Despite this wide variation in precursor proteins, the resulting insoluble protein deposit in amyloidosis is struc- turally alike in all forms of the disease, comprising B-pleated sh eets complexed with serum amyloid P (SAP) and glycosaminoglycans (GAG). This con \201 gura tion can resist proteolysis and is thus extremely stable, to the detri- ment of the organ a ffected. Whilst all forms of amyloidosis can lead to systemic dis- ease, AL amyloidosis is unique in that it can be associated with either local ( \200\230organ-limited \200\231) or systemic disease. \200 History The diagnosis of amyloidosis will rarely be made at initial presentation. However, do consider the diagnosis in patients who present with: \200 \200 \200 \200 \200 Upper airway obstructive symptoms. Haemoptysis. Incidental pulmonary nodules. Dyspnoea and parenchymal in \201 ltrates. Respiratory symptoms in the presence of an underlying parapro teinaemia. Local AL disease results from localized clonal expansion of B cells or plasma cells. The li ght chains secreted by these cells do not circulate outside of their target organ, but inst ead produce local amyloid deposits. Systemic AL disease develops following the release of m onoclonal light chains into the circulation by bone marrow-derived plasma cells. The cause of plasma cell expansion can range from a low grade plasma cell dys- crasia (i.e. primary s ystemic amyloidosis) to an overt malignancy (i.e. amyloidosis associated with myeloma). Examination Patients may exhibit: \200 \200 \200 Macroglossia (systemic AL). Inspiratory stridor (TBA). Fine inspiratory crepitations and hy poxia (diffuse alveo- lar septal amyloidosis). Clinical manifestations Although pulmonary involvement can occur with AA and ATTR disease, this is usually an incidental \201 nding and is rarely of any clinical signi \201 cance. Conversely, AL amyloidosis can present in a number of ways, some of which can overlap \200 Investigations Initial investigations \200 PFTs may demonstrate upper airways obstruction (i.e. low PEFR, abnormal \202 ow volume lo ops) in TBA, or restrictive lung disease (i.e. reduced transfer factor and vital capacity) in diffuse alveolar septal disease. CXR may reveal pulmonary nodules, diffuse parenchy- mal in \201 ltrates or pleural effusions. Laryngeal amyloidosis (local AL) presents with hoarse- ness, stridor or dyspnoea as a resul t of nodular or diffuse in \201 ltrative changes within the larynx. Tracheobronchial amylo idosis (TBA) (local AL) appears as discrete nodules that can macroscopically mimic tumor (2 8%), as submucosal in \201 ltrative plaques (44%) or as cirumferential wall thickening (28 %). All forms cause upper airway obstructive symptoms (cough, hae- moptysis, dyspnoea, and recurrent infections) and are not infrequently initially misdiagnosed as asthma. Diagnostic investigations \200 Biopsy material can be obtained as appropriate at: \200 laryngoscopy or bronchoscopy, although note that amyloid lesions can bleed readily; thoraco scopy; and thoracotomy. \200 CHAPTER 18.8 Pulmonary amyloidosis 459 Management \200 A biopsy demonstrating both congo red staining and green birefringence under polarized ligh t con \201 rms the diagnosis. Further histological analysis should be performed to identif y the precursor protein subtype as distinguishing AA and ATTR from AL disease can alter sub sequent management (Fig. 18.8.1). In many cases, particularly AA disease, ATTR disease and pulmonary nodular disease, pulmona ry involvement is an incidental \201 nding and needs no speci \201 c treatment. In local ized laryngeal or proximal tracheobronchial amy- loidosis repeated local interventions may be required for symptom control, including endoscopic resection, YAG laser therapy, stentin g and tracheostomy. In more diffuse and/or severe disease, management takes on a more suppo rtive role and includes prompt treatment of infections and emergency treatment of haemoptys is (e. g. with tranexamic acid and therapeutic bronchoscopy). In systemic AL disease chemot herapy can be given to target B cell clonal disease and thus limit the supply of precursor proteins. Both low dose melphalan and pred- nisolone and high dose melphalan with autologou s stem cell rescue have been used in this role and should be considered when detectable lev els of paraprotein are present. A centrally funded tertiary referral service for amyloido- sis is currently provided in the UK by the National Amyloidosis Center at the Royal Free Ho spital, London. Patients and clinicians may obtain further information at www.ucl.ac.uk/med icine/amyloidosis/nac/index. html Fig. 18.8.1 Histological example demonstrating the presence of apple-green birefringence. Staging investigations \200 HRCT and/or MRI may demonstrate pulmonary nod- ules, honeycombing, heterogeneous ground gla ss opaci- \201 cation or generalized lymphadenopathy. Serum electrophoresis, urinary Benc e Jones protein and bone marrow aspirate can identify an underlying parap- roteinaemia in s ystemic AL. An elevated serum ALP and hepatomegaly on ultrasound suggest liver involvement. Echocardiography 9myocardial biopsy can identify restrictive and dilated cardiomyopathy. 