Index

A

Abdominal lymphatic disease, 343 Abdominopelvic lymphatic disease, 342, 343 Acadian variant of Fanconi syndrome, 431 Accelerated pulmonary brosis, 759 Accelerated silicosis, 656

ACEI-induced upper airway obstruction, 766 Acute amiodarone-associated cellular NSIP, 759

Acute amiodarone-induced pulmonary toxicity (AIPT), 759 Acute berylliosis, 640

Acute cellular nonspeci c interstitial pneumonia pattern, 756, 757 Acute drug-induced granulomatous lung disease, 758

Acute drug-induced/iatrogenic emergencies with diffuse pulmonary in ltrates and ARDS, 747–760

Acute drug-(tocolytic agent in this case) induced pulmonary edema, 741

Acute eosinophilic pneumonia (AEP), 297, 309, 757 and acute respiratory failure, 740

Acute exacerbation of interstitial lung disease (AE-ILD), 154, 155 Acute exacerbations (AE), 210, 410

Acute brinous, 615

Acute brinous organizing pneumonia (AFOP), 525, 614, 615, 758 conditions associated with, 614

histopathological features of, 615 patterns, 758

Acute interstitial pneumonia (AIP), 155, 587, 588, 618 clinical course, 589, 590

clinical picture, 616

de nition and terminology, 615 diagnosis, 617, 618

diagnostic evaluation, 589 epidemiology, 588, 616 histopathology, 589, 616, 617 history and de nition, 588 imaging, 616

outcome, 619 presentation, 588, 589 radiology, 589 treatment, 590, 618, 619

Acute leukemia, 426

Acute lupus pneumonitis, 224

Acute radiation induced lung injury, 761

Acute respiratory distress syndrome (ARDS), 142, 155, 156, 618 Acute severe cellular ILD and pulmonary edema with acute

respiratory failure, 739 Acute vasculopathy, 760, 761 Adenocarcinoma, 428

Adult respiratory distress syndrome (ARDS), 747 Airway-centered interstitial brosis (ACIF), 27, 32, 34 Airway clearance therapy (ACT), 451

Airway disorders, 225 Airway obstruction, 761

Airways disease in RA (RA-AD), see Rheumatoid arthritis-associated airways disease (RA-AD)

Alarcón-Segovia and Villareal criteria, 228 Alkaptonuria (AKU), 16

Allergic aspergillosis, 43 Allergic Aspergillus sinusitis, 45

Allergic bronchopulmonary aspergillosis (ABPA), 294, 449 clinical manifestations

symptoms, 40, 41 diagnostic criteria, 295

allergic Aspergillus sinusitis, 45 Baxter ABPA, 44

cystic brosis foundation, 44 diagnostic recommendations, 44 differential diagnosis, 44, 45 historical, 43

ISHAM, 43

natural history, 45, 46 Rosenberg and Patterson, 43

epidemiology, 39

host characteristics, 40 laboratory evaluation

eosinophil count, 41 histology, 43

pulmonary function testing, 42 radiographic imaging, 41, 42 recombinant antigens, 41

serum antibodies speci c to Aspergillus fumigatus, 41 serum precipitins, 41

skin testing, 41

total serum immunoglobulin E levels, 41 pathophysiology, 39, 40

studies of therapy for, 47–48 treatment, 46, 48

corticosteroids, 48–50 monitoring for, 50 monoclonal antibodies, 50

Allergic bronchopulmonary mycosis (ABPM), 39, 45 Allogeneic hematopoietic stem cell transplantation, 709 Allogeneic HSCT, 705, 708

Alpha-1 antitrypsin (AAT) de ciency, 448 Alveolar capillaritis, 144

Alveolar hemorrhage, 112, 429 Alveolar lymphocytosis, 708 Alveolar surfactant homeostasis, 390

American Thoracic Society clinical practice guideline, 524 American Thoracic Society/European Respiratory Society, 526, 527 Aminorex, 747

Amiodarone, 766 Amiodarone-induced ARDS, 759

Amiodarone-induced thyrotoxicosis, 739 Amiodarone pulmonary toxicity, 766

Amphetamine-like anorectics, 747 Amyloid, 784

Amyloidoma, 784 Amyloidosis, 358, 784 Amyloids, 77

diagnosis and evaluation, 78–80 Hilar amyloid lymphadenopathy, 86 laryngeal, 84

localised respiratory tract, 83, 84 parenchymal pulmonary, 85 Sjöogren’s disease, 86

systemic AA, 80, 81 systemic AL, 81–83 tracheobronchial, 85

Anaphylaxis, 760

Anaplastic large cell lymphoma, 698 Anaplastic lymphoma kinase (ALK), 782 ANCA-associated small vessel vasculitis

granulomatosis with polyangiitis, 144–146 microscopic polyangiitis, 146

ANCA-associated vasculitis (AAV), 144–146, 157 ANCA-related vasculitis, 753

Angiomyolipomas, 335, 336, 340, 341, 353 in TSC-LAM, 342

Angiosarcoma, 780 Angiotensin-converting enzyme (ACE), 326 Anti-acid therapy

gastroesophageal refux, 410, 411

Anti-basement membrane antibody (ABMA), 148, 149 Anti-basement membrane-related AH (Goodpasture’s like), 755 Anti-CD20 therapy with rituximab, 209

Anti-centromere antibody (ACA), 192 Anti brotic therapy for CTD-ILD, 232, 233 Antibrotics, ILD, 244

Antifungal therapies, 46 Antifungal therapy, 297 Antigen-presenting cells, 39 Anti-histidyl-tRNA synthetase, 229

Anti-IL-5 monoclonal antibody, 281 Anti-infammatory management

diffuse bronchiectasis, 452 immune therapy, 452, 453

Anti-Jo1 positive anti-synthetase syndrome, 229

Antineoplastic drugs of multiagent chemotherapy regimens, 752 Anti-neutrophil cytoplasmic antibodies (ANCA), 130, 143, 144 Antinuclear antibodies (ANA), 192

Anti-oestrogen therapy, LAM, 344 Antiphospholipid antibody syndrome, 138, 147 Antiphospholipid syndrome, 224

Anti-Smith (anti-Sm), 222 Anti-TNF agents, 759 Anti-TNFα agents, 166 Anti-TNF-α blockers, 127

Anti-U1-small nuclear ribonucleoprotein particle autoantibodies (anti-U1-snRNP), 227

Arterial deoxygenation, 182–184 Arterial embolization, 169 Arteriovenous malformation, 468 Asbestos, 552

Asbestosis, 651, 652

Ascaris lumbricoides, 293 Ascaris pneumonia, 293

Aspergillus fumigatus, 37, 39 Aspirated drug tablets, 762 Asthma, 29, 39, 298

Ataxia telangiectasia, 448 ATM gene, 448

Autoantibodies, 429

Autoimmune pulmonary alveolar proteinosis, 394 pathogenesis of, 391

Autosomal dominant polycystic kidney disease (ADPKD), 445 Avacopan, 126

granulomatosis with polyangiitis, 126, 127 Azathioprine, 165, 599

Azithromycin, 428, 707

B

BAL fuid (BALF) analysis, 524, 525 Barium swallow, gastroesophageal refux, 409 Behçet syndrome, 150

Behçet’s disease (BD), 150, 166 clinical features, 167 diagnostic criteria, 167 differential diagnosis, 169 epidemiology, 166

imaging studies, 168, 169 laboratory ndings, 168 parenchymal involvement, 168 pathogenesis, 166, 167 pathologic features, 166 prognosis, 170

pulmonary artery aneurysm (see Pulmonary artery aneurysm (PAA))

pulmonary artery thrombosis (PAT), 168 treatment of, 169

Birt–Hogg–Dubé (BHD) syndrome, 13, 356 Bisphosphonate therapy, 321

Black pleura sign, 478

Bladder carcinoma with topical BCG, 758 Bland pulmonary hemorrhage

etiologies coagulopathy, 152

drugs and medications, 151, 152 idiopathic pulmonary hemosiderosis, 151

valvular heart disease and left ventricular dysfunction, 152 histology, 150, 151

Bleomycin, 766 Bleomycin-induced ARDS, 741 Blood eosinophilia, 278 Blood/BAL eosinophils, 757 Bone marrow failure, 426

Bone marrow transplantation, 198

Bone morphogenetic protein (BMP), 468

Bone morphogenetic protein-receptor type II (BMPRII), 720 Bone morphogenic protein 9 (BMP-9), 179

Borderline neoplastic-non neoplastic disorders, 783

amyloid and non-amyloid immunoglobulin deposition disorders, 784, 785

respiratory papillomatosis, 783, 784 BOS diagnosis criteria NIH 2014, 706

Branching multi-antennary bronchial casts, 371 Brief ataxia rating scale (BARS), 314 Brodifacoum, 753

