neutrophils and lymphocytes, 242 tools, 248

Transcription

1 Index A Acute eosinophilic pneumonia (AEP), Acute interstitial pneumonia (AIP) diffuse alveolar damage (DAD), 91 Hamman-rich disease, 91 Acute lung injury (ALI), 51 AEP. See Acute eosinophilic pneumonia AIP. See Acute interstitial pneumonia ALI. See Acute lung injury Allergic alveolitis. See Hypersensitivity pneumonitis Alveolar hemorrhage, Alveolar hemorrhage syndromes biopsy diagnosis, 63 caveats, differential diagnosis, 63 histologic findings, Alveolitis foamy alveolar macrophages, 45 plasma cell, 43 sarcoidosis, 42 subclinical, 45 Amyloidosis (AL) AA, 307 characteristics, 307 diffuse alveolar septal, nodular parenchymal, 308 plasma cell, 307 primary and secondary, tracheobronchial, 308 Angiotensin converting enzyme (ACE), Anti glomerular basement membrane (Anti-GBM) disease, 265 Antimalarial agents, Antineutrophic cytoplasmic autoantibodies (ANCA) anti-gbm, 366 C-ANCA and P-ANCA, 366 Churg Strauss syndrome, 366, 379 classic polyarteritis nodosa, 378 clinical features, 367 connective tissue disorders, 366 Goodpasture s syndrome, 366, 382 granular cytoplasmic staining, 366 immunosuppressive therapy, 366 microscopic polyangiitis, 377 pulmonary vasculitides, 366 Wegener s granulomatosis, 366, 369, 370, 375, 376 Anti-TNF agents demyelinating diseases, 112 hepatitis B, 113 infliximab, 111 local and systemic reactions, 113 monitoring guidelines, M. tuberculosis, pneumonia, 113 pregnancy, 113 randomized pulmonary disease trials, 112 rheumatoid arthritis, 111 Anti-TNF drugs, 229 Aspiration bronchiolar disease, 306 fibrosing lung disease, gastroesophageal, 305 ATP-binding cassette transporter A3 (ABCA3), 297 Azathioprine connective tissue disease, 229 drug therapy, B BAC. See Bronchioloalveolar carcinoma BAL. See Bronchoalveolar lavage BAL fluid (BALF) AM dominant cell type, 42 foamy/lipidladen, 42 phagocytized hemosiderin, 42 cell counting, 40 diagnostic applications, 38 disease-specific features, eosinophils, 44 immunohistiochemistry, lymphocytes CD4:CD8 ratio, 42 cellular profile, EAA patients, 42 Crohn s disease and cirrhosis, 42 mature lym, 42 sarcoidosis and EAA, mast cells, 44 R.P. Baughman and R.M. du Bois (eds.), Diffuse Lung Disease: A Practical Approach, DOI / , Springer Science+Business Media, LLC

2 388 Index BAL fluid (BALF) (cont.) plasma cells, 43 PMN, 43 reactive type II pneumocytes, 44 Behçet s disease characterization, 380 diagnosis, 380 embolization therapy, 381 massive hemoptysis, 380 prognosis, 381 symptomatology, 380 use azothioprine and cyclophosphamide, 381 Biological controls cotes states, 81 staff members, 79 trend analyses, 81 Bladder cancer, 109 Bronchiolitis acute inhalation injury, 349 biopsy diagnosis, 68 and bronchiectasis consequences and infections, 357 CT scan, 357 cylindrical, varicose and cystic types, 357 cystic fibrosis, 358 definition, 357 development, 358 indentify, 357 lesions, 357 mosaic attenuation, 357 Swyer-James and Kartagener syndrome, 358 caveats, 68 chronic cellular, classification acute/chronic, 346 alveoli, 346 bronchiectasis development, 346 clinical implications, 346 constrictive obliterans, etiological, 344, 345 inflammatory process, 346 obliterans features, pathologics, 344 proliferative obliterans, 346 connective tissue diseases ( see Connective tissue diseases) COPD, 356 cryptogenic constrictive explanation, 355 improvement, 355 lung biopsy, 355 symptom, 355 transmural, 355 X-ray, 355 defined, 344 differential diagnosis, 68 diffuse inflammatory lung disease, 358 diffuse panbronchiolitis, drug-induced bronchiolitis features, 349 iatrogenic, organizing pneumonia, 349 isolation, 349 sauropus androgynus, 349 follicular alveolar septal inflltration, 357 definition, infections and imaging, 357 therapy, 357 food flavorer s lung, 350 image patterns centrilobular nodules, 347 CT and HRCT, 347 differences, 347 mosaic attenuation, 347 tree-in-bud, 347 infection acute, 348 Aspergillus, 348 Bronchoalveolar lavage (BAL), 348 nontuberculous mycobacteria, 348 postinfectious, 348 viruses and bacteria consequences, 348 inflammatory and fibrotic changes, 66 interstitial lung disease, 356 langerhans cell histiocytosis, 359 latency, 350 lung function test airflow obstruction, 346 BOS and lung transplantation, 347 mild airways obstruction syndrome, lung transplantation ( see Lung transplantation) lymphangioleiomyomatosis, 359 malignant, 358 mesenchymal reaction, 66 mineral dust develop airflow obstruction, 349 smoking, 349 morphological lesions, 344 neuroendocrine cell hyperplasia, obliteration, plastic manufacture, 350 respiratory disease, 356 size and communications, 344 smoking, stabilize lung function, 350 therapy anti-inflammatory, immunosuppressive, 347 macrolide, 348 nasal cannula, 348 Bronchioloalveolar carcinoma (BAC), 22, 23, 311 Bronchoalveolar lavage (BAL) bronchoscopy, CD4/CD8 ratio, 247 confounding factors, 40 cytology, 272 description, 38 DILD, 38 etiological factor, 38 immunoglobulins, 248 lymphocytes, 243, 244, 247

