European Respiratory Society
ERS Handbook of Respiratory Medicine

The ERS Handbook of Respiratory Medicine, now in its second edition, is a concise, compact and easy-to-read guide to each of the key areas in respiratory medicine. Its 18 chapters, written by clinicians and researchers at the forefront of the field, explain the structure and function of the respiratory system, its disorders and how to treat them.

  • European Respiratory Society
  1. Page xxix
  2. Page xxxi
    1. Page 1
      Abstract
      • • 

        A few respiratory diseases, such as CF and α1-Pi deficiency, are single-gene conditions.

      • • 

        A large range of respiratory diseases, including asthma, COPD, sarcoidosis, IPF and primary pulmonary hypertension, may have a genetic background.

      • • 

        Non-harmful gene variants can nonetheless confer susceptibility to conditions such as chronic beryllium disease.

      • • 

        The role of epigenetic regulatory mechanisms in respiratory disease is likely to be very significant.

    2. Page 7
      Abstract

      Major features of lung diseases are:

      • • 

        altered deposition of extracellular matrix,

      • • 

        impaired surfactant metabolism,

      • • 

        Distorted endogenous defence mechanisms.

    3. Page 13
      Abstract
      • • 

        The anatomy of the thorax can be divided broadly into the pleura, lungs, mediastinum, diaphragm and heart.

      • • 

        The lungs can be further subdivided into lobes, segments, trachea and bronchi.

      • • 

        The mediastinal space contains structures including the thymus gland, thoracic lymph nodes, thoracic duct, vagus nerve and autonomic nerve plexus.

      • • 

        The thoracic structures include the vital organs for respiration and circulation. This section will focus on the pleura, lungs, mediastinum and diaphragm. The anatomy of the heart is not discussed.

    4. Page 18
      Abstract
      • • 

        The mechanical work of breathing comprises elastic (volume-related) and resistive (flow-related) components.

      • • 

        With expiratory efforts causing PIP to become positive, an EPP is created that results in expiratory flow limitation.

      • • 

        Arterial hypoxaemia can result from alveolar hypoventilation, diffusion limitation, VA/Q′ mismatch and/or right-to-left shunt. Only the latter three mechanisms also lead to a widened PA–aO2 (i.e. inefficient pulmonary oxygen exchange).

    5. Page 29
      Abstract
      • • 

        BAL is an important source of cytological samples.

      • • 

        Fine-needle aspiration has increased the impact of cytological diagnoses.

      • • 

        Cell blocks are easy to prepare and useful for immunocytochemistry.

      • • 

        Reactive cytological features in respiratory samples can be characteristic but nonspecific.

      • • 

        Cytology can be used to diagnose respiratory infections.

      • • 

        Lung carcinoma presents a variety of characteristic patterns.

      • • 

        Lymphoproliferative disorders are more readily diagnosed in BAL fluid or fine-needle aspirates.

      • • 

        Immunocytochemistry and molecular biology add to cytological diagnoses.

    6. Page 39
      Abstract
      • • 

        The respiratory system is exposed to a variety of microbiological, physical and chemical insults through inhaled air.

      • • 

        Innate, intrinsic and adaptive, acquired host immune defences cooperate in lowering the risk of damage to respiratory structures in an integrated host defence system.

      • • 

        In disease states, one or more of these complex mechanisms can be impaired and/or dysfunctional.

  3. Page 45
    1. Page 45
      Abstract
      • • 

        Cough is characterised by irritant receptor hypersensitivity.

      • • 

        Nonacid reflux into the airways frequently precipitates cough.

      • • 

        Clinical history followed by therapeutic trials is the management strategy of choice.

    2. Page 51
      Abstract
      • • 

        Dyspnoea is a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity.

      • • 

        The mechanisms of dyspnoea are complex and multifactorial: there is no unique central or peripheral source of this symptom.

      • • 

        The sense of heightened inspiratory effort is an integral component of exertional dyspnoea and is pervasive across health and disease.

      • • 

        The NVD theory of dyspnoea states that the symptom arises when there is a disparity between the central reflex drive (efferent discharge) and the simultaneous afferent feedback from a multitude of peripheral sensory receptors throughout the respiratory system. The feedback system provides information about the extent and appropriateness of the mechanical response to central drive.

      • • 

        Despite the diversity of causes, the similarity of described experiences of dyspnoea suggests common underlying mechanisms.

    3. Page 59
      Abstract
      • • 

        Chest pain can be a feature of a wide range of pathology.

      • • 

        An accurate history is essential to direct appropriate investigation of patients presenting with chest pain.

    4. Page 61
      Abstract
      • • 

        It is essential to bear in mind that breathlessness can have a variety of causes.

      • • 

        Physical examination should follow the taking of the medical history and differential diagnoses, and is an opportunity to confirm normality or discover abnormality.

      • • 

        Physical examination comprises inspection, palpation, auscultation and percussion.

      • • 

        The respiratory physician must not forget that disease of other systems may also be the cause of the symptoms and that comorbidity is common.

  4. Page 66
    1. Page 66
      Abstract

      Measurement of lung volumes in clinical practice has been proven to be important to assist in the following:

      • • 

        Diagnosis of pulmonary defects,

      • • 

        Evaluation of candidates for lung volume resection surgery,

      • • 

        Prognosis of COPD and interstitial lung diseases,

      • • 

        Evaluation of the bronchomotor response to constrictor and dilator agents as well as to physical exercise.

    2. Page 72
      Abstract
      • • 

        Palv is lower and higher than Pao during inspiration and expiration, respectively.

      • • 

        The lungs exert inward elastic recoil that increases with VL.

      • • 

        Body plethysmography allows the measurement of both Raw and VL.

      • • 

        The FOT allows the measurement of respiratory resistance during spontaneous breathing with minimum patient collaboration.