2 4-hour urinary protein collection is required to exclude or con \201 rm nephrotic syndrome . Radiolabelled SAP scanning can help quantify and moni- tor overall disease burden, althou gh this test is not widely available. Beware FDG-PET which can give false-positive results in pulmonary nodular amyloidosis. Prognosis Systemic AL disease has an unfavorable outcome with median survival estimated at just 13 months. Prognosis of local AL disease can vary, ranging from several months for tho se with diffuse TBA disease to unlimited for those with parenchymal nodular disease. AUTHOR \200\231S TIPS \200 Amyloidosis is one of many conditions that can mimic lung cancer always aim to obtain a tis sue diagnosis. Once amyloidosis is diagnosed it is essential that sys- temic AL be distingu ished from local AL disease, given the change this can make to management. Further reading Aylwin AC, Gishen P, Copley SJ. Imaging appearance of thoracic amyloidosis. J Thorac Imaging 2005;20(1):41 \200\22346. Berk JL, O \200\231Regan A, Skinner M. Pulmonary and tracheobronchial amyloidosis. Semin Respir Crit Care Med 2002;23(2):155 \200\223515. Cordier JF (2005). Pulm onary amyloidosis in hematological disor- ders. Semin Respir Crit Care Med 2005;26(5):502 \200\223513 Fig. 18.8.2 MRI thorax demonstrating both TBA disease affecting the carina and nodular pare nchymal disease involving the left lower lobe. This page intentionally left blank 461 Index Note: page numbers in italics refer to Figures and Tables. A A-a gradient 28 \200\22330 AAS V (anti-neutrophil cytoplasmic antibody in pulmonary hypertension 267 AL amyloidosis 460 \200\2231 ALK-1 gene 265, 459 all-trans retinoic acid, use in AAT de \201 ciency 122 allergen avoidance 65 allergen desensitization 66 allergen immunotherapy 62 allergic bronchopulmonary aspergillosis anti-fungal therapy 198 antigen avoidance, in extrinsic allergic alveolitis 150 (ANCA)-associated systemic vasculitides) 272 \200\2235 ANCA patterns 275 \200\2236 anti-glo merular basement membrane antigen challenge tests, extrinsic allergic alveolitis 149 \200\22350 anti-glomerular basement membrane antibody disease 278 (ABPA) 196 antibody disease 278 anti-IgE therapy, asthma 74, 86 anti-in \202 ammatory therapy, cystic \201 brosis 245 an ti-leukotrienes 73 \200\2234, 80, 85 \200\2236 in cystic \201 brosis patients 237, 246 comparison 276 diffuse alveolar hemorrhage (DAH) 277 \200\2238 disease activity monitoring 277 prognosis 277 treatment 276 \200\2237 allergic occupational asthma 398 allergic rhinitis 64 in COPD 102 effectiveness in asthma control 85 anti-neutrophil cytoplasmic antibody diagnostic tests 64 \200\2235 treatment 65 \200\2236 allergy, role in asthma 60 alpha1-antitrypsin assay 99 alpha1-antitrypsin augmentation ther apy 122 alpha1-antitrypsin de \201 ciency 90, 92, 93 abdominal pain, cystic \201 brosis 253 \200\2234 abscesses 180 \200\2231 see ANCA anti-neutrophil cytoplasmic antibody differentiation from empyema 355 TB 193 (ANCA)-associated systemic vasculitides see AASV clinical features 120 investigations 120 \200\2231 management 122 natural history 121 patho physiology 120 support groups 122 altitude sickness 406 alveolar adenoma 314 alveolar macro phage function, PAP 442 alveolar silico-lipoproteinosis 392 alveolitis, HIV-associated 220 amantadine 187 ambulatory oxygen therapy (AOT) 39, 124 accessory respiratory muscles 32 accident risk, obstructive sleep apnoea 364 ACE inhibitors antioxidant drugs, in COPD 102 antiretroviral drugs effect on LIP 143 interactions 215 \200 \22316 as cause of respiratory disease 387 cough sensitivity 178 antituberculous drugs 189 acetazolamide, in Cheyne \200\223Stokes drug resistance 190 in HIV patients 215 \200\22316 LFT monitoring 190 in opportunist mycoba cterial infection 195 respiration 381 acid/base balance 30 \200\2231 Acinetobacter sp. as cause of HAP 173 acrome galy 163 actinomycosis 183 acute asthma 78 \200\22380 anti-tumor necrosis factor, in asthma 75 antiviral therapy 187 in cystic \201 brosis 245 aortic arch, right-sided 5 AP chest X-ray 4 apoptosis, COPD 93 argon plasma coagulation (APC) 320 arrhythmias, in pulmonary hypertension 267 arterial bloo d gasses irritant induced 398 in COPD 99, 103 indications 125 patient assessment 126 acute extrinsic allergic alveolitis 148 acute interstitial pneumonia (AIP) 140 acute mounta in sickness 404, 406, 407 acute rejection, lung transplantation aminophylline, in acute asthma 80 aminosalicylates, association with 424, 426, 427 pneumonitis 161 acute respiratory distress syndrome acid/base balance 30 in acute asthma 79 in COPD 98 \200\2239 in HIV patients 