3 Index neutrophils and lymphocytes, 242 tools, 248 C Chest radiograph (CXR), sarcoidosis description, 140 organ involvement, patients, 157 parenchymal disease, 154 radiographic resolution, 166 scadding scale, 142, 143 surface echocardiogram, 145 Childhood interstitial lung diseases (ILDs) ABCA3 gene mutations, 282, 283 adult disorders, 279, 281 aspiration, bronchiolitis obliterans adenovirus infection, 288 diagnosis, 288 therapies, classification and terminology, connective tissue disease, 289 description, diagnosis approach, 275 bronchoscopy and BAL, genetic test, history and physical examination, HRCT, 277 initial clinical testing, 276 lung biopsy, 278 PFT, 277 epidemiology, 274 follicular bronchiolitis/lymphocytic pneumonitis, 289 hypersensitivity pneumonitis, 287 lung biopsy, 279 lymphocytic inflammation, management, 278 mutation, 272 NEHI, neuroendocrine cell hyperplasia, 272, 273 PAP, pathogenesis, PIG, pulmonary hemorrhage syndromes, 287 SFTPB, 279, 281, 282 SFTPC, Children, ILDs. See Childhood interstitial lung diseases Churg Strauss syndrome clinical presentation and diagnosis allergic/asthmatic phase, 378 alveolar-type inflltrates, 378 ANCA presentation, 379 corticosteroid dose, 379 description, 378 development, 379 eosinophilic phase, 378 histopathology, 378 life-threatening, 378 necrotizing granuloma, renal failure, 378 skin biopsy, 379 steroid taper, 379 use leukotriene receptor, 379 parenchymal necrosis region, 60, 62 prognosis and treatment corticosteroids, 379 deaths, cardiac involvement, 379 documented outcomes, 379 hydroxyurea, alpha-interferon and rituximab, 379 Collagen vascular diseases (CVDs) biopsy diagnosis, 57 caveats, differential diagnosis, histologic findings, 56 Combined pulmonary fibrosis and emphysema, 264 Computed tomography (CT) high-resolution, 277 lung disease, 320 small airways diseases, 30 Connective tissue diseases (CTD) bronchiolitis, 232 follocular bronchiolitis, 351 histopathological considerations, ILD anti-tnf drugs, 229 assessment and management, 230 autoantibodies, azathioprine, 229 bronchoscopic evaluation, 221 clinical manifestation, 223 corticosteroids, cyclophosphamide, 228 cyclosporine, 229 drug induced lung disease, 220 lung transplantation, 230 management considerations, 227 mycophenolate mofetil, pulmonary hypertension, surgical lung biopsy, tacrolimus, 229 lung-dominant, lupus erythematosus, 351 multidisciplinary approach, NSIP, 196, 225 pulmonary manifestations, 218 radiologic characteristics, 197, rheumatoid arthritis characteristics, 350 defined, 350 development, 351 diagnosis, 350 histopathologic lesions, 351 HRCT and CT, 350 increase airflow obstruction, 351 interstitial lung disease, 350 lung function test, 350 obliterative bronchiolitis, 351 penicillamine, 351 treatment, 350

4 390 Index Connective tissue diseases (CTD) (cont.) rheumatologic diseases, 218, 219 screening, Sjögren syndrome, 351 UCTD, 197, 201, 225 COP. See Cryptogenic organizing pneumonia Corticosteroids connective tissue disease, drug therapy, 102, Cryptogenic organizing pneumonia (COP) community-acquired pneumonia, 90 HRCT scanning, 91 organizing pneumonia, 90, 91 CTD. See Connective tissue disease CVDs. See Collagen vascular diseases CXR. See Chest radiograph (CXR), sarcoidosis Cyclophosphamide connective tissue disease, 228 drug therapy, Cyclosporine, 229 Cytoplasmic-Antineutrophic cytoplasmic autoantibodies (C-ANCA), 366, 369, 370, 377, 378, 382 D DAD. See Diffuse alveolar damage Dendritic cells (DC), 196 Desquamative interstitial pneumonia (DIP). See also Respiratory bronchiolitis-associated interstitial lung disease alveolar macrophage accumulation, 92 chest imaging, 259 clinical features, 259 clubbing, 91 diagnosis, 260 history, 258 lower lobe ground glass abnormality, 92 pathology, physiology, 259 relationship, smoking, 259 treatment, 260 Diagnosis NSIP agreement, 200 histological features, 201, 209 HRCT, 200 radiologic (see Radiologic evaluation) Differential diagnosis DILD, 42 inflammatory cells, 42 Diffuse alveolar damage (DAD), 51 Diffuse lung disease (DLD) alveolar hemorrhage syndromes biopsy diagnosis, 63 caveats, differential diagnosis, 63 histologic findings, bronchiolitis, classification connective tissue disease, 87 drug-induced-related, 87 environmental exposure, 87 IIP, primary disease, collagen vascular diseases, cytotoxic therapy, 98 diagnosis, 50 diagnostic approach algorithm, 92, 93 bronchoscopy, 94 histopathology, 95 pulmonary function testing, 94 radiology, 94 drug reaction caveats, clinical patterns, 69 fludarabine, histologic findings acute lung injury (ALI), 51 alveolar space inflammation, 51 DAD, 51 fibrosis, 51 hypersensitivity pneumonitis (HP), 52 interstitial infiltrate, 51 type 2 cell proliferation, 51 HRCT, 50, 96, 97 hypersensitivity pneumonitis caveats, 65 differential diagnosis, histologic findings, 64 idiopathic pulmonary fibrosis, lymphangioleiomyomatosis biopsy diagnosis, 66 caveats, 66 differential diagnosis, histologic findings, 65 nonspecific interstitial pneumonia biopsy diagnosis, 56 caveats, 56 differential diagnosis, histologic findings, 55 pathology reports, 53 pneumoconioses, pulmonary alveolar proteinosis, 65 randomized, placebo-controlled trials, 95, 96 sarcoidosis, surgical lung biopsy, role, systematic approach, 86 tissue sampling cytologic and histologic techniques, 50 inflammation and fibrosis, 51 TBBx, 50 vasculitis, DIP. See Desquamative interstitial pneumonia Disease-specific features AIP, 46 chronic interstitial lung disease, 47 diffuse interstitial lung disease, 46 focal lung sensitization, 45 fungal infections, 45 DLD. See Diffuse lung disease