Bronchial artery embolization, 166 Bronchiectasis, 39, 211, 448

diagnostic criteria for, 454 Bronchiolitis, 25

airway-centered interstitial brosis, 27 cellular, 27

chest imaging studies, 30, 31 classi cation, 26

clinical syndromes, 26 connective tissue diseases, 32

diagnostic criteria, 28, 30 diffuse, 27, 28

diffuse panbronchiolitis, 33 drug-induced bronchiolitis, 33 epidemiology, 25

follicular, 27

hematopoietic stem cell transplantation, 33 idiopathic forms of, 32

airway-centered interstitial brosis, 32 cryptogenic adult bronchiolitis, 32

infectious causes of, 32

inhalational lung injury, secondary to, 31 lung biopsy, 31

mineral dusts, 31

organ transplantation, 32, 33 organic dusts, 31 proliferative, 28

pulmonary function testing, 31 respiratory, 27

treatment

airway-centered interstitial brosis, 34 constrictive bronchiolitis, 33, 34 diffuse panbronchiolitis, 34

follicular bronchiolitis, 34 proliferative bronchiolitis, 34

volatile favoring agents, 32 Bronchiolitis obliterans (BO), 25, 705, 706

clinical presentation, 706 diagnosis, 706 FEV1/FVC ratio, 707

handheld spirometer at home, 707 management, 707

natural history, 707 pathophysiology, 705 risk factors, 707 treatments, 707, 708

Bronchiolitis obliterans organizing pneumonia (BOOP), 603, 758 Bronchiolitis obliterans syndrome (BOS), 32, 33, 412, 709 Bronchoalveolar lavage (BAL), 208, 277, 284, 387, 411, 523–526,

550, 594, 699

cell gene expression patterns, 526 cellularity, 196

fuid of sarcoidosis patients, 526 gastroesophageal refux, 409 lymphocytosis, 525

PAP, 393

Bronchocentric granulomatosis, 297 Bronchodilators, LAM, 344 Bronchopulmonary aspergillosis, 296 Bronchoscopy, 57, 59, 62, 138

with BAL and cryobiopsies, 529 with bronchoalveolar lavage, 674

Bronchovascular bundles, 377 Bypass metabolism, 175

C

Calcineurin inhibitors, 612 Calcium, 478

Calmodulin binding transcription activator 1 (CAMTA1), 779 Campylobacter, 411

Carbon monoxide diffusing capacity (DLco), 193 Cardiac biomarkers, 752

Cardiac magnetic resonance imaging (CMR), 80 Cardiotox, 738

Casitas B lineage lymphoma (CBL) gene, 376 Castleman disease, 691, 693

Catastrophic drug-induced bronchospasm, 762–763 Cavitating tumors, 781

CD1a, 319 CD4/CD8 ratio, 525

Cell-mediated mechanisms, 162 Cellular bronchiolitis, 27

Central conducting lymphatic anomalies (CCLA), 379 Centrilobular emphysema, 568

Channel-type LM/central conducting LM, 379 clinical presentation and diagnosis, 379, 381 course/prognosis, 379, 381 etiopathogenesis, 379, 381

management, 379, 381

Yellow nail syndrome, 380, 381 Chenodeoxycholic acid (CD), 404

Chest radiography, pulmonary Langerhans’ cell histiocytosis, 315 Chronic beryllium disease (CBD), 640

clinical description, 643 diagnosis, 643–645 epidemiology, 642 exposure, 641

fock worker`s disease, 649–651 genetics, 642, 643 immunopathogenesis, 642 treatment, 645

Chronic eosinophilic pneumonia (CEP), 530 Chronic granulomatous disease (CGD), 447 Chronic GVHD, 709

Chronic hypersensitivity pneumonitis, 514 Chronic liver cirrhosis, 180

Chronic lung allograft dysfunction (CLAD), 412 Chronic necrotizing aspergillosis, 45

Chronic obstructive pulmonary disease (COPD), 29, 56, 560, 564, 566 Chronic rhinitis, 285

Chronic rhinosinusitis, 299 Chronic silicosis, 655, 656 Churg–Strauss syndrome, 95 Chylomicrons, 368 Chylopericardium, 371 Chyloptysis, 370 Chylothorax, 378

Chylous complications, 337

Chylous pulmonary congestion (CPC), 371 Ciclosporine A, 169

Cigarette smoke, 309, 311 Cirrhosis, 427

Clustered regularly interspaced short palindromic repeats (CRISPR), 453

Coagulopathy, 152

Coal mine dust lung disease, 656, 657 chronic obstructive disease, 657 complicated, 657

dust diffuse brosis, 658, 659 simple, 657

Cocaine, 120, 153, 754 Cocaine inhalation, 153, 154

Cocaine-induced vasculopathy, 122 Cocaine-levamisole toxicity, 754 Colchicine, 169

Combined pulmonary brosis and emphysema (CPFE), 310, 426, 559 biology, 573

clinical manifestations, 563 complications and outcome, 573

comorbidities, 577 lung cancer, 574–576 mortality, 573, 574

pulmonary brosis, acute exacerbation of, 576

Combined pulmonary brosis and emphysema (CPFE) (Cont.) pulmonary hypertension, 574

diagnosis, 571, 572 epidemiology and etiologies

genetic predisposition, 560, 561 systemic diseases, 561, 563 tobacco smoking and male sex, 560

history, 559, 560 HRCT, 562, 568 imaging, 565

computed tomography characteristics and patterns, 565, 566 emphysema extent, quanti cation of, 567

imaging phenotypes, 566 thick-walled large cysts, 566

management, 577

emphysema, measures and treatment of, 577 pulmonary brosis, treatment, 577 pulmonary hypertension, 577

pathology, 569–571 PH in, 724

pulmonary function and physiology, 564, 565 pulmonary hypertension, 717

research de nition, 572

Complex lymphatic anomalies (CLAs), 367

clinical manifestations, diagnosis and treatments, 368 Complex thoracic lymphatic disorders of adults, 367

channel-type LM/central conducting LM, 379 clinical presentation and diagnosis, 379, 381 course/prognosis, 379, 381 etiopathogenesis, 379, 381

management, 379, 381

Yellow nail syndrome, 380, 381

congenital lymphatic anomalies, diagnosis and management of, 373

generalized lymphatic anomaly, 374 clinical presentation and diagnosis, 375 course/prognosis, 375 etiopathogenesis, 374

management, 375 Gorham Stout disease, 378

clinical presentation and diagnosis, 378 course/prognosis, 379 etiopathogenesis, 378

management, 379

Kaposiform lymphangiomatosis, 375, 376 clinical presentation and diagnosis, 377 course/prognosis, 378 etiopathogenesis, 376, 377 management, 377, 378

lymphatic development, 370

lymphatic disorders, clinical presentation of, 370, 371 pulmonary lymphatic system

structure and function, 368–370 Conjunctivitis, 116

Connective tissue disease (CTD), 223, 239

Connective tissue disease-associated ILD (CTD-ILD), 239, 505, 533 Connective tissue diseases (CTDs), 550

anti brotic therapy, 232, 233 bronchiolitis, 32

diagnosis, 221

lung involvement in, 221 lung transplantation, 233

Constrictive bronchiolitis, 33, 34, 226 Contrast-enhanced thoracic MRI, 165

Conventional or digital subtraction angiographies, 168 Corticosteroids, 165, 224, 231

ABPA, 48–50

Cotrimoxazole, granulomatosis with polyangiitis, 127 COVID-19 or infuenza vaccination, 752, 760

Crack lung, 154 Crazy paving, 393

Cricoarytenoid involvement in RA-AD, 212 Crohn disease, 150

Cryobiopsy, 528

Cryptogenic adult bronchiolitis, 32 Cryptogenic brosing alveolitis, 559

Cryptogenic organizing pneumonia (COP), 603, 758 CTLA4-Ig

granulomatosis with polyangiitis, 127

Cyclophosphamide (CYC), 107, 115, 117, 122, 145, 165–166, 197, 227, 231, 599

granulomatosis with polyangiitis, 123 ILD, 244

Cyclosporine, 231 Cylindrical bronchiectasis, 30

Cystic brosis (CF), 39, 439, 441, 442 diffuse bronchiectasis, 443, 453

Cystic Fibrosis Foundation Consensus Criteria, 44

Cystic brosis transmembrane conductance regulator (CFTR) gene, 211, 441

Cystic lung disease, 226, 337, 352, 357 Cystic tumors, 780, 781

D

Deep venous thrombosis, 138 Degranulated eosinophils, 277 DeMeester score, 407 Dendritic cells, 251, 252 Desmosines, 627

Desquamative interstitial pneumonia (DIP), 309, 326, 358, 525 clinical course, 594

diagnostic evaluation, 593 epidemiology, 593 histopathology, 594 history and de nition, 593 presentation, 593 radiology, 593, 594 treatment, 594, 595