211 informatio n about gas exchange 28 \200\2239 pre-operative analysis 44 relationship between PaO2 and S aO2 30 amiodarone, pneumotoxicity 164, 385 \200\2236 amoebiasis 199 amylase levels, pleural \202 uid 330 amyloid tumor 314 amyloidosis 458 \200\2239 anemia, breathlessness 163 anatomy 2 \200\2233 (ARDS) 161, 430 \200\2233 after liver transplantation 203 drug-induced 385 in fulminant hepatic failure 162 mechanica l ventilation 434, 435 adaptive servo-ventilation (ASV) 381 adenoid cystic carcinoma, trach eal 454 adenomatoid tumor 312 adenovirus infection causes of obstructive sleep apnoea 363 arterial blood sampling 28 arterial gas embolism (AGE) 409 arterial O2 desaturation 25 arte riovenous malformations 315, 458 \200\2239 ASA Physical Status classi \201 cation system 4 4 asbestos, \201 bre types 414 asbestos exposure ANCA (anti-neutrophil cytoplasmic antibody) AASV 272 \200\2235 patterns in AASV 275 \200\2236 clinical characteristics 185 in immune compromise patients 207 angiofolicular lymph node hyperplasia adenovirus vaccines 186 \200\2237 ADH, inappropriate secretion 286 adjuvant chemotherapy, N SCLC 298 adjuvant radiotherapy, NSCLC 297 adverse effects of medication (Castleman \200\231s disease) 315 angiomyolipomas 313, 452 \200\2233 anion gap 31 anthropom etry, COPD 99 antibiotic allergies, cystic \201 brosis 238, 246 antibiotic resistance lung cancer risk 283 mesothelioma risk 348 asbestos-related disease 414 \200\22316 anticholinergics 70 anti-leukotrienes 74 beta2-adrenoreceptor agonists 69 corticosteroids 7 2 cromones 73 theophylline 70 see also mesothelioma in cystic \201 brosis patients 238 in HAP 174 \200\2235 Streptococcus pneumoniae 168 in T B 190 asbestosis 417 \200\22318 aspergilloma (mycetoma) 196 aspergillosis 197 in cystic \201 brosis patients 237 in HIV patients 216 in immune compromised patients 206 in lung transplant recipients 423 antibiotics ageing, effect on lungs 16 agricultural workers, COPD 412 air travel see \202 ying air \202 ow 12 airway clearance in acute asthma 80 antituberculous 189 in aspiration pneumonia 179 in bronchiectasis 228 \200\2239 in community-acquired pneumonia 168 \200\2239 in COPD 102, 113 in cystic \201 b rosis chronic infections 238, 244 \200\2235 in HAP 174 \200\2235 in lung abscess 181 in lun g transplant recipients 428 prior to lung transplantation 426 Aspergillus disease, immune-mediated 196 aspiration 160, 176 \200\2238 aspiration pneumonia 177 \200\2239 aspiration of pneumothorax 335, 336 aspiration syndromes 177 \200\2238 aspir in sensitivity 64 asplenia, associated infections 205 assist-controlled ventilation (ACV) 4 38 asthma bronchiectasis 228 in cystic \201 brosis 238, 245 airway hyper-responsiveness 60 airway in \202 ammation, asthma 60 airway management 435 \200\22336 airway radius 12 airway remodeling, asthma 62 airways antibody therapy, in asthma 61 anticholinergics 70, 101, 102 anticoagulation acute 78 \200\22380 adherence with therapy 83 airway remodeling 62 antibody therapy 61 bron chial challenges 21 anatomy 2 effect of corticosteroids 71 pathological changes in COPD 92 in COPD exacerbations 113 in pulmonary embolism 262 INDEX 462 in connective tissue disorders 157 in extrinsic allergic alveolitis 149 in immune compromis ed patients 202 in pulmonary alveolar proteinosis 443 in sarcoidosis 153 in UIP 129 asthma (cont.) biopsy chronic management 82 \200\2237 Churg \200\223Strauss syndrome 446 \200\2237 de \201 nitio n and epidemiology 60 diagnosis 60 differential diagnosis 80, 84 disability assessment 395 distinction from COPD 80 diving 411 economic costs 82 effect of high altitude 409 eosinophi lic airway in \202 ammation in HIV patients 212 in immune compromised patients 203 in interstitial pneumonia 132 in lun g cancer 289, 290 in malignant pleural effusion 344 \200\2235 in mesothelioma 349 pleural 3 31 in sarcoidosis 155 transbronchial \201 ne needle aspiration 318 \200\22319 in UIP 129 bronchodilator response 21 bronchodilators anticholinergics 70 beta2-adrenoreceptor agonists 68 \200\2239 in COPD 101 \200\2232, 102 \200\2233 in COPD exacerbations 112 \200\22313 in cystic \201 brosis 245 effectiveness in asthma control 85 theophylline 69 \200\22370 biphasic positive airway pressure (BIPAP) 437 bird fancier \200\231s lung 148, 150 assessment 84 lung function decline 82 maternal deaths 76 occupational 83, 398 \200\223402 pathophysiolog y 60 \200\2232, 63 in pregnancy 76 \200\2237 relationship to allergic rhinitis 64 reliever and controller medication 68 risk factors for mortality 84 symptom control 83 symptoms 82 t riggering factors 83 asthma action plans 86, 87 asthma management 82, 83 see also extrinsic allergic alveolitis (EAA) bisphosphonates, in cystic \201 brosis 255 \200\2236 blastomycosis 198, 216 bleomycin, pn emotoxicity 164, 386 Blesovsky \200\231s disease (folded lung) 312, 415 blood, use in pleur odesis 338 blood cultures, HIV patients 211 