5 Index DLD, radiologic signs consolidation description, 18, 20 differential diagnosis, 18, 19 crazy paving pattern description, 21, 22 occurrences, cysts centrilobular emphysema, 22, 23 pulmonary, 22, 23 ground-glass attenuation description, 18, 20 differential diagnosis, 18, 19 honeycombing, 22 lines crazy paving pattern, reticular, 21 thickened interlobular septa, mosaic attenuation, 23, 24 pattern, nodules centrilobular, 18, 20 description, 18 differential diagnosis, 18, 20 perilymphatic, 18, 20 soft tissue density, 18, 20 tree-in-bud pattern, 18, 21 traction bronchiectasis/bronchiolectasis, 22 Drug-induced diffuse lung disease (DI-DLD) ARDS and ILD, 326 bronchoscopy, lung biopsy, and BAL, 333 clinical patterns, description, 326 disorders and therapy, 326 estimation, 331 histological data, 333 history and physical examination, HRCT, 331 limited dose, 331 mechanism, injury, 332 nitrofurantoin, 334 oncological patients, diagnosis, 331 polymyxin and aminoglycoside antibiotics, 334 pulmonary function tests, 334 pulmonary toxic effects, 331 risk and adverse reactions, sulfasalazine, 334 Drug-induced lung disease, 86, 87 Drug therapy antimalarial agents, anti-tnf agents demyelinating diseases, 112 hepatitis B, 113 infliximab, 111 local and systemic reactions, 113 monitoring guidelines, M. tuberculosis, pneumonia, 113 pregnancy, 113 randomized pulmonary disease trials, 112 rheumatoid arthritis, 111 azathioprine, classification, common toxicities and suggested monitoring, 103 corticosteroids, 102, cyclophosphamide, leflunomide, 106 methotrexate, mycophenolate mofetil, 108 N -acetyl cysteine, 107 pirfenidone, rituximab, sirolimus, 114 thalidomide, 110 usual dosage, 103 Dyspnea, 4, , 275, 302 E EAA. See Extrinsic allergic alveolitis Endobronchial ultrasound, 155 Environmental exposure, 87 Eosinophilia, Eosinophilic granuloma Histiocytosis X, lung biopsies, smoking, 260 unifocal LCH, 261 Epidemiology fibrotic obliteration, 124 IPF, 124 LAM, 124 pathophysiology concomitant emphysema, 127 CREST syndrome, 125 development and severity, PH, 125 genetic predisposition, 127 hilar lymph nodes, 125, 126 PH classification, WHO, 125 role, pulmonary endothelial dysfunction, 126 PFT and FVC, 124 PLCH, 124 prevalence, PH and sarcoidosis, 124 scleroderma, 124 SSc-PAH, 124 Erdheim Chester disease, 309 Extrapulmonary sarcoidosis acute sarcoid arthritis, 151 antimalarials and methotrexate, 162 azathioprine, corticosteroids, dysregulation, calcium metabolism, 149 eye corticosteroids, 146 CXR and CT, lacrimal gland and optic neuritis, 145 lacrimal gland biopsies, 146 uveitis, isocular, 145 heart diagnosis, granulomatous inflammation, 148 medications,