Diffuse alveolar damage (DAD), 588, 604, 618 causes of, 617

etiologies

acute exacerbation of interstitial lung disease, 154, 155 acute interstitial pneumonia, 155

acute respiratory distress syndrome, 155, 156 cocaine inhalation, 153, 154

hematopoietic stem cell transplantation, 153 histology, 153

Diffuse alveolar hemorrhage (DAH), 137, 139, 224, 752, 753 clinical presentation, 140, 141

diagnosis, 142, 143 diffuse alveolar damage

acute exacerbation of interstitial lung disease, 154, 155 acute interstitial pneumonia, 155

acute respiratory distress syndrome, 155, 156 cocaine inhalation, 153, 154

hematopoietic stem cell transplantation, 153 histology, 153

generalized approach to diagnosis, 142 lung, histopathologic section of, 150 miscellaneous causes

human immunode ciency virus, 156

pulmonary capillary hemangiomatosis, 156 pulmonary capillaritis

anti-basement membrane antibody disease, 148, 149 antiphospholipid antibody syndrome, 147 granulomatosis with polyangiitis, 144–146 histology, 144

isolated pulmonary capillaritis, 146 lung allograft rejection, 149 microscopic polyangiitis, 146

systemic lupus erythematosus, 146, 147 treatment, 156–158

Diffuse bronchiectasis, 439 airway clearance therapy, 451

anti-infammatory management, 452 clinical presentation, 440, 441

cystic brosis, novel therapies for, 443, 453 diagnosis of, 449, 450

epidemiology, 441 genetic causes of, 441

cystic brosis, 441, 442

disorders of mucociliary clearance, 441 primary ciliary dyskinesia, 442–445

primary immunode ciency disorders, 447, 448 idiopathic bronchiectasis, 449

immune therapy, 452, 453 infections, management of, 451, 452 management of, 450, 451 pathophysiology, 439, 440

surgery, 453

Diffuse cystic lung diseases (DCLDs), 351 Birt-Hogg-Dubé Syndrome, 355

diagnostic approach, 356 pathogenesis, 355, 356

pathological and radiographic characteristics, 356 prognosis and management, 356

characteristic chest CT ndings of, 352 diagnosis of, 360

history and gender predilection of, 351 HRCT, 351 lymphangioleiomyomatosis, 352

diagnostic approach, 353 pathogenesis, 352, 353

pathologic and radiographic characteristics, 353 prognosis and management, 353, 354

lymphoproliferative disorders amyloidosis, 358

light chain deposition disease, 358 lymphocytic interstitial pneumonia/follicular

bronchiolitis, 357, 358

smoking-related diffuse cystic lung disease, 358 presentations, 351

pulmonary langerhans cell histiocytosis, 354 diagnostic approach, 355

pathogenesis, 354

pathological and radiographic characteristics, 354 prognosis and management, 355

Diffuse ecchymotic, 116

Diffuse large B cell lymphoma (DLBCL), 691, 694 Diffuse panbronchiolitis, 27, 28, 33, 34

Diffuse proliferative, 28

Diffusing capacity for carbon monoxide (DLCO), 138, 141, 412 Dilated pulmonary lymphatics, 372

Direct cytotoxic damage, 354

Disease-modifying antirheumatic drugs (DMARDs), 766 DNAH11 gene, 444, 454

Dramatic extrapulmonary accumulation, 185

Dronedarone, 747 Drug causality, 758 Drug overdose, 739

Drug-induced / Iatrogenic ARDS, 749–750 Drug-induced acute trans xing chest pain, 767

Drug-induced allergic or hypersensitivity-related emergencies, 741 Drug-induced alveolar hemorrhage, 741, 753

Drug-induced and iatrogenic cardiac and/or overload pulmonary edema, 752

Drug-induced and iatrogenic respiratory problems, 768 Drug-induced angioedema, 761–762

Drug-induced ARDS, 752 Drug-induced bronchiolitis, 33 Drug-induced chemotherapy lung, 752 Drug-induced EGPA, 757 Drug-induced esophageal erosion, 767

Drug-induced/iatrogenic airway emergencies, 761–763, 766 Drug-induced/iatrogenic respiratory disease

acute ventilatory depression and respiratory failure, 743 adverse effects, 743

adverse iatrogenic and drug reactions, 733 antirheumatics, 747

catastrophic bronchospasm, 743

catastrophic intraand peri-operative, 764–765 check list, 739

chemotherapeutic agents, 743 coronary vasospasm, 743 drug reactions, 739

fash pulmonary edema, 743 histopathology descriptors, 743 hypersensitivity, anaphylaxis, 743 incidence, 742

life-threatening, 748 pathology, 745–746 pulmonary toxins, 743 route of administration, 743

Drug-induced/Iatrogenic ARDS, 749–750 Drug-induced (DI) lung injury, 733 Drug-induced lupus, 755

Drug-induced pulmonary edema, noncardiac type, 751, 752 Drug-induced-pulmonary in ltrates, 756

Drug-induced respiratory disease (DIRD), 733 diagnostic criteria, 744 immunosuppressants, 744

invasive procedures, 744 lung biopsy, 744

Drug-induced respiratory reactions, 739 Drug-related ANCAs, 755

Dry cough, 626

Dust diffuse brosis, 657 Dysfunction of motile cilia, 445

Dyskeratosis congenita (DC), 426, 487 Dyspnea, 28, 130, 137, 138, 186 Dyspneic chronic liver disease, 177

E

EBV infection, 696

Electronic cigarettes (-e-cigarettes), 744 Electronic nicotine delivery systems (ENDS), 744 Embolization, 353

Emphysema, 426, 566

measures and treatment of, 577

Endobronchial radial ultrasonography (EBUS), 699 Endobronchial ultrasound-guided transbronchial needle aspiration

(EBUS-TBNA), 529

Endoglin (ENG), 720

Endoscopic bronchial or trans-bronchial biopsy, 699 Enzyme-linked immunosorbent assay (ELISA), 143 Enzyme replacement therapy (ERT), 491 Eosinophil cell lineage, targeting, 276

Eosinophil differential cell counts, 525 Eosinophil lineage, drugs targeting, 277 Eosinophilia, 41, 275 Eosinophilia-myalgia syndrom, 747 Eosinophilic bronchitis, 298

Eosinophilic granulomatosis with polyangiitis (EGPA), 93, 101, 143, 286

ANCA status, 99

cardiac manifestations, 96 clinical features, 285, 286 clinical manifestations, 94 complementary investigations, 97 diagnosis, 98, 287, 288

diagnostic and classi cation criteria of, 288 differential diagnosis, 98, 289

ENT manifestations, 96 gastrointestinal involvement, 97 genetic predisposition, 93 history and nomenclature, 285 imaging, 286

immune dysregulation, 94 laboratory studies, 286, 287 long-term outcome, 290 maintenance therapy, 100 neurological manifestations, 96 ophthalmological manifestations, 97 pathogenesis, 93, 287

pathology, 285 phenotypes of, 287 prevalence, 94 prognosis, 98, 99

pulmonary manifestations, 95, 96 remission induction, 100

renal manifestations, 97 skin manifestations, 96

treatment and prognosis, 99, 100, 289, 290 triggering factors, 94

Eosinophilic granulomatous with polyangiitis (EGPA), 102, 757 Eosinophilic lung diseases, 275

classi cation of, 278 Eosinophilic pneumonia, 275

determined cause, eosinophilic lung disease of, 293 allergic bronchopulmonary aspergillosis, 294 ascaris pneumonia, 293

biology, 296

bronchocentric granulomatosis, 297 diagnostic criteria, 294, 295

drug, toxic agents, and radiation-induced eosinophilic pneumonias, 297, 298

imaging, 296

larva migrans syndrome, 293 Löffer syndrome, 293, 294 parasitic origin, 293 pathogenesis, 294

strongyloides stercoralis infection, 293 treatment, 297

tropical eosinophilia, 293 eosinophil biology, 275

eosinophil cell lineage, targeting, 276 eosinophil differentiation and recruitment, 275 features of

clinical presentation, 277 diagnosis, 277, 278 historical perspective, 277 pathology, 277

miscellaneous lung diseases with associated eosinophilia, 298 physiologic and immunologic role, 276

release of mediators, 276

undetermined cause, eosinophilic lung disease of, 278 eosinophilic granulomatosis with polyangiitis, 285–290 hypereosinophilic syndrome, 290–292

idiopathic chronic eosinophilic pneumonia, 279–282, 284 idiopathic hypereosinophilic obliterative bronchiolitis, 292

Episcleritis, 116

Epithelial sodium channels (ENaC), 448

Epithelioid hemangio-endothelioma (EHE), 779, 780 Epstein–Barr virus (EBV) infection, 778 Erdheim-Chester disease (ECD), 249, 256, 258

bone radiography, 261 cardiovascular imaging, 260

cellular and molecular pathogenesis, 256, 257 chest studies, 260

clinical presentation, 258–260 diagnosis, 260

disease monitoring, 261 epidemiology, 256 immunohistochemistry, 258 pathology, 262

prognosis, 264 treatment, 262–264

Esophagus, 406

Eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4), 721

European bronchiectasis registry (EMBARC), 449 Exertional dyspnoea, 346

Extracorporeal Life Support Organization (ELSO), 158 Extranodal NK/T cell lymphoma, nasal type, 696 Extrathoracic lesions, 313, 314