blood \202 ukes (schistosomiasis) 199 blood g as abnormalities 19 blood pressure, effect of obstructive sleep bronchoscopy in haemoptysis 40, 41 HIV patients 212 interventional techniques 320 \200\2231 in investiga tion of wheeze 53 in patients with tracheal tumors 454 solitary pulmonary nodules 49 transb ronchial \201 ne needle aspiration 318 \200\2239 apnoea 364 buffering capacity 30 bullae 92 bullous emphysema, surgical treatment 116 \200\22317 buprop ion 100 Burkholderia cepacia complex infection, cystic blood supply to lung 2 BODE score 419 \200\22320 body mass index 99 body plethysmography 20 bone marrow transplantation 164, 204 \200\2235 anticholinergics 70 anti-IgE therapy 74 anti-leukotrienes 73 \200\2234 anti-tumor necrosis factor 75 beta2-adrenoreceptor agonists 68 \200\2239 during breastfeeding 77 corticosteroid s 70 \200\2232 cromones 73 cyclosporin A 75 gold 75 methotrexate 74 \200\2235 mucolytics 75 multidisciplinary approach 86 non-pharmacological 86 patient assessment 82 \200\2234 pharm acological management 84 \200\2236 during pregnancy 76 \200\2237 theophylline 69 \200\22371 atopy, Aspergillus sp. 196 atrial septostomy, in pulmonary \201 brosis 236 \200\2237, 243, 244, 422 viral infections 207 Buruli ulcer 194 busulphan, pneumotoxicity 164, 386 bone mineral density, reduction in cystic \201 brosis 255 \200\2236 bone morphogenetic protein type II (BMPR-II) C C- \201 bres 16 C-reactive protein (CRP) 167 calci \201 cation mutations 265 bone TB 192 brachytherapy 320 \200\2231 brain, imaging in lung cancer 290 brain metastases, radiotherapy 297 breastfeeding, asthma management 77 breathing 10 \200\22312 breathing res erve 25 breathing retraining 110 breathlessness 18, 38 \200\2239 metastatic 203 \200\2234 pleural plaques 416 solitary pulmonary nodules 49 calcifying \201 brous pseudotumour of pleura 310 calcineurin inhibitors, side effects 423 , 424 calcium channel blockers, in pulmonary assessment during pregnancy 76 disability assessment 394 in lung cancer 284 management in I PF 129 MRC dyspnoea scale 99, 106, 394 symptomatic relief 110 hypertension 267 cancer patients, pulmonary embolism 262 Candida infections 197, 207 hypertension 269 atypical cystic \201 brosis 232 augmentation therapy, AAT de \201 cienc y 122 autoantibody testing 53 autonomic control 15 avian in \202 uenza 185 azathioprine in IPF 130 in sarcoidosis 155 in HIV patients 216 candidate gene studies, COPD 91 cannabis, pneumotoxicity 385 Caplans syndrome 391 carbon di oxide elimination 13 British Everest Expedition 405 bromocriptine, pneumotoxicity 386 bronchial challenges 21 br onchial gland tumors 315 bronchial stents 321 bronchial tree 3 bronchiectasis in COPD 94 \200\2235 carbon dioxide inhalation, in Cheyne \200\223Stokes respiration 381 carbon dioxide retention, diving 408 carbon dioxide transportation 13 \200\22314 carbon mon oxide diffusing capacity azoospermia, cystic \201 brosis 256 azygos \201 ssure 2, 5, 7 in AAT de \201 ciency 120 airway clearance 228 antibiotic therapy 228 \200\2239 anti-in \202 ammatory therapy 229 causes 224 \200\2235 in ciliary dyskinesia 449 in connective tiss ue disorders 156 diagnosis 225 \200\2236 epidemiology 224 immunomodulatory therapy 229 in i n \202 ammatory bowel disease 161 microbiology 225 monitoring 227, 230 non-tuberculous myc obacterial (DLCO) 21 \200\2232 carcinoid 308 B B lymphocyte de \201 ciency, associated diagnosis 308 \200\2239 treatment 309 \200\22310 cardiac disease infections 205 in candidates for lung transplantation 419 pulmonary manifestations 164 bacillary angiomatosis 316 bakery workers, COPD 412 \200\22313 barotrauma 409 base de \201 cit 31 base excess 30 basophils, role in asthma 60 benign asbestos pleural effusion (BAPE) 414 benign endobronchial tumors 315 \200\22316 benign parenchymal tumors 312 \200\22315 be nign pleural tumors 312 beta2-adrenoreceptor agonists 68 \200\2239, 101 \200\2232 cardiac re-synchronization therapy (CRT) 381 cardiogenic pulmonary oedema, NIV and CPAP 375 cardiopulmonary exercise testing (CPET) 24 pre-operative 45 cardiovascular disease infections 230 pathophysiology 224 patient education 229 \200\22330 physiotherapy assessmen t 227 treatment aims and principles 228 association with obstructive sleep apnoea 364 \200\2235 in COPD 95, 103 carinal resection 296 carrier testing, cystic \201 b rosis 249 Castleman \200\231s disease (angiofolicular lymph node in acute asthma 79 in COPD exacerbations 112 \200\22313 effectiveness in asthma control 85 molecular mechanism of action 68 beta blockers, as cause of respiratory bronchiolitis 53 bronchiolitis obliterans organizing hyperplasia) 315 pneumonia see cryptogenic organizing pneumonia (COP) catamenial pneumothorax 337 cataplexy 383 cataracts, in COPD 103 caval \201 lters 262 CD1 antigen staining 450 disease 385 bevacizumab 288 bilevel positive airway pressure (BiPAP) 381 biological agents, use in NSCLC 298 bronchiolitis