      • • 

        Respiratory mechanics can be monitored in sedated mechanically ventilated patients performing post-inspiratory and post-expiratory pauses.

    3. Page 77
      Abstract
      • • 

        TLCO measures alveolar function.

      • • 

        TLCO is the product of KCO and VA.

      • • 

        KCO (or TL/VA) is the more specific index of alveolar integrity.

      • • 

        KCO is low in emphysema and fibrosis.

      • • 

        KCO is high in extrapulmonary restriction.

    4. Page 82
      Abstract
      • • 

        Ventilatory carbon dioxide responsiveness is determined as the slope of the linear iso-oxic V′EPETCO2 relationship (ΔV′EPETCO2), using steady-state, constant-concentration inspirates or hyperoxic rebreathing. ΔV′EPETCO2 reflects central and, if PaO2 is not excessive, also carotid chemoreceptor activity. Being appreciably shorter, the latter test is preferred, although ΔV′EPETCO2 reflects only central chemoreflex activity.

      • • 

        Ventilatory hypoxic responsiveness is determined from the curvilinear isocapnic V′EPETO2 response, using steady-state, constant-concentration inspirates or rebreathing. It reflects solely carotid chemoreceptor activity. Expressing V′E versus SaO2 linearises the profile, with the slope (ΔV′ESaO2) providing the hypoxic responsiveness index (however, PaO2, not SaO2, is the actual stimulus). This can also be estimated using the Dejours hypoxia-withdrawal test: abrupt oxygen administration from a prior hypoxic background acutely suppresses carotid-body activity to cause a transient, rapid V′E decline; the maximum decrease as a fraction of the total hypoxic V′E providing the hypoxic index.

    5. Page 87
      Abstract
      • • 

        ABG is mandatory for the diagnosis of respiratory failure and of A–B disorders.

      • • 

        Pulmonary gas exchange status is best evaluated by the integrated reading of PaO2 and PaCO2.

      • • 

        A–B status is best evaluated by the integrated reading of PaCO2 and pH, with concomitant measurement of serum electrolytes.

      • • 

        Mixed A–B disorders are very common in clinical practice.

      • • 

        The correct interpolation of ABG represents a fundamental step for the diagnosis and treatment of A–B disorders.

      • • 

        The study of serum chloride is fundamental to further investigate the causes of metabolic disorders affecting A–B equilibrium.

    6. Page 94
      Abstract

      CPET is considered the gold standard for:

      • • 

        an objective measure of exercise capacity,

      • • 

        identifying the mechanisms limiting exercise intolerance,

      • • 

        establishing indices of the patient's prognosis,

      • • 

        evaluating the effects of therapeutic interventions.

    7. Page 99
      Abstract
      • • 

        Bronchial challenge with methacholine/histamine is a sensitive measure of asthma but lacks specificity.

      • • 

        Indirect measures of bronchial responsiveness (exercise, inhaled AMP, hypertonic saline and mannitol, and EVH) are specific, but not sensitive, measures of asthma.

      • • 

        Indirect measures of bronchial responsiveness (exercise, etc.) respond rapidly (1–3 weeks) to inhaled steroids.

      • • 

        Direct measures of bronchial responsiveness (methacholine and histamine) respond slowly to inhaled steroids (>3 months).

      • • 

        Direct measures of bronchial responsiveness (methacholine and histamine) are presently the most exact monitoring tool for asthma.

      • • 

        BHR may predict later active asthma.

    8. Page 103
      Abstract
      • • 

        Sputum and exhaled breath analysis are useful noninvasive tools to appraise airway inflammation, particularly in a longitudinal sense.

      • • 

        Eosinophils are the most significant sputum biomarkers for the evaluation of airway inflammation.

      • • 

        Many inflammatory mediators can be measured in the fluid phase of sputum but their usefulness remains at research level.

      • • 

        Nitric oxide is the most reliable exhaled biomarker to assess eosinophilic airway inflammation. Other exhaled biomarkers need further validation and a clear demonstration of their utility in the diagnosis and/or follow-up of airway diseases.

  5. Page 109
    1. Page 109
      Abstract
      • • 

        Bronchoscopy provides diagnostic information in suspected lung cancer and diffuse lung disease, and in patients with persistent infection or local pulmonary infiltrates.

      • • 

        Bronchoscopy also has therapeutic uses in tumour treatment, and more recently in asthma and emphysema.

    2. Page 114
      Abstract
      • • 

        BAL is used to sample immune and inflammatory cells and many other components from the peripheral air spaces of the lungs in health and disease.

      • • 

        BAL is mainly used in research and to assist in the clinical diagnosis of ILDs or lower respiratory tract infections.

      • • 

        BAL findings must be interpreted in conjunction with results from clinical, pathological and radiological investigations.

      • • 

        A standardised procedure must be followed.

    3. Page 122
      Abstract
      • • 

        PFNB is indicated when a cytohistological diagnosis of a peripheral lung lesion is required.

      • • 

        PFNB may also be indicated for diagnosis of mediastinal mass and expansive lesions of the pleura and chest wall.

      • • 

        The most common guidance system for PFNB is CT; biplane fluoroscopy and ultrasound can also be used.

      • • 

        The sensitivity of PFNB for lung cancer is 85–95%.

      • • 

        The most frequently reported complication is minor pneumothorax (25%).

    4. Page 124
      Abstract
      • • 

        MT/P has the advantage compared with VATS that it can be performed under local anaesthesia or conscious sedation, in an endoscopy suite using non-disposable rigid (or semi-rigid) instruments. Thus, it is considerably less expensive.

      • • 

        The leading indications for MT/P are pleural effusions, both for diagnosis – mainly in exudates of unknown aetiology – or for staging in diffuse malignant mesothelioma, lung cancer and for talc poudrage, the best conservative method today for pleurodesis.