Extra-thoracic manifestations (ExRA), 205 Eye catchers, 768

F

Fabry disease (FD), 431, 490, 491 FAM111B, 431–432

Familial hypocalciuric hypercalcemia (FHH), 492 Familial interstitial pneumonia, 422, 423 Familial pulmonary brosis (FPF), 422

Fatal deterioration of previously diagnosed indolent pulmonarybrosis, 760

18F-Fluorodeoxyglucose-positron emission tomography (FDG-PET), 165

Fibreoptic bronchoscopy, 450 Fibrosing organizing pneumonia, 613 Fibrosing sarcoidosis, 515

Fibrotic hypersensitivity pneumonitis (HP), 533 Fibrotic ILD, 537, 542

Five-factor score (FFS), 122 Flavocoxid, 747

Flavoring-induced lung disease, 654, 655

Fluticasone, azithromycin and montelukast (FAM), 707 Foamy macrophages, 391

Follicular bronchiolitis, 27, 34, 357, 690 diagnostic approach, 357 pathogenesis, 357

pathological and radiographic characteristics, 357 prognosis and management, 357, 358

Follicular lymphoma, 693, 694

Forced vital capacity (FVC), 412

Foreign body bronchiolitis and ILD, 763

G

Gas exchange deterioration, 715 Gastroesophageal junction (GEJ), 403, 406 Gastroesophageal refux (GER), 403

anatomy/embryogenesis, 404 de nitions, 403

diagnosis

barium swallow, 409 bronchoalveolar lavage/sputum, 409 clinical history/physical exam, 405

esophageal pH and impedance testing, 406–408 esophagus and peristalsis, 406

high resolution esophageal manometry, 408, 409 lower esophageal sphincter and diaphragm, 406 upper esophageal sphincter, 405, 406

epidemiology, 403 gastric contents, 404 and microbiome, 405

pathobiology of GER/microaspirate, 404, 405 treatment, 409

and acute exacerbations of IPF, 410, 411 anti-acid therapy, 410, 411 laparoscopic anti-refux surgery, 410

lung transplant, refux/GER/microaspiration in, 412 management of, 414

Gastroesophageal refux disease (GERD), 33, 403 Gastro-esophageal regurgitation (GER), 193 Gaucher’s disease (GD), 431, 488, 489 Generalized lymphatic anomaly (GLA), 374

clinical presentation and diagnosis, 375 course/prognosis, 375 etiopathogenesis, 374

management, 375 Genetic testing, PAP, 393 Genital scares, 167

Genome-wide association study (GWAS), 93 Glomerulonephritis, 141

Glucocorticoids, granulomatosis with polyangiitis, 122, 123 GM-CSF autoantibodies, 391–393

GM-CSF receptor, 430

GM-CSF signaling de ciency test, 393 GM-CSF signaling disruption, 389, 390 Golde score, 723

Gonadal toxicity, 101

Goodpasture Syndrome, 143, 148, 149 Gorham Stout disease (GSD), 378

clinical presentation and diagnosis, 378 course/prognosis, 379 etiopathogenesis, 378

management, 379

Graft vs. host disease (GVHD), 704

Graft vs. leukemia/lymphoma effect (GVL), 704 Granuloma formation, 111

Granulomatosis with polyangiitis (GPA), 66, 67, 107 cardiac involvement, 116, 117

clinical features, 67 DAH, 144–146 diagnosis

biology, 119

diagnostic approach, 118, 119 immunology, 119, 120

pathology, 120, 121

radiology, endoscopy, and nonbiological investigations, 120 diagnostic features of, 119

differential diagnosis, 121, 122

disease activity, prognosis and damage scores, 122 epidemiology, 109, 110

eye manifestations, 116 gastrointestinal manifestations, 117

gynecological and obstetric manifestations, 117 history, 107, 109

imaging studies, 67 kidney manifestations, 115

limited/localized vs. severe/diffuse/systemic forms, 118 localized/limited/early systemic GPA, treatment of, 126 lung biopsy in, 121

neurological manifestations

central nervous system manifestations, 115 peripheral nervous system manifestations, 115 spinal cord and cranial nerve involvement, 115

outcomes and prognostic factors relapse, 129, 130

survival and causes of deaths, 128, 129 pathogenesis, 110, 111

pediatric GPA, 118 pulmonary function studies, 67

quality of life, damage and disease burden on, 130 skin and oral mucosal manifestations, 115, 116 symptoms, 111

ear, nose, and throat manifestations, 111, 112 pulmonary manifestations, 112, 115

systemic/severe/generalized forms, induction treatment for cyclophosphamide, 123

glucocorticoids, 122, 123 induction approaches, 124 maintenance therapy, 124, 125 rituximab, 123, 124

treatment, 67, 122

adjuvant measures and prevention, 128 avacopan, 126, 127

cotrimoxazole, 127 CTLA4-Ig, 127

intravenous immunoglobulins, 126 manifestations, 128

plasma exchange, 126

relapsing and refractory GPA, 128 urological manifestations, 115 venous thrombosis, 117

Granulomatous infammatory tumors, 111 Granulomatous interstitial lung disease, 757, 758 Granulomatous organizing pneumonia (GOP), 615 Ground glass opacity (GGO), 517

GVHD prophylaxis, 709

H

Hamman–Rich syndrome, 615 Hard metal lung, 640, 647, 648 Heart damage, 285

Hematological malignancies, pulmonary manifestations, 703–709 Hematoma around the upper airway, 762

Hematopoietic stem cell transplantation (HSCT), 33, 153, 426, 704–709

Hemoptysis, 313 Hemorrhagic cystitis, 101

Hemosiderin-laden macrophages, 148 Henoch-Schönlein purpura, 149

Hepatopulmonary syndrome (HPS), 175, 426 de nition of, 182

epidemiology, 175–176, 182

molecular pathogenesis, 175–176, 182, 183 risk factors, 182

screening and diagnosis, 175–176, 183–185 treatment, 175–176, 185, 186

Herbal therapies, 747

Hereditary hemorrhagic telangiectasia (HHT), 465, 468, 720, 721 Heritable PAH, 723

Hermansky-Pudlak syndrome (HPS), 13, 486, 487, 552, 553 molecular pathogenesis, 183

Hiatal hernia, 406

High-dose corticosteroid therapy, 198

High-resolution computed tomography (HRCT), 191, 194, 199, 208, 504, 547

pulmonary Langerhans’ cell histiocytosis, 315–318 High resolution esophageal manometry, 408, 409 Histiocytes, 251

HLA typing, 168 Hugues-Stovin syndrome, 169

Human immunode ciency virus (HIV), 137, 156 Hyalinising granuloma, 783 Hydroxychloroquine, 428

4-hydroxycoumarin, 753 Hypereosinophilia, 275, 278

Hypereosinophilic obliterative bronchiolitis, 292 Hypereosinophilic syndrome (HES), 98

clinical and imaging features, 291 de nition, 290

laboratory studies, 291 pathogenesis, 290, 291 treatment and prognosis, 292

Hyperinfation, 33

Hyperplasia of lymphoid elements, 683 Hypersensitivity, 39

Hypersensitivity pneumonitis (HP), 29, 424, 509, 549 Hypothyroidism, 428

Hypoxemia, 284, 534

I

Idiopathic acute eosinophilic pneumonia (IAEP), 277, 284 diagnostic criteria for, 282

Idiopathic bronchiectasis, 449

Idiopathic bronchiolocentric interstitial pneumonia, 27

Idiopathic chronic eosinophilic pneumonia (ICEP), 279, 280, 283, 284 bronchoalveolar lavage, 280, 281, 284

clinical features, 279, 282 diagnostic criteria for, 280 differential diagnosis, 281

idiopathic and smoking-related acute eosinophilic pneumonia, 282 imaging, 279, 282

laboratory studies, 280, 282 lung biopsy, 284

lung function tests, 281, 284 outcome, 281, 282

treatment and prognosis, 281, 284

Idiopathic hypereosinophilic obliterative bronchiolitis, 292 Idiopathic infammatory myopathies, see Myositis Idiopathic interstitial pneumonias (IIPs), 533, 603, 625, 708 Idiopathic pleuroparenchymal broelastosis (iPPFE), 628

diagnostic criteria for, 630

Idiopathic pneumonia syndrome (IPS), 704

Idiopathic pulmonary brosis (IPF), 12, 191, 310, 412, 505, 506, 533, 547–549

common variants associated with, 424

Idiopathic pulmonary hemosiderosis etiologies, 151

Idiopathic pulmonary upper lobe brosis, 625 Idiopathic systemic eosinophilic vasculitis, 289 Illicit/abused drugs and agents, 743

Immune checkpoint inhibitors (ICI), 739, 743 Immune dysregulation, 93 Immunode ciency, 426

Immunoglobulin E (IgE), 39 Immunohistochemical analysis, 700 Immunohistochemistry, 252 Immunosuppressive therapies, 231 Indium–tin oxide (ITO), 646, 647 Infection, 224

management of, 451, 452 Infammatory bowel disease (IBD), 448

Infammatory myo broblastic tumor (IMT), 782, 783 Inhalation of acid anhydrides, 152