obliterans syndrome (BOS) 422, 424 \200\2235 bronchoalveolar cell carcinoma 166 bronchoalveolar lavage (BAL) INDEX 463 children management 168 \200\22360 non-resolving 170 prevention 170 \200\2232 severity assessment 16 8 time to stability 170 compliance of lungs 11 compliance with treatment 104 CD4+ T cells see helper T cells cellular pathology, COPD 92 \200\2233 central apnoea 362 ce ntral nervous system (CNS), TB in \202 uenza-related deaths 186 obstructive sleep apnoea 363, 369 viral infections 184 chlorambucil, pneumotoxicity 386 chondroid hamartoma 313 chronic asthma management 82 \200 \2237 chronic hypoxaemia, as indication for involvement 189 central pattern generator (CPG) 15 central sleep apnoea 377 \200\2238 ambulatory oxygen therapy 126 cystic \201 brosis patients 246 see also Cheyne \200\223Stokes respiration LTOT 124 \200\2235 cepacia syndrome, cystic \201 brosis 236 \200\2237, 243 cerebral metastases chronic obstructive pulmonary disease (COPD) computed tomography alpha1-antitrypsin de \201 ciency 120 \200\2232 co-morbidities 95, 103 cor pulmonale 103 d isability assessment 106 \200\2237 distinction from asthma 80 effect of high altitude 407 e pidemiology 90 exacerbations 95, 96 genetic factors 90 \200\2231 HIV patients 220 investiga tions 99 \200\223100 nocturnal hypoventilation 376 nutritional status 418 occupational caus es 414 \200\22315 opportunistic mycobacterial infection 194 \200\2235 overlap syndrome 372 \200\2233 pathological changes 92 \200\2233, 116 physiological abnormalities 93 \200\2235, 116 pulmonary hypertension 269 severity classi \201 cation 100, 101 systemic effects 95 acute interstitial pneumonia 140 artifacts 7, 8 asbestosis 415, 416 assessment 8 bronchiect asis 226 of cerebral metastases 290 Churg \200\223Strauss syndrome 446 ciliary dyskinesia 4 49 cryptogenic organizing pneumonia 144 \200\2235 COPD 98 desquamative interstitial pneumon ia 138 extrinsic allergic alveolitis 149 empyema 355 haemoptysis 40 HIV patients 211 immune compromised patients 202 in investigation of wheeze 53 lymphoid interstitial pneumonia 142 \200\2233 lung abscess 180 lung cancer 288 \200\2239, 290 lung cancer screening 306 \200 \2237 malignant pleural effusions 344 mesothelioma 349 normal appearances 5 \200\2238 NSIP 132 organizing pneumonia 166 pleural effusion 54, 55, 330 \200\2231 pleural thickening 416 \200\22317 pulmonary alveolar proteinosis 443 pulmonary embolus 43, 261 pulmonary hypertens ion 266 RBILD 136 sarcoidosis 153 solitary pulmonary nodules 48 \200\2239 superior vena cav a obstruction 285, 286 techniques 5 UIP 128, 129 imaging 290 radiotherapy 297, 301 cerebral oedema, high altitude-related 406, 407 chaperone therapy, AAT de \201 ciency 122 chemical pleurodesis 338 in malignant pleural effusions 345 chemical pneumonitis 177 chemokines, role in asthma 62 chemoradiotherapy, NSCLC 297, 298 ch emoreceptors 16 chemotherapy in carcinoid 309 in mesothelioma 350 in non-small cell lung cancer 297 \200\223298 in small cell lung cancer 300 \200\2231 chest drains 54, 335 \200\2236 in pleural infection 356 chest pain 42 \200\2233 in lung cancer 284 chest wall pain differential diagnosis 42 investigation 43 chronic obstructive pulmonary disease management chest wall restriction, nocturnal assessment of progress 103 commonly used drugs 101 dietary supplementation 109 dyspnoea imp rovement 110 exacerbations 103, 112 \200\22314 lung transplantation 419 \200\22320 mechanic al ventilation 434 non-invasive ventilation 375 pharmacological therapy 101 \200\2233 pulmo nary rehabilitation 107 \200\2239 self-management 110 smoking cessation 100 \200\2231, 102 of stable disease 100 surgical treatment 116 \200\22318 transcutaneous electrical muscle hypoventilation 376 chest X-rays in AAT de \201 ciency 121 in acute asthma 79 in asbestosis 417 aspiration 160 in bronchiec tasis 225 bronchoalveolar cell carcinoma 166 in community acquired pneumonia 167 in cryptog enic organizing pneumonia 144 in COPD 98 in cystic \201 brosis exacerbations 236, 237 in desquamative interstitial pneumonia 138 in extrinsic allergic alveolitis 148 in empyema 355 in HIV patients 211, 212 in immune compromised patients 202 in investigation of wheeze 53 in lymphoid interstitial pneumonia 142 in lung abscess 180 lung cancer screening 306 malign ant pleural effusions 344 mesothelioma 43 in mesothelioma 349 nocardiosis 182 normal appear ance 4 \200\2235 in NSIP 132 of Pancoast tumor 285 per-operative 44 in pleural effusion 54 \200\2235, 330 pleural plaques 416 in pneumoconiosis 392, 393 in pulmonary alveolar protein osis 442 in pulmonary hypertension 266 in RBILD 136 in sarcoidosis 153, 154 in SARS 432 sig ns of respiratory muscle weakness 33 solitary pulmonary nodules 48 \200\2239 in TB 188, 189 technical factors 4 in UIP 128 in Wegener \200\231s granulomatosis 273 congestive cardiac failure, pleural stimulation 109 effusions 342, 343 walking aids 