      • • 

        MT/P can also be used efficiently in the management of early empyema and pneumothorax.

      • • 

        In the above indications, MT/P can replace most surgical interventions, which are more invasive and more expensive.

      • • 

        MT/P is a safe procedure, even easier to learn than flexible bronchoscopy, provided sufficient experience with chest-tube placement has been gained.

      • • 

        MT/P as part of the new field of interventional pulmonology should be included in the training programme of chest physicians.

    5. Page 128
      Abstract
      • • 

        Thoracentesis may be diagnostic or therapeutic in patients with a pleural effusion.

      • • 

        Ultrasound examination is valuable in guiding the procedure.

      • • 

        There are no absolute contraindications, and complications are rare, but the possibility should be taken into account.

    6. Page 131
      Abstract
      • • 

        Symptoms of central airway stenosis occur late, after ⩾50% (on exercise) or 80% (at rest) of the tracheal lumen is obstructed.

      • • 

        The diagnostic accuracy of spirometric indices and visual flow–volume loop criteria in detecting central airway stenosis is relatively poor.

      • • 

        Interventional bronchoscopic techniques have been shown to significantly improve objective pulmonary function and quality of life.

  6. Page 136
    1. Page 136
      Abstract
      • • 

        Chest radiography is the first step in radiological diagnosis of chest diseases.

      • • 

        Although it is a common technique, achieving high image quality is challenging and depends on getting several factors right.

      • • 

        The move from film to digital imaging offers exciting opportunities to improve image consistency and data management.

    2. Page 141
      Abstract
      • • 

        CT is the second most important imaging modality of the chest.

      • • 

        CT diagnosis of lung diseases is based on the study of their appearance and distribution patterns together with a careful analysis of patient data.

      • • 

        CT interpretation of diffuse and interstitial lung diseases requires a formal multidisciplinary approach.

      • • 

        MRI is second to CT when it comes to visualising pulmonary structure and pathology.

    3. Page 146
      Abstract
      • • 

        HRCT is the imaging technique that offers the highest image detail of the lung parenchyma.

      • • 

        HRCT is predominantly used to study DILDs but thin-slice CT can be useful in the study of focal lung abnormalities too.

      • • 

        HRCT of the lungs is an essential element in the multidisciplinary discussion of patients with DILD.

      • • 

        HRCT does not replace lung biopsy but helps to decide in which cases a lung biopsy will very likely give more (or important additional) information than CT and in which cases a biopsy is not needed.

    4. Page 151
      Abstract
      • • 

        Nuclear medicine of the lung has a role in the diagnosis of pulmonary embolism and inflammatory diseases, and in the diagnosis and staging of lung cancer.

      • • 

        Perfusion scintigraphy is key in the diagnosis and follow-up of pulmonary embolism as it is safe, cheap and noninvasive.

      • • 

        Gallium-67 scintigraphy is useful in identifying and localising intrathoracic inflammation and infection.

      • • 

        FDG-PET and PET/CT are used in diagnosis, treatment targeting and treatment in lung cancer.

    5. Page 154
      Abstract
      • • 

        Transthoracic ultrasound can be performed with the most basic ultrasound equipment and allows for immediate and mobile assessment of patients with a wide variety of respiratory diseases.

      • • 

        The major indications for the use of transthoracic ultrasound are the description of pleural effusions, pleural thickening, diaphragmatic dysfunction, and chest wall and pleural tumours.

      • • 

        Other applications of transthoracic ultrasound include the diagnosis of a pneumothorax, pulmonary consolidation, tumours, interstitial syndromes and pulmonary embolism.

      • • 

        Furthermore, ultrasound is ideal to guide thoracentesis, drainage of effusions and other thoracic interventions, and is particularly useful in intensive care units where radiographic equipment is unavailable.

      • • 

        Major advantages of the technique include its mobility, dynamic properties, lack of radiation and low cost.

      • • 

        The ultrasonographic appearance of the normal thorax and the most common pathologies are reviewed in this section.

  7. Page 159
    1. Page 159
      Abstract
      • • 

        ALI and its most severe manifestation, ARDS, are defined as PaO2/FIO2 ⩽300 mmHg and ⩽200 mmHg, respectively, with bilateral infiltrates as radiological criteria. The ARDS Definition Task Force proposes a new classification according to the severity of ARDS, i.e. mild: PaO2/FIO2 >200 mmHg and ⩽300 mmHg; moderate: PaO2/FIO2 >100 mmHg and ⩽200 mmHg; and severe: PaO2/FIO2 ⩽100 mmHg, because of its better predictive value for mortality (fig. 1).

      • • 

        Principles of protective ventilator settings for patients with ALI/ARDS are low tidal volume (i.e. VT 6 mL per kg ideal body weight, plateau pressure <30 cmH2O and peak pressure <35 cmH2O).

      • • 

        Permissive hypercapnia may be helpful to realise protective mechanical ventilation.

      • • 

        Protection of the lungs may also be provided by the pump-driven veno-venous ECMO or pumpless ILA.

    2. Page 162
      Abstract
      • • 

        Respiratory failure is failure of one or both of the respiratory system’s gas exchange functions.

      • • 

        It is diagnosed by arterial blood gas assessment.

      • • 

        The clinical presentations of acute, chronic and acute-on-chronic respiratory failure can differ greatly.

    3. Page 166
      Abstract
      • • 

        NIV is the gold standard therapy in acute dyspnoeic COPD patents with a pH <7.35 and PaCO2 >45 mmHg (6.0 kPa) and has been shown to halve mortality in this situation.

      • • 

        Patients with an acute exacerbation of COPD and pH <7.30 being treated with NIV should be managed in a high-dependency or ICU area as they are at risk of deterioration and requirement for invasive ventilation.