6 392 Index Extrapulmonary sarcoidosis (cont.) myriad, cardiac disorders, 148 noninvasive tests, 149 prognosis, heart failure, 149 leflunomide and cyclophosphamide, 163 liver granulomatous hepatitis, 147 hepatomegaly and cirrhosis, 147 symptoms, 147 lupus pernio skin lesions, nose, lymphadenopathy and skeletal muscle weakness, 151 manifestations and treatment, 152 morbidity and impact, patients life, 145 mycophenolate and mycophenolic acid, 163 neurologic cerebral and spinal, 148 cranial nerves, neurosarcoidosis, 147 treatment, corticosteroids, 148 palpable breast mass/lesion, mammography, 151 reproductive tracts, 151 sarcoid bone involvement, mechanisms, 151 skin antimalarial drugs, 146 lesions, classification, 146 Lupus pernio, 146 splenic, abdominal tract, 150 treatment, 161 upper respiratory tract, SURT, 150 Extrinsic allergic alveolitis (EAA) CD4:CD8 ratio, 41 cellular BALF profile, 41 hypersensitivity pneumonitis, 42 lipid-laden AMs, 42 oxygen free radicals, 40 F Farmer s lung emphysematous changes, 244 IgG response, 241 prevents (OR) control, 241 source, antigen, 240 treatment, 249 G GGO. See Ground glass opacification Goodpasture s disease alpha 3 collagen chain, 381 alveolar hemorrhage, 381 antibodies, 381 anti-gbm, 381 development factors, 381 diagnosis, outcome, 381 pulmonary and systemic symptoms, 381 treatment, 382 Goodpasture s syndrome, 331, 366, 382 Granulomas GLUS syndrome, 158 hepatic sarcoidosis, 147 histologic features, 155 inflammatory lesions, 146 non-necrotizing, sarcoid inflammation, 141 synovial biopsy, 151 TNF mediator, 142 Ground glass opacification (GGO), 197 H Hemorrhagic cystitis, 109 Hermansky Pudlak syndrome (HPS) character, 309 fibrosis, High-resolution computed tomography (HRCT) bronchial wall thickening, 92 DLD diagnosis, 94 focal consolidation, 91 ground-glass abnormality, 91 lung disease, 320 NSIP characterization, 207 radiologic characteristics, 197, serial, 200, 208 surgical lung biopsy, 200, 207 radiologic evaluation diagnosis, diffuse lung disease, differential diagnosis, infiltrative lung diseases, 18, 20 techniques, thickened interlobular septa, 20, 21 surgical lung biopsy, 97 traction bronchiectasis, 88 HP. See Hypersensitivity pneumonitis HPS. See Hermansky Pudlak syndrome HRCT. See High-resolution computed tomography Hypersensitivity pneumonitis (HP) acute chest radiograph, 242 high-resolution computed tomography, 242 laboratory test, 242 organic dust toxic syndrome (ODTS), 242 provoking agent, 241 symptoms, caveats, 65 chronic chest radiographs, 245 diagnosis, 245 diffuse coarse reticulonodular opacities, 245 high-resolution computed tomography, 245 mosaic pattern, 244 peribronchiolar inflammation, 244 symptoms, 244 description, 29 diagnostic assessment algorithm, 246, 247 clinical syndromes, 245 precipitating IgG antibody, 246 differential diagnosis, 19, 64 65

7 Index drug-induced, 44 epidemiology and risk factors etiologic agents, 240 MHC class I, class II alleles, non-tuberculous mycobacteria (NTM), 241 pigeon breeder s disease (PBD), 241 prevalence and incidence, etiologic agent, 239, 240 foamy/lipid-laden AM, 42 histologic findings, 64 increased Lym, 45 invasive tools for diagnosis bronchoalveolar lavage, histopathology, 248 inhalation challenge, 248 nodules, ground glass density, 18, 20 subacute air trapping, 243 bronchiolocentric micronodules, 243 diagnosis, 243 gradual development, histopathology, 243 lung fields, 243 pulmonary function test, 243 treatment and outcome, 249 upper lobe predominance, 25 I Idiopathic interstitial pneumonias (IIPs) AIP, 91 clinical characteristics, 197 COP, IPF, 88 LIP, 92 NSIP, provisional type, NSIP, 195 RBILD and DIP, unclassifiable, 92 Idiopathic pulmonary fibrosis (IPF) acute exacerbations, 173 acute respiratory failure, 188 biopsy diagnosis, 55 caveats, UIP, 55 clinical course and prognosis heterogeneous, 172 survival, 172 differential diagnosis, epidemiology chronic aspiration, 174 environment role, 174 infections, 174 meta-analysis, 174 occupational disease, 174 genetics, high-resolution computed tomography (HRCT), 88 histopathology, 88 incidence, 174 lung transplantation, novel agents anticoagulants, 188 interferon-g (IFN-g ), 188 pirfenidone, 188 PAH, pathogenesis, prognosis and thearpy, 172 radiographic manifestations, relative frequency, clinicopathologic sub groups, 88, 90 surgical lung biopsy(slb), 172 therapy ancillary, 187 indications, responses, 187 treatment, UIP clinical features, features, 172 pattern, 172 IIPs. See Idiopathic interstitial pneumonias ILDs. See Interstitial lung diseases Interstitial lung diseases (ILDs). See also Pulmonary hypertension connective tissue disease anti-tnf drugs, 229 assessment and management, 230 autoantibodies, azathioprine, 229 bronchoscopic evaluation, 221 clinical manifestation, 223 corticosteroids, cyclophosphamide, 228 cyclosporine, 229 drug induced lung disease, 220 lung transplantation, 230 management considerations, 227 mycophenolate mofetil, pulmonary hypertension, surgical lung biopsy, tacrolimus, 229 diagnostic approach, 38 differential diagnosis, diffuse, 42 drug therapy antimalarial agents, azathioprine, classification, common toxicities and suggested monitoring, 103 corticosteroids, 102, cyclophosphamide, leflunomide, 106 methotrexate, mycophenolate mofetil, 108 N -acetyl cysteine, 107 pirfenidone, rituximab, sirolimus, 114 thalidomide, 110 usual dosage, 103 patient evaluation ( see Patient evaluation) 393