109 conjunctiva, sarcoid nodule 152 connective tissue disorders 156 chronic pulmonary aspergillosis 197 chronic thromboembolic pulmonary hypertension (CTEPH) 2 69 \200\22370 association with lymphoid interstitial pneumonia 142 investigations 156 management principles 157 pleural effusion 352 \200\2233 Churg \200\223Strauss syndrome (CSS) 272, 275, 276, 446 \200\2237 chylothorax 330 cilia, ultrastructure 448 ciliary dyskinesia 450 \200\22328 cirrhosis consolidation 166, 167 consolidation thoracic irradiation, SCLC 301 constant work rate (CWR ) protocols 24 constipation, in cystic \201 brosis 253 contact tracing, TB 191 continuous hyperfractionated accelerated in AAT de \201 ciency 120, 121 pleural effusions 342 clamping of chest drains 336 clear cell tumor 313 clindamycin 178 clinical probability scor ing, PE 260 \200\2231 clubbing, in lung cancer 286 co-trimoxazole, in AASV 276 coal dust ex posure, association with radiotherapy (CHART) 297 continuous positive airway pressure (CPAP) 437 in acute setting 374 \200\2235, 434 \200\2235 in Cheyne \200\223Stokes respiration 381 mech anisms of action 374 in obstructive sleep apnoea 367 \200\2239, 370 in overlap syndrome 372 \200\2233 COPD 412 Coal Miners \200\231 Respiratory Disability Scale 394 coal workers pneumoconiosis 390 \200 \2231 cocaine, pneumotoxicity 387 coccidioidomycosis 188, 216 collagen vascular disease see connective tissue controlled mechanical ventilation (CMV) 436 cor pulmonale 103 in obstructive sleep apnoea 365 coronary artery bypass grafting (CABG), disorders pleural effusion 352 combination antibiotic therapy, HAP 174 combination inhalers 69, 101, 102 combination thera py, asthma 85 community-acquired pneumonia (CAP) coronavirus infections, clinical characteristics 185 Cheyne \200\223Stokes respiration 377 corticosteroids at altitude 404 investigations 380 pathophysiology 380 treatment 381 in asthma 61, 80, 70 \200\2232, 85 in community-acquired pneumonia 169 in connective tissue disorders 157 in cryptogenic organizing pneumonia 145 in COPD 101, 102 clinical features 166 discharge from hospital 170 epidemiology 166 investigations 167 chickenpox, cystic \201 brosis patients 247 INDEX 464 bleomycin 386 bromocriptine 386 busulphan 386 cannabis 380 chlorambucil 386 cocaine 387 cyc lophosphamide 385 diagnosis 384 disease-modifying anti-rheumatic drugs 385 interstitial dis ease 384 lupus pleuritis 384 \200\2235 methotrexate 386 methysergide 386 mitomycin 387 \200 \2237 nitrofurantoin 385 \200\2236 pleural effusion 341 pulmonary arterial hypertension 385 taxanes 387 TNF blockers 387 duro-pleural \201 stula 330 DVLA corticosteroids (cont.) delayed pulmonary toxicity/restrictive in COPD exacerbations 113 in cystic \201 brosis 245 in idiopathic pulmonary \201 brosis 130 in lymphoid interstitial pneumonia 143 in sarcoidosis 154 \200\2235 side effects 72 su ppression of in \202 ammation 71 cotton textile workers, COPD 412 cough 36 \200\2237 syndrome 205 delayed sleep phase syndrome 383 desensitization to allergens 66 desquamative interstitial pneumonia (DIP) 138 \200\2239 diabetes mellitus cystic \201 brosis-related 246, 252 \200\2233 pulmonary manifestations 162 TB treatment 1 89 in idiopathic pulmonary \201 brosis 129 \200\22330 in lung cancer 284 cough pressures 34 cough re \202 ex sensitivity 34 cranial irradiation in NSCLC 292 in SCLC 301 diabetic control, cystic \201 brosis exacerbations 237, 238 diaphragm palsy, associated with connective tissue disorders 158 diaphragms normal chest X-ray appearance 4 paralysis 38 weakness 32, 33 critical care, referral in acute asthma 80 Crohn \200\231s disease 161 cromones 73 cross-in fection control, cystic \201 brosis 243 \200\2234 cryotherapy 320 Cryptococcus neoformans 197, 207 diathermy 320 diet, role in lung cancer 283 dietary supplementation, COPD 109 diffuse alveo lar damage (DAD), in AIP 140 diffuse alveolar hemorrhage (DAH) 277 \200\22378 Medical Standards of Fitness to Drive 370 noti \201 cation of sleep disorders 371 dynamic hyperin \202 ation 20, 25, 94 dysfunctional breathing 38, 39 dyspnoea see breathle ssness dysproteinaemias, association with LIP 142 in HIV patients 216 after HSCT 205 cryptogenic \201 brosing alveolitis (CFA) 133 cryptogenic organizing pneumonia diffuse alveolar-septal amyloidosis 460 diffuse parenchymal lung disease (DPLD), (COP) 144 \200\2235, 205 generic algorithm 390 fulminant disease 141, 145 in RA 157 diffuse pleural thickening 416 \200\22317 diffuse pulmonary in \201 ltration, differential E earlobe, arterialised blood sampling 28 Early Lung Cancer Action Program CURB65 score 168 Cushing \200\231s syndrome 286 diagnosis 153 diffusing capacity for carbon monoxide in carcinoid 308 cyclophosphamide in AASV 276, 277 in idiopathic pulmonary \201 brosis 13 0 in non-speci \201 c interstitial pneumonia 133 pneumotoxicity 387 (DLCO) 21 \200\2232 Diro \201 laria immitis 199 disability, relationship to