Inhaled GM-CSF, PAP, 397 Interferon, 429 Interlobular septa, 377

International Rare Diseases Research Consortium (IRDiRC), 18 Interstitial lung disease (ILD), 196, 224, 225, 239, 240, 309, 422, 425,

426, 504, 547, 625, 639, 640, 670 bronchoscopy or surgical lung biopsy, 541 cancer mimics, 779

epithelioid hemangio-endothelioma, 779, 780 lymphangitic carcinomaosis, 779, 780

characterization, 535 chest imaging, 534 chest radiograph, 535 classi cation, 670, 671 clinical assessment, 535 clinical features, 535

clinical presentation, 672, 673 computed tomography, 534 continuous image acquisition, 535 craniocaudal distributions, 536 de nition, 206, 671, 672 diagnosis, 534, 536

clinical features, 673

laboratory investigations, 673, 674 pathology, 674

radiology, 673 diagnostic criteria, 240, 534

controversies in, 242 diagnostic scenarios, 672

disease progression and prognosis, 242–244 epidemiology, 672

family history, 534

with human immunode ciency virus infection, 540 with idiopathic pulmonary brosis, 537 integration of individual features, 541

laboratory assessment, 539 laboratory studies, 540

with lymphangioleiomyomatosis, 540 management

antibrotics, 244 cyclophosphamide, 244 decisions, 543

MMF, 244 treatment, 677, 678

MCTD, 228 MDD, 541, 542 myositis, 230, 231

phenotyping unclassi able ILD, 675–677 physical exam, 535

progression and prognosis, 674 proposed features of, 676 pulmonary function test, 534 radiological features, 536

rare variants associated with, 422 Sjӧgren's syndrome, 226, 227 subtypes, 533

surgical lung biopsy, 541 symptoms, 535

with systemic sclerosis, 538

transbronchial biopsies and transbronchial lung cryobiopsies, 541 typical clinical features, 242

Interstitial pneumonia with autoimmune features (IPAF), 240, 676 characteristic features of, 241

Interstitial pneumonias, 239 Interstitial pneumonitis, 138

Interstitial/in ltrative lung disease (ILD), 733 Intrapulmonary lymph nodes, 683 Intrathoracic pseudotumors, 782

infammatory myo broblastic tumor, 782, 783 sclerosing mediastinitis and hyalinising granuloma, 783

Intravascular large B cell lymphoma, 694, 695 Intravenous immunoglobulin combined with steroids, 231 Intravenous immunoglobulins

granulomatosis with polyangiitis, 126 Irreversible pulmonary brosis, 752 ISHAM group diagnostic criteria, 44 ISHAM stages of ABPA, 46 Isocyanates, 152

Isolated pulmonary capillaritis, 146 Itraconazole therapy, 49, 297

J

Job syndrome, 448

Juvenile idiopathic infammatory myopathies (IIM)., 229

K

Kaposiform lymphangiomatosis (KLA), 375, 376 clinical presentation and diagnosis, 377 course/prognosis, 378

etiopathogenesis, 376, 377 management, 377, 378

KRAS mutations, 776

L

Langerhans cell histiocytosis (LCH), 249, 254, 355, 716, 717 with centrilobular nodules, 319

Laparoscopic anti-refux surgery (LARS), gastroesophageal refux, 410

Larva migrans syndrome, 293 Laryngeal amyloidosis, 84

Late-onset noninfectious pulmonary complications (LONIPCs), 705 Late radiation-induced injury, 767

Left ventricular dysfunction, 152 Leukocytosis, 588

Levamisole, 754

Life-threatening drug-induced/iatrogenic emergencies, 747–768 Light chain deposition disease (LCDD), 358

Lipid homeostasis, 398 Lipoid pneumonia, 430

Liver transplantation (LT), 175, 427 Liver vascular malformations, 466, 467 Löffer syndrome, 293

Lower esophageal sphincter

gastroesophageal refux, 406 L-tryptophan, 747

Lung allograft rejection, 149 Lung biopsy, 284

pulmonary Langerhans’ cell histiocytosis, 318 Lung cancer, 574–576

Lung disease, 344 idiopathic, 742 Lung brosis, 505

Lung function tests (LFTs), 554 Lung parenchymal involvement, 724 Lung pathology, PAP, 394

Lung toxicities, drugs, 704 Lung transplant

refux/GER/microaspiration in, 412

Lung transplantation, 321, 354, 398, 412, 427, 428, 708 for CTD-ILD, 233

Lymphangioleiomyomatosis (LAM), 333, 353, 367, 719, 720 abdominopelvic lymphatic disease, 342, 343

advanced disease, interventions for, 345 ATS/JRS diagnostic algorithm, 338 ATS/JRS diagnostic criteria, 345 clinical scenarios suggestive, 336 DCLD, 352

diagnostic approach, 353 pathogenesis, 352, 353

pathologic and radiographic characteristics, 353 prognosis and management, 353, 354

diagnosis and workup, 336, 338 drug treatment

anti-oestrogen therapy, 344 bronchodilators, 344 experimental therapies, 345 mTOR Inhibitors, 344

histological appearance, 340 investigations, 343

management, general measures, 340 oxygen therapy, 345

parenchymal lung disease, 340 pathogenesis, 333, 334 pleural disease, 340, 341 pregnancy, 343

presentation, 335 prevalence, 336 prognosis, 340

pulmonary hypertension, 345, 346 renal angiomyolipoma, 341 tuberous sclerosis, 343, 344

Lymphangitic carcinomaosis, 779 Lymphatic capillary, 369

Lymphatic development, complex thoracic lymphatic disorders, 370 Lymphatic disorders, clinical presentation of, 370, 371

Lymphatic fuid, 369

Lymphatic pulmonary edema, 371 Lymphatics, 368, 369 Lymphedema, 381

Lymphocytic alveolitis, 699 Lymphocytic infammation, 525

Lymphocytic interstitial pneumonia (LIP), 357 diagnostic approach, 357

pathogenesis, 357

pathological and radiographic characteristics, 357 pattern, 687, 689

prognosis and management, 357, 358 Lymphocytosis, 392

Lymphoid neoplastic proliferation, 700 Lymphoma, 226

Lymphoma cells, 694

Lymphomatoid granulomatosis (LYG), 695 Lymphoproliferative disease, 225, 226 Lymphoproliferative disorders

amyloidosis, 358

light chain deposition disease, 358 lymphocytic interstitial pneumonia/follicular

bronchiolitis, 357, 358

smoking-related diffuse cystic lung disease, 358 Lymphoproliferative lung disorders, 684–685 Lysinuric protein intolerance (LPI), 430, 431, 491 Lysosomal diseases, Hermansky-Pudlak syndrome, 431 Lysosomal lipid storage disease, 489

Lysosomal storage disorders, 431, 488

M

Macrophages, 277

Macrophages containing smoking-related inclusions, 525 Major histocompatibility complex, 162

Malignant lymphoproliferative diseases, 683 Malignant mimics of orphan lung diseases, 775

borderline neoplastic-non neoplastic disorders, 783 amyloid and non-amyloid immunoglobulin deposition

disorders, 784, 785

respiratory papillomatosis, 783, 784 interstitial lung diseases, 779

epithelioid hemangio-endothelioma, 779, 780 lymphangitic carcinomaosis, 779 lymphomatoid granulomatosis, 780

intrathoracic pseudotumors, 782

infammatory myo broblastic tumor, 782, 783 sclerosing mediastinitis and hyalinising granuloma, 783

lung adenocarcinoma/bronchioloalveolar carcinoma, 776 multiple cystic/cavitary lung disorders

cavitating tumors, 781 cystic tumors, 780, 781 organizing pneumonia, 775, 776

pulmonary hypertension pulmonary artery sarcoma, 781

pulmonary mucosa-associated lymphoid tissue, 777–779 rare tumors vs. orphan lung diseases, 785, 786

MALT lymphomas, 692, 693, 699 Manometry catheter, 408 Massive BAL eosinophilia, 757 Mastitis, 117

Maturation, 110 Medical therapy, 181

Medical thoracoscopy, 699 Meningioma, 336, 344 Mepolizumab, 46, 50, 281, 289

Methionyl-tRNA synthetase (MARS), 430 Methotrexate, 165, 231, 757 Methylmethacrylate cement embolism, 761 Methylprednisolone, 428

Microaspiration, 403, 414 Microbiome, 405

Microscopic polyangiitis (MPA), DAH, 146

Migratory pulmonary in ltrates, differential diagnosis of, 607 Mineral dusts, 31

Mitochondrial enzyme, 431

Mitogen-activated protein kinase (MAPK), 251 Mitomycin-C, 747

Mitral stenosis, 152

Mixed connective tissue disease (MCTD), 223, 228, 229 epidemiology, 227

pathophysiology, 227 prognosis, 228 pulmonary manifestations

ILD, 228

pulmonary hypertension, 228

Model for end-stage liver disease (MELD) score, 179 Monoclonal antibodies, 46

ABPA, 50

MonoMAC syndrome, 430 Morphologic domain, 240, 242

Mother against decapentaplegic homolog 4 (MADH4), 720 Motile cilia, 445

mTOR inhibitors, LAM, 344 mTOR pathway, 334

Mucociliary clearance disorders, 441 Mucosa-associated lymphoid tissue (MALT), 86, 683 Mucus plugging, 41