      • • 

        In acute hypoxaemic respiratory failure, NIV and entrained oxygen therapy may be tried initially but if improvement in arterial blood gas tensions and dyspnoea do not occur rapidly, urgent consideration should be given to progression to invasive ventilation.

      • • 

        A combination of NIV and cough assistance with insufflation–exsufflation may be helpful in neuromuscular patients with acute chest infection and reduced cough efficacy.

    4. Page 171
      Abstract
      • • 

        Oxygen therapy is prescribed to correct hypoxaemia and should thus be titrated to SaO2.

      • • 

        In acutely hypoxaemic patients, oxygen should be delivered to correct SaO2 to 94–98%.

      • • 

        In those with hypercapnic respiratory failure or at risk of ventilatory decompensation, a target of SaO2 of 88–92% should be the aim.

    5. Page 174
      Abstract
      • • 

        A careful history and physical examination is necessary to assess the risk of post-operative pulmonary complications

      • • 

        Pulmonary function testing is not routine except in the case of evaluation for lung resection

      • • 

        A number of strategies are available to reduce the risk of complications

    6. Page 178
      Abstract
      • • 

        LTV is defined by the requirement for daily ventilatory support for >3 months.

      • • 

        The majority of LTV recipients use NIV via pressure pre-set ventilators.

      • • 

        NIV should be started for symptomatic nocturnal hypoventilation or daytime hypercapnia in restrictive disorders.

      • • 

        NIV extends survival in MND/amyotrophic lateral sclerosis patients.

  8. Page 183
    1. Page 183
      Abstract

      For the aetiological diagnosis of LRTIs:

      • • 

        Gram stain and culture of sputum are valuable in hospitalised patients, if of good quality, for the microbiological diagnosis of LRTI caused by Streptococcus pneumoniae or Haemophilus influenzae,

      • • 

        Urinary antigen detection is a very helpful and rapid test for the diagnosis of pneumococcal or Legionella infections,

      • • 

        Serology is rarely helpful in the management of the individual patient with LRTI,

      • • 

        Molecular tests for the detection of respiratory viruses and atypical pathogens in specific patient populations are desirable.

    2. Page 190
      Abstract
      • • 

        URTIs are the most common infectious illness in the general population, and are the leading cause of missed work and school.

      • • 

        Most URTIs are viral in origin, and typical agents are rhinoviruses, coronaviruses, adenoviruses, coxsackieviruses, influenza and parainfluenza viruses, human metapneumovirus, and respiratory syncytial virus.

      • • 

        URTIs rarely cause permanent sequelae or death but can progress to otitis media, bronchitis, bronchiolitis, pneumonia, sepsis, meningitis, intracranial abscess and other infections.

      • • 

        Diagnosis is usually purely clinical; diagnostic investigations should only be performed in special circumstances, such as influenza, group A streptococcal pharyngitis, infectious mononucleosis and pneumonia.

      • • 

        Infection will often be self-limiting, with no specific treatment necessary; the only indications for antibiotic treatment are group A streptococcal pharyngitis, bacterial sinusitis and pertussis.

    3. Page 194
      Abstract
      • • 

        Up to 75% of COPD exacerbations are of infective aetiology.

      • • 

        Haemophilus influenzae is the most frequent pathogen causing exacerbations.

      • • 

        Relapse rate may be as high as 20%.

      • • 

        Spectrum of antibacterial activity, risk factors for relapse and bacterial resistance to antibiotics are the criteria used for the selection of antibiotics.

    4. Page 199
      Abstract
      • • 

        Pneumonia is very common and has significant mortality.

      • • 

        Severity assessment, aided by a severity assessment score, is a key management step.

      • • 

        A variety of different pathogens can cause pneumonia.

      • • 

        Antibiotic management is initially empirical, and based on guidelines and knowledge of local microbial patterns and resistance rates.

    5. Page 203
      Abstract
      • • 

        Incidence of hospital-acquired pneumonia is ∼0.5–2%, with risk factors including age, type of hospital and type of ward.

      • • 

        Mortality is high (30–70%).

      • • 

        Diagnosis can be difficult, and requires a combined clinical and bacteriological approach.

      • • 

        Antimicrobial therapy must be both prompt and appropriate, and should be modified as culture results become available.

    6. Page 207
      Abstract
      • • 

        Common causes of acquired immunodeficiency are immunosuppressive medication (corticosteroids, cytotoxic chemotherapy and biologicals), radiation, HIV infection and asplenia.

      • • 

        The pathogen type depends on the nature of the underlying immune defects.

      • • 

        Correct assessment of individual risk factors for pneumonia (community versus hospital acquired and immunosuppressed patient) helps to improve treatment.

      • • 

        Diagnostic and treatment algorithms may help to reduce mortality and the use of antibiotics.

      • • 

        These algorithms are solely defined for community- and hospital-acquired pneumonia in major guidelines.

    7. Page 211
      Abstract
      • • 

        Different types of immunosuppression confer vulnerability to different respiratory pathogens, which may be bacterial, viral, mycobacterial or fungal.

      • • 

        The approach to treatment should include comprehensive diagnostic evaluation, indications for empirical antimicrobial treatment and a plan in case of treatment failure.

    8. Page 215
      Abstract
      • • 

        Pleural infection is common and serious, with a mortality rate of ∼15%.

      • • 

        Blood, in addition to pleural fluid, should always be cultured. A higher microbiological yield is achieved if pleural fluid is sent in both a universal container and blood culture bottles.

      • • 

        Initial management is with broad-spectrum antibiotics and prompt chest drainage.

      • • 

        Lung abscess has a 10% mortality rate.

      • • 

        Invasive procedures are only required when a lung abscess does not respond to prolonged empirical antibiotics or an underlying neoplasm is suspected.