lung function 395 \200\2236 disability assessment (ELCAP) 305, 307 Echinococcus sp. 198 echocardiography in COPD 99 in pulmonary hypertension 266 \200\2237 asthma 395 COPD 106 \200\2237 exercise tests 395 lung function 394 \200\2235 symptoms 394 cyclosporin A, in asthma 75 cystic \201 brosis 53, 248 elderly people, viral infections 183 electrophysiological studies, myasthenia abdominal pain 253 \200\2234 bone mineral density reduction 255 \200\2236 CFTR gene mutatio ns 248 diagnosis 232 \200\2233, 248 \200\2239 distal intestinal obstructive syndrome gravis 57 disease-modifying anti-rheumatic drugs, eletrocardiography in COPD 99 in hyperventilation 39 preoperative 44 in pulmonary hypertens ion 266 pneumotoxicity 385 disease-modifying therapy COPD exacerbations 112 \200\22313 idiopathic pulmonary \201 brosis 130 disease-targeted t herapies, pulmonary (DIOS) 254 fertility 256 genetic advice 249 liver disease 254 \200\2235 meconium ileus 254 natural his tory 249 neonatal screening 250 nutritional status 418 pancreatic problems 252 \200\2233 pr egnancy 249 \200\22350, 256 \200\2237 prenatal diagnosis 249 specialist care 242 survival 2 42, 239 upper airways 256 embolisation hypertension 268 \200\2239 in arteriovenous malformation 457 in haemoptysis 41 distal intestinal obstructive syndrome (DIOS) 254 emphysema distractive stimuli, value in dyspnoea 110 diuretic therapy, pulmonary hypertension 267 div ing 408 in desquamative interstitial pneumonia 138 HRCT appearance 98 pathenogenetic mechanisms 120 pathology 92 surgical 336 surgical treatment 116 \200\22318 see also chronic obstructive p ulmonary barotrauma 409 carbon dioxide retention 409 decompression illness 408 \200\2239 in heredita ry haemorrhagic telangiectasia 457 disease (COPD) immersion pulmonary oedema 409 long-term pulmonary effects 410 after pneumothorax 338 pulmo nary oxygen toxicity 408 and respiratory disorders 409 saltwater aspiration syndrome 409 empirical antibiotic treatment, in HAP 174 \200\2235 empyema 331 cystic \201 brosis lung disease 246 \200\2237 haemoptysis 239 infective exacerbations 236 \200\22340 lung transplantation 418, 420, 422, 426 management 242 \200\2233 microbiology 243 pathology 242 physiological features 242 pneu mothorax 239 treatment 243 \200\2236 diagnosis 355 surgery 358 end-expiratory lung volume (EELV) 10 endemic mycoses 197, 198 dog heartworm 199 dopamine agonists, in periodic leg movements in HIV patients 216 endobronchial resection 320 endobronchial tumors, benign 315 \200\22316 endobronchial ultra sound 289 Endobronchial Valve for Emphysema Palliation in sleep 382 dorsal respiratory group (DRG) 15 doubling time (Td), solitary pulmonary cystic \201 brosis-related diabetes mellitus nodules 49 Trial (VENT) 118 (CF-RD) 246, 252 \200\2233 doxapram, in COPD exacerbations 113 doxycycline, use in pleurodesis 344 drainage, lung absc esses 181 driving 370 \200\2231 endocrine diseases, pulmonary cysts, in lymphangioleiomyomatosis 452 cytokines, role in asthma 61 cytomegalovirus (CMV) i nfection 207 manifestations 162 \200\2233 endoglin gene 457 endometriosis in HIV patients 216 in lung transplant recipients 423, 428 and obstructive sleep apnoea 364 catamenial pneumothorax 337 thoracic 315 drug interactions, after lung transplantation 427 endometritis, TB 193 endoscopic lung volume reduction 118 endothelin receptor antagonist th erapy, drug susceptibilities, opportunist D D-dimer test 261, 263 decompression illness 408 \200\2239 decongestants 65 dehydration, r isk at high altitudes 404 mycobacteria 194 drug-induced lung disease pulmonary hypertension 268 ACE inhibitors 385 amiodarone 385 \200\2236 ARDS 385 beta blockers 385 endotracheal tube management 435 \200\2236 endurance training 107 engraftment syndrome 205 INDEX 465 Entamoeba histolytica 199 environmental exposures, role in COPD 90 eosinophilic airway in \202 ammation assessment, folded lung (Blesovsky \200\231s disease) 312, 415 food allergy, relationship to pollen sen sitivity 66 forced vital capacity (FVC) 19, 22 foreign body aspiration 177, 178 functional assessment, COPD 99 functional residual capacity (FRC) 10, 20 fungal diseases health care associated pneumonia (HCAP) 172 health status assessment 106 heart disease, car cinoid 308 heart failure asthma 84 eosinophilic bronchitis 61 eosinophilic pleural \202 uid 330 eosinophils, role in asthma 61 EPHX (Microsomal Epoxide Hydrolase) Cheyne \200\223Stokes respiration 380 \200\2231 sleep disordered breathing 378 treatment 38 1 Aspergillus 196 \200\2237 in cystic \201 brosis patients 237, 243 endemic mycoses 197, 19 9 in HIV patients 216 in immune compromised patients 206 \200\2237 in lung transplant recip ients 423 molds 197 \200\2238 Pneumocystis jeroveci 197 treatment 198 yeasts 197 fusariosis 197 \200\2238 heart lung transplantation, in pulmonary gene 91 hypertension 269 heart sarcoidosis 152 Heerfordt syndrome 152 heliox, in acute asthma 