8 394 Index Invasive tools, HP bronchoalveolar lavage alveolitis, 247 BAL neutrophils, 248 granulomatous diseases, 247 histopathology fibrosis, non-specific interstitial pneumonia pattern (NSIP), 249 patchy mononuclear cell infiltration, 248 terminal bronchioles, 248 inhalation challenge provocation test, 248 suspected agent, 248 IPF. See Idiopathic pulmonary fibrosis L LAM. See Lymphangioleiomyomatosis Langerhans cell histiocytosis (LCH), 303 LCH. See Langerhans cell histiocytosis Leflunomide, 106 LIP. See Lymphocytic interstitial pneumonia Lipoid pneumonia alveolar macrophages, 307 description, 306 oils, treatment, 307 Lung biopsy alveolar red blood cells, 63 bronchiolitis, 68 pattern recognition, 57 protocol, 278 tissue diagnosis, 50 UIP, 55 VATS, 278 Lung dominant connective tissue disease, Lung fibrosis, 249 Lung transplantation bone marrow graft airflow obstruction development, 354 chronic GVHD, 353 description, 353 disorders, 353 hematopoietic stem cell transplantation, 354 hypogammaglobulinemia, 354 photo-dynamic therapy, 354 prevalence, risk and infection, 354 obliterative bronchiolitis acute cellular rejection, 352 autoimmune injury, 353 biopsies surveillance, 353 clinical outcomes, 352 description, 351 diagnosis, BOS, function tests, 352 histopathological changes, 352 imaging, 352 lymphocytic inflltration, 352 management and rejection process, 353 re-transplantation, 353 survival, 352 transbronchial biopsies, 353 treatment, 353 paraneoplastic pemphigus, Lymphangioleiomyomatosis (LAM) biopsy diagnosis, 66 caveats, 66 differential diagnosis, epidemiology, 301 extrapulmonary features, 302 histologic findings, 65 imaging and diagnosis basilar and peripheral, 303 LCH, management angiomyolipoma, pleural disease, 304 progression, 304 pulmonary disease, pathophysiology hematoxylin and eosin, 301, 302 TSC1 and TSC2, presenting features, prognosis, 305 pulmonary physiology, 302 tuberous sclerosis (TS), 300 Lymphangitic carcinomatosis description, 311 Kerley s B line, 312 transbronchial biopsy, 312 Lymphocytic interstitial pneumonia (LIP) hypoxemia, 92 lymphoproliferative disorder, 92 Lymphocytic pneumonia, 330 M Methotrexate, , 162, 335, 375 Microscopic polyangiitis clinical presentation and diagnosis biopsy, 377 features, 377 treatment and prognosis C-ANCA and P-ANCA, 378 induces remission, 378 manifestation, 378 prognostic factor, survival, 377 M. tuberculosis, Mycophenolate mofetil (MMF) connective tissue disease, drug therapy, 108 N N -acetyl cysteine (NAC), 107 Neuroendocrine cell hyperplasia of infant (NEHI) ABCA3, BPD,

9 Index corticosteroids, 284 etiology, 284 Neurofibromatosis (NF), 310 NF. See Neurofibromatosis Nonspecific interstitial pneumonia (NSIP) autoimmune serologies, 90 biopsy diagnosis, 56 caveats, 56 clinical characteristics auto-antibody prevalence, 197, 201 description, diagnostic criteria, 197, 201 IIPs and bronchoalveolar lavage, 197 interstitial lung disease and pulmonary physiology, 197 laboratory evaluation, 197, 200 Raynaud s phenomenon, 197 UCTD, 197 description, 195 differential diagnosis, histologic findings, 55 hypersensitivity pneumonitis, 88 inflammation and fibrosis, 90 management and treatment azathioprine/cyclophosphamide, 209 survival and response, patients, nomenclature, 195 pathogenesis CTD and fibrotic lung disease, 196 DC and IPF, 196 idiopathic, 196 vs.uip, 196 pathologic characteristics agreement level, 208 description, features, 201, 209 histological features, 201, 209 surgical lung biopsies, 201, 208 prognosis histological features, 209 IPF/CFA, 209 radiographic characteristics description, 197, GGO, 197 HRCT features, 197, 207 vs. IPF, 200 serial radiographic, 200, 208 surgical lung biopsy, HRCT, 200, 207 NSIP. See Nonspecific interstitial pneumonia O Occupational and drug-induced disorders acute alveolar damage, 338 acute beryllium characteristics, 325 clinical features, 325 definition, 325 diagnosis and treatment, 325 manifestation and pathological examination, anti-inflammatory drugs aspirin, 335 leukotriene, 335 methotrexate, 335 naproxen, 335 penicillamine, 335 antineoplastic agents bleomycin, 337 busulfan, 337 cyclophosphamide, 337 dyspnea, mitomycin C, lung toxicity, 337 nitrosoureas, 337 procarbazine, 337 asbestosis, lungs and pleura accumulation, 324 clinical features, 324 description, 324 environmental and domestic exposure, 324 fiber retention, 324 lung function and clinical diagnosis, 325 lymph node, 324 plain chest radiogram, cardiovascular drugs amiodarone, 326 angiotensin-converting enzyme (ACE), 326 beta-adrenergic antagonists, 326 protamine sulfate, 326 clinical features breathlessness, cough, 322 diagnosis and complications, 323 radiographic and lung function, treatment, 323 coal workers pneumoconioses ( see Pneumoconioses) drug-induced DLDs ( see Drug-induced diffuse lung disease) drug induced lung disease, hard metal disease Cobalt exposure, 326 diagnosis, 326 leukocyte inhibition, 326 rhinitis and fibrosing alveolitis, 326 sintering process, 325 steroids and cyclophosphamide, 326 illicit cocaine, 338 heroin, 338 methadone and propoxyphene, 338 induced lung disease description, 319 environmenal issues, 320 pneumoconioses (see Pneumoconioses) respiratory effects, inhalants, 338 respiratory effects, silicosis, Organizing pneumonia +/-bronchitis pneumonia (BOOP), 329

