for iPPFE or secondary PPFE have been established.
Corticosteroids, immunosuppressants, or N-acetyl cysteine have been shown to achieve no improvement or, if any, transient response [48, 62, 63]. Nonetheless, very preliminary observations have suggested that antibrotic agents, pirfenidone and nintedanib, might have some bene t [64, 65]. Sato et al. reported that pirfenidone treatment was followed by FVC stabilization in a patient with iPPFE [64]. Moreover, Nasser et al. showed that nintedanib treatment inve patients with PPFE, among whom three had iPPFE and two had PPFE secondary to chemotherapy, was followed by reduced FVC decline in all patients [65]. However, given that these studies included only a small number of patients, prospective studies including a larger cohort are needed to con rm their ndings. Although several reports have recently demonstrated that lung transplantation may be an effective treatment option for iPPFE and secondary PPFE [62, 66–71], evidence has still been limited. Owing to reports of recurrent infections among patients with PPFE, such as aspergillosis and non-tuberculosis mycobacterial infection [3], infection control should be taken into consideration in such cases.
Prognosis
The prognosis of PPFE is heterogeneous. Yoshida et al. reported two patterns of disease progression: rapid FVC decline over a short time period and slow decline over a long period [72]. However, clinical differences at the baseline had not been described in detail between the two patterns. They speculated that, in patients with PPFE, FVC decline gradually to a certain point in time, after which FVC begins to drop rapidly. To date, several studies have performed survival analysis among patients with PPFE [5, 8, 9, 25, 72, 73], subsequently revealing 5-year survival rates and median survival durations ranging from 29% to 58% and 2.0–8.0 years, respectively, with wide variability (Table 36.7). Although a
few studies have directly compared the prognosis between PPFE and other ILDs, such as IPF, Fujisawa et al. reported that patients with iPPFE had signi cantly shorter survival than those with IPF [25]. Collectively, these observations suggest that PPFE seems to have a poor prognosis.
Several prognostic factors have been identi ed. Indeed, Suzuki. et al. identi ed male gender and low erector spinae muscle attenuation (ESMMA) determined through CT imaging as independent factors for poor prognosis among patients with iPPFE [73]. Similarly, Khiroya et al. found that male sex was a predictor of increased risk of mortality among patients with PPFE [53]. Histologically, the coexistence of granulomas was associated with a signi cant decrease in mortality. Moreover, multivariate analysis by Kono et al. and Kato et al. identi ed low %FVC and high brosis score assessed through HRCT as factors for poor prognosis in iPPFE, respectively [8, 9]. Recently, Ishii et al. reported that serum KL-6 levels were signi cantly associated with outcomes in 52 patients with PPFE (48 iPPFE, 4 secondary PPFE) such that patients with KL-6 levels >600 U/mL showed signi cantly shorter survival than those with KL-6 levels <600 U/mL [7]. Given that patients with PPFE usually have normal upper limits or slightly higher serum KL-6 levels, the increase in KL-6 levels might have been attributed to the coexistence of lower-lobe ILDs. This suggest that patients with PPFE who develop lower-lobe ILDs may have poor prognosis. Similarly, Kono et al. recently demonstrated that patients with iPPFE who had lower-lobe ILD exhibited signi cantly worse survival with higher serum KL-6 levels than those without the lower-lobe ILD [8]. More importantly, patients with a lower-lobe UIP pattern had signi cantly shorter survival than those with a lower-lobe non-UIP pattern, suggesting that a radiologic UIP pattern in the lower- lobe ILD is an important prognostic determinant. Accordingly, Kato et al. also described that a lower-lobe UIP pattern was an independent factor for poor prognosis among patients with iPPFE [9]. Moreover, patients with iPPFE who had a lower-lobe UIP pattern tended to exhibit poorer prognosis than those with IPF [17].
During the course of PPFE, two particular conditions should be considered: pneumothorax and acute exacerbation. Recurrent pneumothorax often occurs especially in advanced diseases with multiple bullae, with pneumothorax incidence rates ranging from 17% to 75% [3, 5, 6, 8, 9, 23, 72]. The pneumothorax is often intractable and occasionally accompanied by pneumomediastinum. Recently, it has become evident that patients with PPFE develop acute exacerbation, as seen in those with IPF [7, 17, 24, 73, 74]. Suzuki et al. reported that acute exacerbation occurred in 8 (18.6%) of 43 patients with iPPFE over a median observation period of 31.1 months [73], while Ishi et al. found acute exacerbation in 7 (13%) of 52 patients with PPFE [7]. Outcomes fol-
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Table 36.7 Survival analysis of patients with pleuroparenchymal broelastosis
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Case number |
|
|
|
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|
Author |
Year |
(idiopathic/secondary) |
Age, years |
FVC, % |
RV/TLC, % |
DLCO, % |
5-year survival rate, % |
Median survival time, years |
|
|
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|
Nakatani, alet. [23] |
2015 |
12 |
62* |
70.6 |
|
|
|
2.3 |
|
|
(8/4) |
(27–70) |
(53.8–108.6) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Yoshida, alet. [72] |
2016 |
22 |
56.11±6.0† |
66.20±0.1 |
46.12±4.9 |
72.23±5.5 |
|
7.3 |
|
|
(20/2) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Enomoto, alet. [5] |
2017 |
44 |
70 |
54 |
55 |
68 |
28.9 |
2.9 |
|
|
(44/0) |
(65–75) |
(46–67) |
(45–91) |
(45–91) |
|
|
|
|
|
|
|
|
|
|
|
Ishii, alet. [7] |
2018 |
52 |
62.5 |
66.8 |
42.7 |
73.2 |
58 |
8.0 |
|
|
(48/4) |
[14.5]‡ |
[21.3] |
[9.3] |
[40.2] |
|
|
Suzuki, alet. [7] |
2018 |
43 |
69.0 |
54.4 |
|
68.7 |
29.8§ |
2.9§ |
|
|
(43/0) |
(64.0–74.0) |
(45.8–65.7) |
|
(47.9–91.9) |
|
|
|
|
|
|
|
|
|
|
|
Kato, alet. [9] |
2019 |
36 |
74 |
62.5 |
|
37.5 |
|
2.0 |
|
|
(36/0) |
(35–84) |
(38.3–98.3) |
|
(7.7–67.0) |
|
|
|
|
|
|
|
|
|
|
|
Kono, alet. [8] |
2019 |
40 |
65.22±6.1 |
65.22±6.1 |
45.8±1.9 |
99.25±1.1 |
57.7§ |
6.7§ |
|
|
(40/0) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Fujisawa, alet. [25] |
2019 |
18 |
68.5 |
67 |
|
76.0 |
35.0§ |
2.9§ |
|
|
(18/0) |
(62.3–72.0) |
(52.8–82.4) |
|
(69.5–96.2) |
|
|
|
|
|
|
|
|
|
|
|
*: median (range), †: standard±mean deviation, ‡: interquartile range, §: unpublished data
FVC force vital capacity, RV/TLC residual volume/total lung capacity, DLCO diffusing capacity for carbon monoxide
Fibroelastosis Pleuroparenchymal 36
635