Multicenter International LAM Ef cacy of Sirolimus (MILES) Trial, 354

Multicentric Castleman disease (MCD), 691 Multidisciplinary (MDT) diagnosis, 527 Multiplanar reformats (MPR), 506

Multiple cystic/cavitary lung disorders (MCLD) cavitating tumors, 781

cystic tumors, 780, 781 Multisystem disease, 310 Mutations, 441 Mycophenolate, 198

Mycophenolate mofetil (MMF), 165, 208 ILD, 244

Mycosis fungoides, 698 Myelodysplasia, 426

Myeloid cell dysfunction, 390, 391 Myositis

epidemiology, 229 pathophysiology, 229, 230 prognosis, 232

pulmonary manifestations and treatments ILD, 230, 231

respiratory muscle weakness, 231 Myositis-associated pulmonary arterial

hypertension, 232

N

Nanoparticles, 653, 654 Nasal deformity, 112

Nasal natural-killer (NK)-cell lymphoma, 107 Nasal nitric oxide, 444

Nasal polyposis, 299 NDUFAF6, 431–432 Nebulised hypertonic, 451

Negative pressure pulmonary edema (NPPE), 766 Neoplastic lymphocytes, 693

Neurodegenerative CNS LCH, 314 Neuro bromatosis type 1 (NF1), 492

pulmonary hypertension, 718, 719 Neutrophils, 391

NF1-associated pulmonary hypertension, 718 Niemann-Pick disease, 431, 489, 490 Nintedanib, 198, 209, 244

NK cells, 162

NK2 homeobox 1 (NKX2-1), 494 Nodular amyloidosis, 784

Nodular lymphoid hyperplasia, 685

Non-amyloid immunoglobulin deposition disorders, 784

Nonamyloidotic monoclonal immunoglobulin deposition disease (NAMIDD), 784

Non-IPF ILDs, diagnostic characteristics, 677

Non-IPF progressive brotic interstitial lung diseases, 553–555 Nonproductive cough, 28

Non-small cell lung cancer, 576

Non-speci c interstitial pneumonia (NSIP), 392, 507, 595, 607 clinical course, 598

diagnostic evaluation, 596 epidemiology, 596 histopathology, 597, 598 history and de nition, 595, 596 presentation, 596

radiology, 596, 597 treatment, 598, 599

Non-tuberculous mycobacteria (NTM), 449

O

Obliterative bronchiolitis (OB), 211 Obstructive sleep apnea, 152 Obstructive ventilatory defect, 281 OFD1, 445

Omalizumab, 46

Oral mucosal lesions, 116

Oral pulmonary vasodilator medications, 181 Organ transplantation, 32, 33

Organic dusts, 31

Organizing pneumonia, 615, 708 AFOP, 614, 615

clinical course and outcome, 613 clinical features, 604, 605 de nition and terminology, 603 diagnostic criteria of, 619 differential diagnosis, 609, 610 disorders in, 608

epidemiology, 604

etiological diagnosis of, 610, 611 forms with respiratory failure, 613, 614

frequency of symptoms and signs in, 605 GOP, 615

histopathological diagnosis of, 608 histopathological pattern of, 604 imaging, 605

diffuse in ltrative form, 607, 608 isolated nodular form, 605, 607 multifocal form, 605

isolated nodular form of, 607 malignant mimics, 775, 776

lung adenocarcinoma/bronchioloalveolar carcinoma, 776 migratory opacities in, 606

pathogenesis, 604

syndrome, clinico-pathological diagnosis of, 608, 609 treatment, 611–613

Organizing pneumonia (OP), 525, 708 Orphan, 3

Orphan diseases, 11, 340, 450 clinical research

challenges of, 15

end points for trials, 17

national and international networks, 16 orphan drug development, 17

patient organizations, 15, 16

clinical research, challenges to overcome, 11 causation/disease pathogenesis, 13 disease complexity, 13

genetic component, variable degree of penetrance and environmental interactions, 12, 13

pulmonary alveolar proteinosis, 13, 14 reliable data on prevalence, lack of, 12 small number of patients, 12 unclear/imprecise de nitions, 13

diagnosis, lack of access to challenges but not negativity, 14 delay in, 14

success stories, 15

rare diseases, clinical research in, 19 referral centers, 17

economic burden, 18 ignorance, 18 patients, 18, 19

Orphan drugs, 8

Orphan lung diseases, 3, 625

care of patients, stakeholders, 4 clinical trials, 7

components of, 6 diagnostic challenges, 5 empowerment of patients, 8 expert centers, 6, 7

orphan drugs, 8 Orphanet, 8

patient organizations, 7

rare pulmonary diseases, wide spectrum of, 4, 5 research in, 7, 8

Orphanet, 8 Osler-Weber-Rendu disease, 468 Oximetry, 595

Oxygen therapy, LAM, 345

P

PAP syndrome, algorithm, diagnosis of, 395 Paraseptal emphysema, 568

Parenchymal excavated nodule, 113 Parenchymal involvement in BD, 168

Parenchymal lung disease, lymphangioleiomyomatosis, 340 Parenchymal pulmonary amyloidosis, 85, 86

Parenteral macro-aggregated albumin scanning, 184 Patient organizations, 7, 15, 16

Patients with pulmonary brosis (idiopathic and/or systemic disease-­ related), 759, 760

PCD diagnostic testing, recommendations, 444 PCR molecular genetic analysis, 700

Pediatric GPA, 118 Penetrance, 424 Pepsin, 404

Peri-operative emergencies, 763–766 Peristalsis, 406, 408

Peroxisome proliferator-activated receptor gamma (PPARγ), 390 Pharmacobezoars, 768

Pharmacological management, 554, 555 Phosphodiesterase 5 inhibitor medications, 181 Pill aspiration syndrome, 762

Pirfenidone, 244, 427

Plasma exchange, granulomatosis with polyangiitis, 126 Plasmapheresis, 149

Platelet derived growth factor (PDGF), 162 Platypnea, 183

Pleural disease, 212, 224 lymphangioleiomyomatosis, 340, 341

Pleural effusions, 381 Pleurisy, 212

Pleuroparenchymal broelastosis (PPFE), 196, 426, 512, 625, 767 clinical manifestations, 626

diagnosis, 630–632 epidemiology, 625, 626

high-resolution computed tomography ndings of, 628 laboratory ndings, 626, 627

pathologic features, 629 pathologic ndings of, 629 prognosis, 632, 634

radiological coexisting lower-lobe interstitial lung disease in, 628 radiologic features, 627

respiratory function, 627

survival analysis of patients with, 633 treatments, 632

Pneumocystis, 756

Pneumocystis jirovecii pneumonia (PJP), 779 Pneumonic-type lung adenocarcinoma (PTLA), 776 Pneumothorax, 354

Pneumotox, 738, 747, 754, 768 Poikiloderma, 431

Portopulmonary hypertension (PoPH), 175, 187 epidemiology, 175–177

molecular pathogenesis, 175–176, 178, 179 risk factors, 177

screening and diagnosis, 175–176, 179, 180 treatment for, 175–176, 180–182

Positive end expiratory pressure (PEP) techniques, 451 Positron emission tomography (PET), 79

Possible honeycombing, 566

Postoperative amiodarone-associated/induced ARDS, 759 Postoperative leftovers (surgical sponge, throat packs, surgical fabric

or catheters), 768

Post-transplant lymphoproliferative disorders (PTLDs), 698, 699 PPARγ agonist, PAP, 397

PR3-ANCA-associated vasculitis, 110 Prednisolone, 199

Prednisone, 598

Preemptive simulation practice, 760 Pregnancy, LAM, 343

Primary ciliary dyskinesia, 442–445

Primary immunode ciency disorders (PID), 447 chronic granulomatous disease, 447, 448 common variable immunode ciency, 447 X-linked agammaglobulinemia, 447

Primary lymphoproliferative disease, 683 Primary pulmonary artery sarcoma, 781

Primary pulmonary B -cell non-hodgkin lymphomas (PPBL), 691 Primary pulmonary Hodgkin lymphoma (PPHL), 698

Primary pulmonary lymphomas (PPL), 691 Primary pulmonary MALT lymphoma, 692, 693

Primary pulmonary non-Hodgkin lymphoma (NHL), 691 Prognosis, 516, 517

Progressive brosing ILD, 676 Progressive brotic phenotype, 518 Proliferative bronchiolitis, 34 Prostacyclin, 181 Pseudo-lymphoma, 685 Pseudomonas aeruginosa, 449 Pseudotumors, 775

Pulmonary alveolar microlithiasis (PAM), 473 biomarkers and dietary intervention, 477 clinical features, 477–479

diagnosis, 480 diagnostic criteria, 482

differential diagnosis of, 479 epidemiology, 473 management, 480, 481

pathogenesis, 473, 475, 476 pathologic appearance of, 474 pathological evaluation of, 474 radiographic appearance, 478 SLC34A2 mutations, 475