10 396 Index P Patient evaluation clinical history acute vs. chronic onset, 4 demographic and family factor, 6 extrapulmonary symptoms, 6 occupational and environmental exposure history, 4 racial factors, 6 radiation and drug exposures, 6 respiratory symptoms, 4, 5 smoking, 5 6 invasive testing, 15 key elements, laboratory testing additional tests, 9 10 invasive procedures, peripheral blood and urine, 9 10 pulmonary function, 11 noninvasive testing, 14 physical examination extrapulmonary signs, 8 pulmonary findings, 7 8 thoracic imaging, 8 9 PBD. See Pigeon breeder s disease Pediatric, ILDs. See Childhood interstitial lung diseases Perinuclear-Antineutrophic cytoplasmic autoantibodies (P-ANCA), 366, , 382 PFT. See Pulmonary function tests PFTs. See Pulmonary function tests PIG. See Pulmonary interstitial glycogenosis Pigeon breeder s disease (PBD) data indicates, 241 indoor microbial contamination, 249 provocation test, 248 Pirfenidone, , 188 PLCH. See Pulmonary Langerhans cell histiocytosis PMN. See Polymorphonuclear neutrophils Pneumoconioses asbestosis, 69 berylliosis, 69 caveats, 69 coal worker Caplan s syndrome, 323 chest radiography, 324 clinical features, 324 develop silicotic nodules, 323 diagnosis and management, 324 lesion, 323 lung function, 324 macrophages and fibroblasts accumulation, 323 reumatoid, 323 tissue ration, 323 description, 68 histiocytic dust macules, 68 induced lung disease characteristics, 321 chest roentgenography, 320 clinical implications and risk, 320 CT and HRCT, 320 dust accumulation, lungs, 320 function and test, 320 particles deposition, 320 interstitial inflammation and peribronchiolar airspaces, 69 silicosis/silicatosis, Polymorphonuclear neutrophils (PMN) characteristic, 43 Eos and, 43 Progressive multifocal leukoencephalopathy (PML), 113 Pulmonary alveolar microlithiasis (PAM) chest radiograph, mutations, 310 ossification, 311 Pulmonary alveolar proteinosis (PAP) biopsy diagnosis, 65 causes, 287 caveats, 65 clinical presentation bronchoscopy, 298, 299 crazy-paving, dyspnea, 297 hematologic malignancies, description, 294 differential diagnosis, 65 geneticmolecular basis, 288 GM-CSF, histologic findings, 65 pathogenesis ABCA3, 297 GM-CSF, 294, 297 treatment GM-CSF, therapies, 300 WLL, 299 Pulmonary arterial hypertension (PAH) ancillary staging techniques, 183 BAL, 183 CRP scores, CT scans, 182 histological features, 179 HRCT, hypoxemia, minute walk test, MRI, 183 survival rates, 182 Pulmonary fibrosis collagen vascular disease, 44 sarcoidosis patients, 39 Pulmonary function tests (PFTs) deconstructing complex patterns ancillary tests, 78 disease processes, 77 emphysema, 77 FVC/DLco ratio, 78 Kco levels, 78 lymphocytic bronchiolitis, 78 pathological processes, 78 description, 72 diagnosis, 143