80 he lper T cells, role in asthma 61 heparin see anticoagulation hepatic metastases, management in epidermal growth factor receptor (EGFR) antagonists 298 epithelioid haemangioendothelioma 313 Epstein Barr virus infection, lung transplant recipients 423 Epworth Sleepiness Scale 365 equal pressure point (EPP) 12 erlotinib (Tarceva) 298 ethambut ol resistance 190 exacerbations of asthma 78 \200\22380, 82 carcinoid 309 \200\22310 hepatocyte transplantation, AAT de \201 ciency 122 hepatopulmonary syndrome 161, 203 hereditary haemorrhagic telangiectasia assessment of risk 84 during pregnancy 76 role of eosinophils 61 exacerbations of COPD inve stigations 112 management 103, 112 \200\22314 pathophysiology 95, 96 prevention 103, 114 G galactomannan test 197 gas diffusion 14 gas exchange 13 \200\22314 (HHT) 456 \200\2237 Hering \200\223Breuer re \202 ex 16 herpes simplex virus infections, immune compromised patients 207 HHT3, HHT4 genes 457 HHV-6 207 high altitude 404 abnormalities in COPD 94 effect of high altitude 407 gastroesophageal re \202 ux (GOR) 160 exacerbations of cystic \201 brosis 236 \200\2237 diseases related to altitude 406 effect on pre-existing lung disease 407 effects on normal lung 404 \200\2235 cystic \201 brosis patients 246 investigations 37 management ladder 37 pathophysiology 36 role in BOS 424, 437 diagnosis 237 \200\2238 failure to improve 239 \200\22340 treatment 238 \200\2239 high frequency oscillatory ventilation (HFOV) 437 high resolution computed tomography exacerbations of IPF 140 \200\2231 exacerbations of NSIP 141 exercise testing 24 \200\2235, 39, 106 \200\2237 (HRCT) 5 gastrogra \201 n 254 gastrointestinal disease in acute interstitial pneumonia 140 in asbestosis 415, 416 in bronchiectasis 226 in Churg \200\223Strauss syndrome 446 in ciliary dyskinesia 449 in connective tissue disorders 156 i n cryptogenic organizing pneumonia 144 \200\2235 in COPD 98 in desqumative interstitial pne umonia 138 in extrinsic allergic alveolitis 149 in HIV patients 211 in lymphoid interstitia l pneumonia 142 \200\2233 in NSIP 132 organizing pneumonia 166 in RBILD 136 in sarcoidosis 153 in UIP 128, 129 disability assessment 397 in pulmonary hypertension 266 exercise therapy, cystic \201 bro sis 245 exhalation 11, 32 exhaled nitric oxide measurement 84 expiratory reserve volume (ER V) 20 extensive stage small cell lung cancer 300 extra-corporeal membrane oxygenation causes of pleural effusion 352 in lung transplant recipients 424 pulmonary manifestations 1 60 \200\2231 gastrointestinal TB 192 \200\2233 ge \201 tinib (Iressa) 298 gene mutations, hereditary ha emorrhagic telangiectasia 457 genetic advice, cystic \201 brosis 249 genetic factors \215 COPD 90 \200\2231 in lung can cer 283 in obstructive sleep apnoea 365 (ECMO) 439 extrapleural pneumonectomy (EPP) 359 extrinsic allergic alveolitis (EAA) 196 classi \201 c ation and course 148 \200\2239 epidemiology 148 investigation and diagnosis 149 \200\22350 treatment 150 Geneva Score, revised 261 giant cell arteritis 272 goiter 162 gold exudative pleural effusion 329, 340 \200\2231 differentiation from transudates 329, 340, 342 hilar lymphadenopathy 143, 152 pneumotoxicity 387 use in asthma 75 eye, sarcoidosis 152 in amyloidosis 458 differential diagnosis 153, 155 Goodpasture \200\231s syndrome 278 granular cell tumors 315 \200\22316 granulocyte monocyte colony stimulating histoplasmosis 198, 216 HIV patients 218 alveolitis 220 assessment 210 \200\22311 bacterial pneumonia 213 causes of pulmonary infections 210 clinical features of pulmonary infection 211 COPD 220 fungal infections 216 immune reconstitution in \202 ammatory syndrome (IRIS) 213, 219 \200\22320 F fecal impaction, cystic \201 brosis 253 farm workers, COPD 412 farmer \200\231s lung 14 8, 150, 196 factor (GMCSF) role in PAP 442 in treatment of PAP 444 granulomata see also extrinsic allergic alveolitis (EAA) 18FDG 302 fertility differential diagnosis 153 in sarcoidosis 154 ground glass appearance, HRCT 132, 138, in cystic \201 brosis 256 in primary ciliary dyskinesia 449 140, 142 GST (Glutathione S Transferase) gene 91 guided TBNA 319 Guillain \200\223Barr syndrome, c linical case 57 FEV1 19, 53 FEV1/VC versus FEV1/FVC 22 \201 brinolytics, in pleural infections 356 \201 broleiomyomas 313 \201 brosing organizing pneumonia 145 \200\2236 \201 brous tumors, p leura 312 \201 ne needle aspiration 289, 318 \200\22320 \201 ngers, sarcoid lesions 152 \201 ssures 2 \201 tness to dive 409 \202 atworms 198 \200\2239 \202 our millers, COPD 412 \200\22313 \202 ow limitation, COPD 94 \202 ow \200\223volume loops 19, 53 \202 ow \200\223volume relationships 12 \202 ying 407 investigations 211 \200\22312 Kaposi sarcoma 218 lung cancer 219 lymphoid interstitial pneu monia 142, 143 lymphoma and lymphomatous H HAART, in patients with tuberculosis 215 \200\22316 hemoglobin dissociation curve 30 haem atopoietic stem cell transplantation