Pulmonary alveolar proteinosis (PAP), 13, 14, 388, 430, 490 clinical manifestations

clinical presentation, 392 pulmonary brosis, 392 secondary infections, 392

diagnosis

bronchoscopy and bronchoalveolar lavage, 393 diagnostic approach, 394

genetic testing, 393 GM-CSF autoantibodies, 393

GM-CSF signaling de ciency test, 393 laboratory studies and biomarkers, 393 lung pathology, 394

natural history and prognosis, 394 pulmonary function testing, 392 radiographic assessment, 393

epidemiology, 389 GATA, 430 history, 388, 389 imaging, 388

lysinuric protein intolerance, 430, 431 MARS, 430

pathogenesis

GM-CSF autoantibodies, 391, 392 GM-CSF signaling disruption, 389, 390 lymphocytosis, 392

myeloid cell dysfunction, 390, 391 surfactant homeostasis, 389

treatment, 395

inhaled GM-CSF, 397 subcutaneous GM-CSF, 396, 397 whole-lung lavage, 395, 396

Pulmonary alveolar proteinosis syndrome, 387 Pulmonary angiography, 165

Pulmonary arterial aneurysm (PAA) differential diagnosis, 169 treatment of, 169

Pulmonary arterial hypertension, 467 Pulmonary arteriovenous malformations, 463

anatomy, 463

clinical presentation, 463 diffuse, 466

pregnancy, 465 screening, 464 treatment, 465

Pulmonary artery aneurysm (PAA), 167, 168 Pulmonary artery involvement

in Takayasu’s arteritis, 163, 164 Pulmonary artery sarcoma, 781 Pulmonary artery thrombosis (PAT), 168

treatment of, 169 Pulmonary capillaritis

etiologies

anti-basement membrane antibody disease, 148, 149 antiphospholipid antibody syndrome, 147 granulomatosis with polyangiitis, 144–146

isolated pulmonary capillaritis, 146 lung allograft rejection, 149 microscopic polyangiitis, 146

systemic lupus erythematosus, 146, 147 histology, 144

Pulmonary capillary hemangiomatosis, 156

Pulmonary exacerbations, 451 Pulmonary brosis, 392, 485, 553

acute exacerbation of, 576 treatment, 577

Pulmonary brosis, genetic and familial familial interstitial pneumonia, 423 heritable forms with autoimmune features

COPA, 429, 430

TMEM173, 429 lysosomal diseases

Hermansky-Pudlak syndrome, 431 lysosomal storage disorders, 431 pulmonary alveolar proteinosis, 430

GATA2, 430

lysinuric protein intolerance, 430, 431 MARS, 430

surfactant pathway, 428 pulmonary involvement, 428 surfactant protein genes, 428 treatment, 428

telomere related genes anti brotic therapy, 427 emphysema, 426

extrapulmonary manifestations, 426 genetic, 424

hematological involvement, 426 hepatopulmonary syndrome, 426 immunode ciency, 426

liver involvement, 427

lung transplantation, 427, 428 mucocutaneous involvement, 426 pulmonary involvement, 425, 426 telomerase complex agonists, 427 telomere length, 424, 425

Pulmonary function, 138

Pulmonary function testing (PFT), 193, 194, 208, 529, 533 Pulmonary hemorrhage, 150

Pulmonary hypertension (PH), 186, 224, 225, 228, 466, 574, 713, 747 classi cation of, 714

clinical classi cation of, 714

combined pulmonary brosis and emphysema, 717 hereditary hemorrhagic telangiectasia, 720, 721 LAM, 345, 346

Langerhans cell histiocytosis, 716, 717 lymphangioleiomyomatosis, 719, 720 management, 577

neuro bromatosis type 1, 718, 719 pathologic assessment of, 715 pulmonary artery sarcoma, 781

pulmonary veno-occlusive disease, 721–723 sarcoidosis, 714–716

small patella syndrome, 723, 724 Pulmonary involvement, 5

Pulmonary Langerhans cell histiocytosis (PLCH), 253, 309, 313, 315, 525, 716, 785

chest radiography, 315 clinical features, 312–314 clinical presentation, 254 course and prognosis, 321, 322 DCLD, 354

Birt-Hogg-Dubé syndrome, 355 diagnostic approach, 356 pathogenesis, 355, 356

pathological and radiographic characteristics, 356 prognosis and management, 356

diagnostic approach, 355 pathogenesis, 354

pathological and radiographic characteristics, 354 prognosis and management, 355

epidemiology, 310, 311

high-resolution computed tomography, 315–318 history and classi cation, 310

lung biopsy, 318

molecular pathogenesis, 253 pathogenesis, 311, 312 pathology, 253, 254, 318, 319 pulmonary function test, 314, 315 treatment, 319–321

Pulmonary lymphatic disorders, 371 Pulmonary lymphatic system, structure and

function, 368–370 Pulmonary lymphatics, 369

Pulmonary lymphatics transport cells, 370 Pulmonary MALT-lymphomas, 692

Pulmonary mucosa-associated lymphoid tissue, 777–779 Pulmonary nodules and masses, 766, 767

Pulmonary perfusion MRI, 164 Pulmonary perfusion scintigraphy, 165 Pulmonary plasmacytoma, 693

Pulmonary primary MALT lymphoma, 778 Pulmonary renal syndrome, 115, 141, 151 Pulmonary toxicity of molecular oxygen, 766 Pulmonary vasculopathy, 199

Pulmonary veno-occlusive disease (PVOD), 152, 721–723

Q

Quantitative CT (QCT) analysis, 517, 518

R

Radiation-induced eosinophilic pneumonias, 297, 298 Radiolabeled albumin, 184

Randall disease, 784 Rare diseases, 11, 12

Rare lung diseases, challenges, 15

Rare pulmonary diseases, wide spectrum of, 4, 5 Rare tumors vs. orphan lung diseases, 785, 786 Raynaud phenomenon, 199, 722

Reactive pulmonary lymphoproliferative diseases, 683, 687, 691 Rebound phenomenon, 767

Recall pneumonitis, 767–768 Recombinant antigens, 41 Reconstructive surgery, 754

Reduced generation of multiple motile cilia (RGMMC), 447 Relapsing polychondritis, 65

clinical features, 65 laboratory ndings, 65 pulmonary function, 65 treatment, 65, 66

Renal angiomyolipoma, lymphangioleiomyomatosis, 341 Rendu–Osler–Weber syndrome, 720

Residual volume (RV), 194 Reslizumab, 290

Respiratory bronchiolitis, 27, 309

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), 323, 324

clinical features, 324

desquamative interstitial pneumonia, 326 epidemiologic and clinical features, 326, 327 epidemiology, 324

histopathological ndings, 324, 325, 327 prognosis and therapy, 325, 326, 328 radiological ndings, 325, 327, 328

Respiratory bronchiolitis interstitial lung disease/desquamative interstitial pneumonia

clinical course, 592 diagnostic evaluation, 591 epidemiology, 591 histopathology, 591, 592 history and de nition, 591 presentation, 591 radiology, 591

respiratory bronchiolitis, 590 treatment, 593

Respiratory cilia, 442 Respiratory damage

drug families, 736–738 idiopathic, 768

Respiratory manifestations of salicylate poisoning, 751 Respiratory muscle weakness, 231

Respiratory papillomatosis, 783, 784 Rheumatoid arthritis (RA), 205, 448 pulmonary involvement in, 205

Rheumatoid arthritis-associated airways disease (RA-AD), 210 clinical features, diagnosis and outcome, 210, 211 cricoarytenoid involvement, 212

epidemiology, 210 risk factors for, 210 subtypes

bronchiectasis, 211 COPD, 211, 212

obliterative bronchiolitis, 211

Rheumatoid arthritis-associated interstitial lung disease clinical features and diagnosis, 207, 208 epidemiology, 206

pathogenesis, 207 prognosis of, 209, 210 risk factors, 206, 207 treatment of, 208, 209

Rheumatoid arthritis-associated nonspeci c interstitial pneumonia (RA-NSIP), 206, 208

Rheumatoid arthritis-associated usual interstitial pneumonia (RA-UIP), 208, 209

Rheumatoid arthritis-related interstitial lung disease (RAILD), 512 Right heart catheterization (RHC), 187

hemodynamic measurement, 177

Right ventricular systolic pressure (RVSP), 179 Rituximab, 145, 198, 227, 700

granulomatosis with polyangiitis, 123, 124 Rosai-Dorfman Destombes disease (RDD), 249, 265

clinical presentation, 267, 268 diagnosis, 268

epidemiology, 265 etiology, 265 histopathology, 266 prognosis, 269 treatment, 268