11 Index fitness to fly tests gold standard means, 82 hypoxaemia, 82 hypoxic inhalation, predictive equations, 82 plethysmograph and reduce oxygen conc., 82 ILD, 277 impairment airway component, 74 alveolar-arterial oxygen, 73 bronchiolar disorders, 74 disproportionate reduction, function, 73 hypersensitivity pneumonitis, 74 muscle weakness, 74 pulmonary vascular disease, 74 reduction in DLco, 73 restrictive ventilatory defect, 74 infant, 277 monitoring changes in disease severity, 78 clinical indication, 79 diagnosis and management, 80 DILDs, emphysema, pulmonary fibrosis, 80 forced vital capacity (FVC), 79 indications ambulatory oxygen, 81 marginal reduction, 79 precedence established, PAH, 80 short term PFT, 79 significant changes, 79 PF variables, 72 prognostic evaluation stages arterial hypoxaemia, 76 CRP, 76 DLco advantages, HRCT, 75 idiopathic pulmonary fibrosis, 75 maximal exercise test, 76 PFT use, 75 prognostic value, 76 scleroderma lung, 77 quality assurance, sarcoidosis lung restriction, 75 morphologic abnormalities, 75 non-caseating granulomas, scadding stage I radiographs, 144 use ancillary role, 72 dyspnoea, 73 lung volumes, 72 minor reductions, 72 multi-compartment disorders, 73 musculoskeletal limitation, 73 patient cohorts, 72 routine baseline stage, 2 workup, granulomatous inflammation, 144 Pulmonary hypertension CAT, IPF, 122 clinical implications, diagnosis, DOE, 121 FDA and WHO group 1, 129 forced vital capacity (FVC), 122 Group 3, PH, 123 microscopic vessels, min walk test (6MWT), 121 N -acetyl cysteine (NAC), 122 physiologic and pathological features, 123 RHC hemodynamics, 123 right heart catheterization (RHC), 122 sarcoidosis, stage IV, 122 transplantation, 132 treatment, Pulmonary interstitial glycogenosis (PIG) cells, 284, 285 etiology, Pulmonary Langerhans cell histiocytosis (PLCH) chest imaging, clinical features, 261 diagnosis, 263 history, pathology, physiology, 262 relationship, smoking, 261 treatment, Pulmonary sarcoidosis dyspnea diagnostic considerations, patients, SAPH, 145 treatment, high frequency, lung involvement, 142 imaging chest CT scanning, scadding scale, CXR classification system, 142, 143 PFTs, physiologic tests, 144 symptoms and signs, 142 Pulmonary vasculitis alveolar hemorrhage syndrome, 366 ANCA, autoantibodies, 366 Behçet s disease, C-ANCA and P-ANCA, 366 churg strauss syndrome ( see Churg strauss syndrome) classic polyarteritis nodosa, 378 connective tissue disorders, 366 constitutional symptoms, 366 description, 365 disorders characterization, 366 forms, 365 giant cell arteritis affect women, 379 bilateral biopsies, 380 Caucasians, 379 complications, 380 cough and aortic arch syndrome, 379 diagnosis, 380 epidemiology and manifestation, 379 inflammation, 379

12 398 Index Pulmonary vasculitis (cont.) seldom and prednisone therapy, 380 symptoms, 379 Goodpasture s disease ( see Goodpasture s disease) immunosuppressive therapy, 366 microscopic polyangiitis ( see Microscopic polyangiitis) pauci-immune capillaritis, 379 syndrome, Takayasu s arteritis, 380 Wegener s granulomatosis ( see Wegener s granulomatosis) Q Quality assurance biological control data, 81 cotes states, 81 inter-laboratory variability, 82 quality control and quality assurance, 81 rigorous quality assurance, 81 trend analyses, 81 two decades, 81 R Radiographic manifestations, IPF conventional chest, 175 CT, cystic radiolucencies, 176 diagnosis, UIP, 176 honeycomb change (HC), 177, 178 pulmonary function tests, 189 Radiologic evaluation characterization, interstitial lung disease biopsy, 27 first choice diagnosis, 26 usual interstitial pneumonia (UIP), collagen vascular disease, 28 CT techniques attention, requirement, HRCT images, 17, 18 cystic lung diseases LAM, 30 PLCH, 30 detection, early interstitial lung disease, 26 diagnosis, diffuse lung disease HRCT, 31 strategy, CT features, 31, 32 diffuse lung disease, signs consolidation, 18 crazy paving pattern, cysts, 22 ground-glass attenuation, 18 honeycombing, 22 lines, mosaic attenuation, 23, 24 mosaic pattern, nodules, traction bronchiectasis/bronchiolectasis, 22 distribution, lung diseases differential diagnosis, 24, 25 interlobular septa, perilymphatic abnormalities, 24 secondary pulmonary lobule, tree-in-bud sign, 21, 25 upper and lower, predominant, 24, 25 hypersensitivity pneumonitis, 29 idiopathic interstitial pneumonias consolidation, 28 diagnosis, UIP, fibrosis, 27 multidisciplinary workshop, 28 predominant CT pattern, 27 sarcoidosis fibrotic, 27, 29 lymphadenopathy and nodules, small airways disease cellular bronchiolitis, 30 constrictive bronchiolitis, 30 CT-based classification, 30, 31 panbronchiolitis, 31 smoking-related lung diseases description, 29 DIP, RB-ILD, 29 Rare interstitial lung diseases (ILDs) amyloidosis, aspiration, BAC, 311 Erdheim Chester disease, 309 HPS, information, LAM, lipoid pneumonia, lymphangitic carcinomatosis, NF, 310 PAM, PAP, 294, PLCH, 305 Raynaud s phenomenon, 197 RB-ILD. See Respiratory bronchiolitis-associated interstitial lung disease Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) focal air trapping, 92 pulmonary function, 91 respiratory bronchiolitis, 92 Respiratory bronchiolitis interstitial lung disease chest imaging, 256 clinical features, 256 history, pathology and diagnosis, 257 physiology, relationship, smoking, 256 treatment, Rheumatologic diseases, 218, 219 Rituximab,