    9. Page 222
      Abstract
      • • 

        Influenza is mostly a self-limiting viral upper respiratory tract infection that is managed in the community. Pneumonia is the most frequent serious complications of influenza.

      • • 

        Neuraminidase inhibitors, such as oseltamivir and zanamivir, are effective in the prophylaxis and treatment of influenza A infection.

      • • 

        The influenza A (H1N1) 2009 pandemic was of low severity compared to the other pandemics of the 20th century.

      • • 

        The SARS outbreak of 2003 resulted in 8096 cases, of which 774 died.

      • • 

        SARS-CoV is the causative agent of SARS. Bats are the natural reservoir for coronaviruses.

      • • 

        The management of SARS is chiefly supportive. Basic infection control measures are the cornerstone of containment of any future outbreak.

  9. Page 229
    1. Page 229
      Abstract
      • • 

        With 8.8 million new cases (0.21 being MDR-TB) and 1.1 million deaths, TB is a first-class health priority.

      • • 

        Diagnosis of pulmonary TB is simple, being primarily based on bacteriology (sputum smear microscopy and culture). Recently, a new molecular technique (Xpert MTB/RIF) for the rapid diagnosis of TB and rifampicin resistance has been recommended as the standard in Europe.

      • • 

        Treatment of pan-susceptible cases of pulmonary TB is effective and cheap.

      • • 

        Management of pulmonary TB in MDR-TB/HIV co-infected cases is particularly complicated.

    2. Page 241
      Abstract
      • • 

        EPTB localisations appear in up to 50% of TB patients.

      • • 

        Obtaining culture confirmation is essential in the treatment of both PTB and EPTB.

      • • 

        Treatment of EPTB does not differ from PTB in the majority of EPTB localisations.

    3. Page 245
      Abstract
      • • 

        TB has a higher incidence among people with impaired cellular immunity.

      • • 

        Diagnosis is often delayed owing to early lack of symptoms or unusual presentation.

      • • 

        Screening for LTBI prior to immunosuppressive treatments can be a useful preventive measure.

    4. Page 248
      Abstract
      • • 

        The risk of LTBI depends on the intensity and duration of exposure to a source case with untreated pulmonary TB.

      • • 

        Some infected contacts will develop TB at a later time-point. Timely detection of infected contacts and preventive treatment of those at highest risk of reactivation is cost-effective and reduces the pool of future cases of active TB.

      • • 

        Before prescribing a preventive treatment, active TB should be excluded by a chest radiograph and, if abnormal, by a bacteriological examination of sputum.

      • • 

        The tests for the detection of latent infection are the tuberculin skin test and the IGRAs. The latter has the advantage of a greater specificity.

    5. Page 251
      Abstract
      • • 

        Important features distinguishing NTM from MTC include lower pathogenicity and lack of human-to-human transmission.

      • • 

        Diagnosis of NTM disease requires both clinical and microbiological criteria to be met.

      • • 

        Treatment is disappointing and is characterised by long duration and side-effects, leading to poor compliance.

    6. Page 255
      Abstract
      • • 

        Think of TB; if you do not, the laboratory cannot help you.

      • • 

        Do not use microbiological tests as screening tests.

      • • 

        Remember that the best way to improve testing sensitivity is to submit high-quality specimens.

      • • 

        Molecular tests cannot replace conventional culture.

      • • 

        If your laboratory does not meet current quality standards (testing and turnaround times), refer your specimens to a larger laboratory.

  10. Page 261
    1. Page 261
      Abstract
      • • 

        The prevalence of rhinitis is increasing in most countries.

      • • 

        Asthma is present in 20–50% of allergic rhinitis patients, while up to 80% of asthma patients have rhinitis.

      • • 

        Treatment is anti-inflammatory and directed according to whether rhinitis is allergic or nonallergic.

    2. Page 264
      Abstract
      • • 

        Asthma is diagnosed based on clinical history and lung function testing. Allergy testing may also have a role.

      • • 

        The differential diagnosis is extensive. In particular, COPD may be difficult to distinguish from asthma.

      • • 

        The goal of pharmacological asthma treatment is to achieve and maintain control of symptoms and prevention of exacerbations.

      • • 

        Asthma is a chronic, lifelong disease and must therefore be managed in partnership with the patient.

    3. Page 274
      Abstract
      • • 

        VCD is not well understood, and there is as yet no consensus definition.

      • • 

        Classically, symptoms appear abruptly, resolve quickly and do not respond well to asthma medication.

      • • 

        Long-term treatment is based around speech therapy and psychotherapy.

    4. Page 279
      Abstract
      • • 

        Respiratory viral infection is the most common cause of acute bronchitis.

      • • 

        Acute bronchitis is usually a self-limiting disease.

      • • 

        The diagnosis of acute bronchitis is purely clinical and in most cases symptomatic treatment is sufficient.

      • • 

        Chronic bronchitis is defined clinically as productive cough for 3 months in each of two successive years.

    5. Page 281
      Abstract
      • • 

        GORD is a common disorder caused by the reflux of gastric contents into the oesophagus because of impaired function of the LOS and may result in oesophageal and extraoesophageal symptoms.

      • • 

        The relationship between reflux and respiratory symptoms or disorders is frequently difficult to establish with a high degree of certainty.

      • • 

        Diagnostic, as well as therapeutic, management remains largely empirical.

      • • 

        Treatment with PPIs has been shown to improve cough in patients with acid GOR-induced cough but the effect of PPIs remains disappointing when treating GOR in other respiratory diseases.

      • • 

        Antireflux surgery is associated with improved allograft function after lung transplantation.

    6. Page 287
      Abstract
      • • 

        COPD is a heterogeneous disease, with two main phenotypes: chronic bronchitis and emphysema.