Ruxolitinib, 429

S

Sarcoidosis, 29, 69, 641, 714–716 bronchosscopy, 70

imaging studies, 69, 70 pulmonary function studies, 69 treatment, 70

SARS CoV2 vaccination, 760 Sclerosing mediastinitis, 783 Sclerosis, 375

Sclerotherapy, 375

Secondary organizing pneumonia (SOP), 603 causes of, 610

Serology domain, 240

Serum amyloid A protein (SAA), 77 Serum phosphate, 478

Severe asthma with fungal sensitization (SAFS), 45 Severe hypoxemia, 184

Severe sudden asthma, 762 Shrinking lung syndrome, 224

Shwachman-Bodian-Diamond syndrome, 448 Sidero brosis, 654

Signaling pathway mutations, 373 Sildena l, 181

Silica-induced interstitial lung disease, 655 Silicone embolism syndrome (SES), 753

Silicone injection in the breast and mammaplasty, 754 Single nucleotide polymorphism (SNP), 423 Single-system disease, 310

Sirolimus, 354, 377

Sjögren’s syndrome (SS), 86, 223, 225, 357 epidemiology, 225

pathophysiology, 225 prognosis, 227 pulmonary manifestations airway disorders, 225 ILD, 226, 227

lymphoproliferative disease, 225, 226 Skin biopsies, 116

Small patella syndrome, 723, 724 Smoking cessation, 355

Smoking-related diffuse cystic lung disease, 358 Smoking-related idiopathic interstitial pneumonias (SR-IIP), 591

desquamative interstitial pneumonia clinical course, 594

diagnostic evaluation, 593 epidemiology, 593 histopathology, 594 history and de nition, 593 presentation, 593 radiology, 593, 594 treatment, 594, 595

non-speci c interstitial pneumonia, 595 clinical course, 598

diagnostic evaluation, 596 epidemiology, 596 histopathology, 597, 598 history and de nition, 595, 596 presentation, 596

radiology, 596, 597 treatment, 598, 599

respiratory bronchiolitis interstitial lung disease/desquamative interstitial pneumonia, 590–593

unclassi able interstitial pneumonia, 599 Smoking-related interstitial brosis (SRIF), 566, 570 Smoking-related interstitial lung diseases (SR-ILD), 309 Spiral CT angiography, 168

Spirometric volumes, 194 Spontaneous pneumothorax, 356 Sporadic LAM, 341

Squamous cell carcinoma, 576 Staging procedures, 700

Staphylococcus aureus, 110 Staphylococcus pseudintermedius, 110

STAT3 gene, 448 Stenosis, 165

STIR thoracic MRI, 165

Strongyloides stercoralis, 293

Subacute bacterial endocarditis (SBE), 464 Subcutaneous GM-CSF, PAP, 396, 397 Surfactant, 428

pulmonary involvement, 428 surfactant protein genes, 428 treatment, 428

Surfactant dysfunction disorders (SDDs), 492, 494 Surfactant protein B (SFTPB), 493

Surfactant protein C (SFTPC), 493 Surfactant protein D (SP-D), 478, 626 Surgical lung biopsy (SLB), 527 Systemic amyloidoses, 78

Systemic lupus erythematosus (SLE), 146, 147, 223 epidemiology, 222

pathophysiology, 222 prognosis, 224

pulmonary manifestations, 224

acute lupus pneumonitis and diffuse alveolar hemorrhage, 224 interstitial lung disease, 224

pleural disease, 224 shrinking lung syndrome, 224

thrombotic manifestations, 224

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) clinical presentations, 193

epidemiology of, 191, 192 genetic associations, 192, 193 imaging, 194, 195 management, 197–199 prognostic evaluation, 195–197

pulmonary function tests (PFTs), 193, 194 risk factors for, 192

T

T helper (Th) lymphocytes, 276 T helper 2 (Th2) cell, 39

T/natural killer-cell primary pulmonary lymphomas (T/NK PPL), 696, 698

Tacrolimus, 169

Tadala l, 181 Takayasu’s arteritis, 161

classi cation, 161 clinical features of, 163

clinical presentation of, 163 diagnosis of, 162 epidemiology, 161, 162 imaging studies, 164, 165 laboratory ndings, 164 pathogenesis, 162, 163 pathologic features, 162 prognosis, 166

pulmonary artery involvement, 163, 164 therapeutic management, 165, 166

Targeted vasodilator therapy, 181 TBX4 syndrome, 723 TBX4-related PAH, 724 Telomere related genes (TRG)

anti brotic therapy, 427 emphysema, 426

extrapulmonary manifestations, 426 genetic, 424

hematological involvement, 426 hepatopulmonary syndrome, 426 immunode ciency, 426

liver involvement, 427

lung transplantation, 427, 428 mucocutaneous involvement, 426

mutation, 424

pulmonary involvement, 425, 426 telomerase complex agonists, 427 telomere length, 424, 425

Telomeres, 424

Temporal homogeneity, 597 Terminal renal failure, 755

Thiazolidinedione (glitazones) family, 747 Thick-walled large cysts, 566, 569 Thoracic bezoars, 768

Thoracic CT scan, 164 Thoracic imaging, 179

Thoracic magnetic resonance imaging (MRI), 169 Thrombotic manifestations, 224

Thrombotic vasculopathy with intravascular brin formation, 754 Thyroid gland, 117

Tissue granulomatous and necrotizing infammation, 755 TMEM173, 429

TNF-alpha inhibitors, 705, 739 TNF antagonists, 758 Tobacco smoking, 322

CPEF, 560

Tobacco-related CPFE syndrome, 573 Tocilizumab, 198, 227

Tofacitinib, 429

Total lung capacity (TLC), 194 Toxic oil syndrome, 298 Trachea, 55

anatomical considerations, 55 clinical presentation, 55, 56 etiologies, 56

idiopathic subglottic stenosis, 56 Tracheal stenosis, 56

bronchoscopy, 58 clinical features, 57 imaging studies, 57 pathogenesis, 56–57

pulmonary function testing, 57 treatment, 58

Tracheobronchial amyloidosis, 68, 85 bronchoscopy, 69

clinical features, 68 imaging studies, 68

pulmonary function studies, 68 treatment, 69

Tracheobronchomegaly, 63 clinical features, 63 etiology, 63

imaging studies, 64 pathophysiology, 63 pulmonary function studies, 64 treatment, 64

Tracheobronchopathia osteochondroplastica, 58, 59 bronchoscopy, 59, 60

clinical features, 59 etiology, 59 imaging studies, 59 pathogenesis, 59

pulmonary function studies, 59 treatment, 60

Tracheomalacia, 60, 61 bronchoscopy, 62 clinical features, 61 etiology, 61 imaging studies, 62

pulmonary function studies, 61 treatment, 62, 63

Transbronchial biopsies, 529, 699 Transbronchial cryobiopsies, 278 Transbronchial lung biopsy (TBB), 318, 526, 527

Transbronchial lung cryobiopsy (TLCB), 527, 528 Transcription activator-like effector nucleases (TALENs), 453 Transhepatic chemoembolization sessions, 761

Trans-jugular intrahepatic portosystemic shunting (TIPS) procedures, 182

Trans-parietal ne needle aspiration/biopsy, 699 Transthoracic echocardiographic (TTE) imaging, 164, 179 Triazoles, 49

Triple threat of aspiration syndromes, 403 Tropical eosinophilia, 293

Tropomyosin 3 gene, 782 Tuberin, 334

Tuberous sclerosis, LAM, 343, 344

Tuberous sclerosis complex (TSC-LAM), 352 angiomyolipomas in, 342

Tumor necrosis factor-alpha (TNF-alpha) inhibitors, 33 Type 1 angioedema, 761

Type-II HPS, 186

U

UIP-IPF, 509

alternative diagnosis, 511 indeterminate, 510 probable, 510

Ultrashort echo times (UTE), 518

Unclassi able interstitial lung disease (ILD), 543, 671, 676 survival of patients with, 675

Unclassi able interstitial pneumonia, 599 Undifferentiated connective tissue disease (UCTD), 596 Unicentric Castleman disease, 691

Unilateral occlusion of a pulmonary artery, 165 Upper esophageal sphincter, 405, 406

US Orphan Drug Act, 11

Usual interstitial pneumonia (UIP), 191, 507, 526, 547, 548 chronic hypersensitivity pneumonitis, 550

connective tissue diseases, 550, 551 drug-induced lung diseases, 551

idiopathic pulmonary brosis, 549 radiation pneumonitis, 552

V

Valvular heart disease, 152, 747 Vascular Doppler, 165

Vascular endothelial growth factor (VEGF), 162 Vascular endothelial growth factor-D (VEGF-D), 337 Vemurafenib (VMF), 321

Veno-occlusive pattern, 716 Venous thrombosis, 117

Video-assisted thoracoscopic lung biopsy, 278 Video-assisted thoracoscopy or open lung thoracotomy, 700 Vitamin K

antagonists, 152 de ciency, 152 replacement therapy, 753

Volatile favoring agents bronchiolitis, 32

W

Warfarin, 199

Wegener granulomatosis, 107, 144 Whole-lung lavage (WLL), 430

PAP, 395, 396

World Health Organization Classi cation of tumors of lung, 691

X

X-linked agammaglobulinemia (XLA), 447

Y

Yellow nail syndrome (YNS), 367, 380, 381

Young syndrome, 449

Z

Zoledronic acid, 375