13 Index S SAPH. See Sarcoidosis-associated pulmonary hypertension Sarcoidosis biopsy diagnosis, biopsy site selection CD4/CD8 ratio, 155 clinical presentations, 154 morbidity and risk complications, 154 TBLB, caveats, 54 CD4:CD8 ratio, 41 clinical findings clinicoradiographic data, 154 SACE, 153 clinical vignette, decision, treat, definition/diagnosis, 140 description, 140 diagnosis algorithm, 154 differential diagnosis, 53 different lobes, 40 EAA, 42 exclusion, granulomatous inflammation, 155 extrapulmonary, fibrotic, 27, 29 histologic findings, 53 idiopathic systemic, granulomatous disease, 158 immunopathogenesis and pathology epidemiologic evidence, 140 genetic predisposition, 141 inflammation, non-necrotizing granulomas, 141 mediator, TNF, 142 Th1-predominant cytokine profile, inflammation and organ systems, 42 Kveim test, 158 lymphadenopathy and nodules, lymphocyte alveolitis, 42 multiple organ involvement ACCESS, 156 idiopathic panuveitis, 156 organ involvement, patients, pathologic diagnosis, 156 pathology, 155 penumbra, 160 prognosis acute and chronic, 164 factors, extrapulmonary, 165 mortality, 165 therapy, natural history, time frame, resolution, 164 pulmonary, Sarcoidosis-associated pulmonary hypertension (SAPH), 145 Scleroderma (SSc), 124 Silicosis accelerated, 322 acute, 322 chronic, 320, 321 collagen deposition, 322 development, 322 dust exposure, occupation, extra-thoracic manifestation, 322 features, 322 meaning, 320 Sirolimus, 114 Smoking-related interstitial lung disease AEP (see Acute eosinophilic pneumonia) anti-gbm disease, 265 combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis, 264 desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis interstitial lung disease, Staging disease severity arterial hypoxaemia, 11 high resolution computed tomography (HRCT), 11 idiopathic pulmonary fibrosis, 11 uses, 11 Steroid-sparing medications azathioprine, methotrexate, 162 Subacute cellular interstitial pneumonitis, Surfactant protein B ABCA3 mutations, 282 chest radiographs, 281 mutation, 279 Surfactant protein C (SFTPC) cystic lesions, 285, 286 pathogenesis, 286 presentation, 285 RSV, 286 Surgical lung biopsy (SLB) accurate histopathologic evaluation, 94 bronchiolitis, 68 clinical setting, 62 connective tissue disease, interstitial lymphocytic infiltrate, 92 patient evaluation, 12 pattern recognition, 57 respiratory bronchiolitis pattern, 92 role clinical diagnosis accuracy, IPF, clinicopathologic subclassification, 95 response, treatment, 95 survival, UIP, 55 usual interstitial pneumonia (UIP), 88 T Tacrolimus, 229 TBBx. See Transbronchial biopsies Thalidomide, 110 Thiopurine S-methyltransferase (TPMT), 107 TNF antagonists, 164 Transbronchial biopsies (TBBx) alveolar hemorrhage, 63 histologic changes, 50 sarcoidosis, 52 tissue sampling, 50 Transbronchial biopsy, 12

14 400 Index U UCTD. See Undifferentiated connective tissue disease UIP. See Usual interstitial pneumonitis Undifferentiated connective tissue disease (UCTD), 197, 225 Usual interstitial pneumonitis (UIP) cardinal histopathological findings, 172 clinical features elevations, BALF, 173 physical examination, CT diagnosis, differential diagnosis, infiltrative lung diseases, 25 pathogenesis alveolar epithelial cell (EC) injury, 183 dysregulation of fibroblasts (FBs), 183 fibroblastic foci (FF), 184 inflammation and fibrosis, 184 mechanisms, 184 myofibroblast differentiation and fate, 184 reticular lines, 21 V Vasculitis biopsy diagnosis, 62 caveats, 62 Churg Strauss syndrome, 60 differential diagnosis, histologic findings, microscopic polyangiitis, 60 Wegener s granulomatosis, 60 VATS. See Video-assisted thoracoscopic surgery Video-assisted thoracoscopic surgery (VATS), 12, 272 W Wegener s granulomatosis beyond standard therapy B-cell-targeted therapy, 376 explanattion, 376 infliximab, 376 investigation, T-cell function, 376 outcomes, 376 plasma exchange, 376 therapeutic agents, 376 biopsy diagnosis, 372 renal and open lung, 373 side effects, 372 blood counts and serology, 370 bronchoscopy, 372 clinical management algorithm treatment, 374 fatal disease, 374 immunosuppressive therapy, 374 induction therapy, 374 manifestations, 374 clinical presentation abnormalities, 368 affect skin, 369 alveolar hemorrhage, 368, 370 C-ANCA, control, 369 description, 368 diagnosis, granulomatous involvement, 369 inflammation and nasal cartilage, 368 manifestation, 369 necrotizing, 368 ophthalmologic involvement, 369 pathognomonic feature, 368 renal outcome, 369 severe headache, 369 symptoms and manifestations, 368 investigations, 370 limited disease, induction therapy, 375 maintenance remission azothioprine, 375 methotrexate, 375 mycophenolate mofetil, 375 therapy duration, 376 trimethoprim/sulfamethoxazole, monitoring therapy, pulmonary function test determination, 372 diffusing capacity, 372 dyspnea, 372 flow volume loop, 372, 373 function data, 372 visual inspection, 372 radiography alveolar hemorrhage, diffuse infltrates, 371 chest roentgenogram and CT, 370 CT and MRI scan, effusions, 371 lesions, 371 lesions cavitate, nodules/masses, 369 pathognomonic feature, 368, 371 remission induction cyclophosphamide, 374, 375 efficacy, 375 infection rate, 375 limit toxicity, 375 management problems, 374 rituximab, 375 standard regimen, 374 treatment, 374 sequelae management, 376 Whole lung lavage (WLL), 299 WLL. See Whole lung lavage