      • • 

        A strong genetic component, in conjunction with environmental insult, probably accounts for the development of COPD.

      • • 

        Smoking cessation is the single most effective intervention in COPD prevention and treatment.

      • • 

        Bronchodilators are central to symptomatic treatment, backed up if necessary by other interventions.

    7. Page 293
      Abstract
      • • 

        Exacerbations are important events; they drive lung function decline and are responsible for much of the morbidity and mortality associated with COPD.

      • • 

        The majority of COPD exacerbations are triggered by respiratory viral infections and/or bacterial infections.

      • • 

        The ‘frequent exacerbator’ phenotype is stable over time and exists across all GOLD stages.

      • • 

        Frequent exacerbators exhibit faster decline in lung function, have worse quality of life, increased risk of hospitalisation and greater mortality.

      • • 

        Both pharmacological and nonpharmacological therapies exist that can help prevent COPD exacerbations.

    8. Page 300
      Abstract
      • • 

        There is clear evidence that COPD is not simply a disease limited to the airways but should be considered a complex and multicomponent syndrome.

      • • 

        FEV1 is not just a lung function parameter for grading COPD severity, but is also a marker for premature death from any cause.

      • • 

        The course of the disease and the prognosis is influenced by extrapulmonary pathology and accompanying comorbidities.

      • • 

        Patients with COPD show comorbidities that are not only related to smoking but also to other lifestyle factors, including diet and inactivity; chronic systemic inflammation seems to link them together and might explain why they often occur together.

      • • 

        Future research is needed to answer the question of whether the successful treatment of comorbidities associated with COPD positively influences the course of the disease itself.

    9. Page 304
      Abstract
      • • 

        Long-acting β2-agonists are effective as add-on therapy to inhaled corticosteroids in asthma and for reducing symptoms in COPD, and act as functional antagonists.

      • • 

        Inhaled corticosteroids are the mainstay of asthma control and suppress activated inflammatory genes, but are largely ineffective in COPD (corticosteroid resistance).

      • • 

        Long-acting muscarinic antagonists are effective bronchodilators in COPD where cholinergic tone is the only reversible component.

      • • 

        Low-dose theophylline may be useful as an add-on therapy in severe asthma and COPD and may reduce corticosteroid resistance.

    10. Page 311
      Abstract
      • • 

        Diagnosis of bronchiectasis is based on the presence of daily production of mucopurulent phlegm and chest imaging that demonstrates dilated and thickened airways. HRCT is the gold standard.

      • • 

        The diagnosis of bronchiectasis should lead to the investigation and treatment of possible causes and associated conditions.

      • • 

        Antibiotics form the mainstay of treatment of bronchiectasis. Acute exacerbations should be treated promptly with short courses of antibiotics.

      • • 

        The efficacy of continuous administration of antibiotics, mucolytics, anti-inflammatory agents and bronchodilators is not clear, but may be considered on an individual basis.

      • • 

        Bronchopulmonary hygiene physical therapy techniques are widely used, yet there is not enough evidence to support or refute them.

      • • 

        Surgery may be considered if the area of the bronchiectatic lung is localised and if the patient’s symptoms are debilitating or life threatening (e.g. massive haemoptysis).

    11. Page 315
      Abstract
      • • 

        Adult pulmonologists need to know about CF; it is common across Europe, patients are surviving into middle age and beyond, and new diagnoses of CF are being made even in old age; as diagnosis by newborn screening becomes more widespread across Europe, it is likely that fitter CF patients will be transferred to the adult clinics, and survival will improve further.

      • • 

        CF is now a true multisystem disease; to the well-known complications of chronic respiratory infection and malabsorption have been added conditions such as cirrhosis, insulin deficiency and diabetes, osteopenia, stress incontinence, and infertility.

      • • 

        Furthermore, with longevity are coming new complications, including the selection of resistant microorganisms and antibiotic allergy; other organ systems will probably be affected in the aging CF population.

      • • 

        Treatment of CF thus requires a dedicated multidisciplinary team, comprising physicians, specialist nurses, physiotherapists, dieticians, clinical psychologists and pharmacists.

      • • 

        The increasing knowledge of the molecular pathophysiology of CF is leading the way in the development of genotype-specific therapies, which will be a paradigm for other diseases.

  11. Page 327
    1. Page 327
      Abstract
      • • 

        The burden of WRA is still very high, accounting for one in 10 cases of adult asthma, and causing morbidity, disability and high costs.

      • • 

        Prevention is very important. Health officials, workplace managers and doctors must be aware of the problem, and strict measures for exposures to known sensitisers should always be followed, conditions at work examined and, when necessary, amended.

      • • 

        Better education of workers and managerial staff as well as medical professionals is key to the prevention and prompt diagnosis and management of WRA and OA. When WRA is diagnosed, prompt management is required and consists of removing or reducing exposure through elimination or substitution of causative agents and, where this is not possible, by effective control of exposure.

      • • 

        Pharmaceutical treatment of OA follows the general asthma guidelines.

    2. Page 332
      Abstract
      • • 

        An influenza-like response (inhalation fever) may follow the inhalation of high quantities of zinc fumes (metal fume fever) or organic aerosols (ODTS).

      • • 

        After inhalation of poorly water-soluble agents, such as nitrogen dioxide, phosgene or cadmium fumes, pulmonary oedema becomes clinically manifest only 4–12 h after exposure.

      • • 

        Acute inhalation injury may be followed by various structural lesions in the airways but also by asthma. Such asthma induced by a single inhalation injury is called acute irritant-induced asthma or RADS.

    3. Page 337
      Abstract
      • • 

        Hypersensitivity pneumonitis (HP) is an immunologically mediated inflammatory lung disease of the parenchyma.

      • • 

        HP is induced by the inhalation of a variety of organic or inorganic antigens, and is characterised by hypersensitivity to the antigens.

      • • 

        The main characteristic of HP is massive lymphocytic inflammation with accumulation of activated T-lymphocytes in the lung interstitium.

      • • 

        The only treatment is to avoid exposure to the offending allergen; if the exposure ceases the symptoms usually subside rapidly, but lung function impairment may persist.

    4. Page 341
      Abstract
      • • 

        Pleural plaques indicate exposure to asbestos, but rarely cause problems.

      • • 

        Asbestos-related diseases (except mesothelioma) are becoming increasingly rare.

      • • 

        Pleural thickening may result from unrecognised benign asbestos pleurisy.

      • • 

        CWP and silicosis are much rarer now in Europe but remain significant problems worldwide.

      • • 

        CWP and silicosis can both be associated with airways obstruction.

      • • 

        Silicosis and asbestosis increase the risk of lung cancer.

    5. Page 345
      Abstract
      • • 

        Recent epidemiological studies have clearly shown that outdoor and indoor air pollution affects respiratory health worldwide, causing an increase in the prevalence of cardiovascular and respiratory symptoms/diseases (i.e. COPD, asthma, hay fever and lung function reduction) and of mortality, both in children and in adults.

      • • 

        Rapid industrialisation and urbanisation have increased air pollution and, consequently, the number of exposed people.

      • • 

        Conservative estimates show that between 1.5 and 2 million deaths per year could be attributed to indoor air pollution in developing countries.

      • • 

        The abatement of the main risk factors for respiratory diseases, and the support of healthcare providers and the general community for public health policies improving outdoor/indoor air quality can achieve huge health benefits.

    6. Page 352
      Abstract
      • • 

        Tobacco use is responsible for more than one in seven of all deaths in the EU.

      • • 

        ∼50% of tobacco-related deaths are due to lung cancer and COPD.

      • • 

        Female smoking is still on the rise in some parts of the EU.

      • • 

        Preventing tobacco use and treating tobacco addicts should be given top priority.

    7. Page 357
      Abstract
      • • 

        Tobacco dependence is a disease and is an important issue for respiratory physicians.

      • • 

        The prevention of tobacco dependence through tobacco control mechanisms is a priority.

      • • 

        Effective and cost-effective treatments for tobacco dependence exist in the form of motivational support and pharmacotherapy.

      • • 

        The treatment of tobacco dependence benefits from knowledge, experience and training, which is not provided in medical schools at the undergraduate level, and should be made a priority.

    8. Page 361
      Abstract
      • • 

        A low barometric pressure at altitude results in reduced inspired oxygen tension and PaO2.

      • • 

        Hypoxaemia triggers adaptive physiological repsonses termed acclimatisation.

      • • 

        Respiratory acclimatisation includes hyperventilation and periodic breathing, which typically prevails during sleep.

      • • 

        AMS, HACE and HAPE may affect travellers after rapid ascent to altitude. Chronic mountain sickness occurs in long-term residents of high mountain areas.

      • • 

        Treatment of high-altitude related illness consists of descent, supplemental oxygen and, if necessary, drugs.

    9. Page 366
      Abstract
      • • 

        Normal lung function and physical work capacity are required for underwater work.

      • • 

        Normal lung function is required to reduce the risk of pulmonary barotrauma.

      • • 

        Cumulative diving exposure is associated with a long-term reduction in lung function of an obstructive pattern, which at some time in the diver’s career may preclude further diving.

    10. Page 369
      Abstract
      • • 

        Radiotherapy for tumour treatment results in pulmonary complications in about 20% of patients.

      • • 

        Radiation-induced lung injury involves vascular damage leading to pulmonary hypertension and develops from an early, inflammatory phase to a late fibrotic phase.

  12. Page 371
    1. Page 371
      Abstract
      • • 

        Interstitial lung diseases are a heterogeneous group of entities with similar clinical presentations.

      • • 

        A pattern-based approach to HRCT can help to discriminate between diseases with similar presentations, making a specific diagnosis in many cases or, at least, narrowing the differential diagnosis.

      • • 

        In controversial cases, surgical biopsy might be necessary and a multidisciplinary approach (integrating clinical presentation and laboratory data, chest imaging, and lung pathology) is considered the best approach to formulate a confident diagnosis.

    2. Page 382
      Abstract
      • • 

        Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology, which commonly affects young and middle-aged adults.

      • • 

        Prevalence of sarcoidosis varies from <1 case to 40 cases per 100 000 population, and overall mortality is 1–5%.

      • • 

        Clinical presentation varies widely, though fever, fatigue and skeletal muscle weakness are often noted.

      • • 

        The decision to treat should be carefully assessed based on the benefit to the patient and disease severity; treatment should mainly be considered if symptoms develop or lung function deteriorates.

      • • 

        The clinical course of sarcoidosis can be unpredictable, so regular monitoring of signs of disease progression is advised.

    3. Page 386
      Abstract
      • • 

        IIPs represent a heterogeneous group of disorders with different clinical and histological features and prognoses.

      • • 

        The most recent ATS and ERS classifications of IIPs include seven different diseases identified by a typical histological pattern: NSIP, cryptogenic organising pneumonia/bronchiolitis obliterans organising pneumonia, acute interstitial pneumonia, respiratory bronchiolitis/interstitial lung disease, desquamative interstitial pneumonia/alveolar macrophage pneumonia and lymphoid interstitial pneumonia.

      • • 

        The terms IPF and NSIP should only be used for chronic fibrosing interstitial pneumonia of unknown cause limited to the lungs. The prognosis in IPF is worse with a histological pattern of UIP.

    4. Page 395
      Abstract
      • • 

        Eosinophilic lung disease covers a wide spectrum of pathology from airways to parenchymal lung disease.

      • • 

        Always exclude secondary causes of eosinophilia before diagnosing acute or chronic eosinophilic pneumonia.

      • • 

        Novel therapies are being introduced for eosinophilia, including tyrosine kinase inhibitors and monoclonal antibodies against IL-5.

    5. Page 399
      Abstract
      • • 

        DIRD is not uncommon, and can involve the larynx, major and lower airways, lung, pleura, pulmonary circulation, neuromuscular system and haemoglobin. Chemotherapy agents, amiodarone, ACE inhibitors, NSAIDs and β-blockers pose particular risk of adverse respiratory effects.

      • • 

        Some DIRDs cause acute life-threatening respiratory distress, requiring immediate management.

      • • 

        The clinical, imaging and pathological expression of DIRD may closely resemble that of illnesses of other causes or that occur idiopathically. Pathology is rarely specific for drug aetiology.

      • • 

        Diagnosing DIRD requires a high degree of awareness, up-to-date knowledge and ruling out of other causes, particularly infection, using BAL and appropriate tests.

      • • 

        Stopping the drug is often followed by improvement in symptoms, signs and imaging. Care should be taken to avoid relapse of the condition for which the drug was given.

      • • 

        Corticosteroid therapy is reserved for severe cases and where dechallenge does not produce satisfactory improvement; duration varies with drug and pattern.

      • • 

        Generally, rechallenge with the drug is discouraged as severe relapse can occur.

  13. Page 411
    1. Page 411
      Abstract
      • • 

        Although early treatment is highly effective, pulmonary embolism is underdiagnosed and, therefore, remains a major health problem.

      • • 

        Diagnostic strategy should be based on clinical evaluation of the probability of pulmonary embolism.

      • • 

        The NPVs and PPVs of diagnostic tests for pulmonary embolism are high when the results are concordant with the clinical assessment.

      • • 

        Additional testing is necessary when the test results are inconsistent with clinical probability.

    2. Page 417
      Abstract
      • • 

        Haemoptysis is often scant or absent in diffuse alveolar haemorrhage.

      • • 

        Vasculitis must often be treated empirically, in the absence of full diagnostic clinical criteria or a histological diagnosis.

      • • 

        Initial treatment should be definitive, even when the diagnosis is tentative.

      • • 

        Chronic infection and malignancy are the most frequent differential diagnosis.

    3. Page 422
      Abstract
      • • 

        Pulmonary hypertension is defined as an increase in mPpa ⩾25 mmHg at rest as assessed by right heart catheterisation.

      • • 

        PAH is a rare condition characterised by chronic pre-capillary pulmonary hypertension leading to right heart failure and death.

      • • 

        PAH can be sporadic (idiopathic PAH), heritable, induced by drugs or toxins, or associated with other conditions such as connective tissue diseases.

      • • 

        Doppler echocardiography is the investigation of choice for noninvasive screening but measurement of haemodynamic parameters during right heart catheterisation is mandatory to confirm pre-capillary pulmonary hypertension (mPpa ⩾25 mmHg and Ppw ⩽15 mmHg).

      • • 

        Recent advances in the management of PAH include prostaglandins, ERA and PDE5 I.

      • • 

        Lung transplantation is an option for severe patients deteriorating despite medical treatment.

  14. Page 428
    1. Page 428
      Abstract
      • • 

        Pleural effusions may present as primary manifestations of many diseases. However, most often, they are observed as secondary manifestations or complications of other diseases.

      • • 

        Cardiac failure is the main cause of pleural effusions. Of noncardiac causes, parapneumonic effusions are commonest, followed by malignant pleural effusions and pleural effusions due to pulmonary embolism.

      • • 

        Small pleural effusions can be detected best by ultrasound (or CT).

      • • 

        Pleural effusion can, in the majority of cases, be diagnosed by case history, clinical presentation, imaging techniques and examination of pleural fluid.

      • • 

        The most important laboratory parameter of pleural fluid is total protein, distinguishing trans- from exudates.

      • • 

        Biopsy procedures such as closed-needle biopsy or medical thoracoscopy/pleuroscopy may be necessary to confirm or exclude malignant or tuberculous causes.

      • • 

        Treatment depends upon the underlying disease.

      • • 

        Local treatment options include therapeutic thoracentesis, chest-tube drainage, chemical pleurodesis and, rarely, surgical interventions.

    2. Page 432
      Abstract
      • • 

        The most likely cause of a primary spontaneous pneumothorax is the rupture of small subpleural bulla.

      • • 

        Pneumothorax usually present with acute chest pain and dyspnoea.

      • • 

        Pneumothorax can be complicated by persistent air leak for >3 days, pneumomediastinum and haemopneumothorax.

      • • 

        Recurrence is the most common indication for surgery in patients with a primary spontaneous pneumothorax.

      • • 

        Surgery is accomplished by a video-assisted thoracoscopy mechanical abrasion, or by parietal apical pleurectomy in association with resection of the lung.

      • • 

        In secondary pneumothorax, the mortality rate for surgery may reach 10% and the morbidity is significant.

    3. Page 439
      Abstract
      • • 

        DNM is a particularly virulent and potentially lethal mediastinal infection.

      • • 

        Initial presentation is toxic shock and respiratory difficulty, sometimes with other signs such as erythema and oedema of the neck and upper chest.

      • • 

        DNM is an emergency, and should be treated with broad-spectrum intravenous antibiotics as well as early and aggressive surgical drainage.

    4. Page 443
      Abstract
      • • 

        NMD have a range of causes, but common principles apply to their treatment.

      • • 

        Treatment focuses on ventilatory assistance and assisted coughing techniques.

    5. Page 448
      Abstract
      • • 

        The two most common chest wall abnormalities are pectus excavatum and pectus carinatum.

      • • 

        The two most common surgical procedures for pectus excavatum repair are the modified Ravitch technique and the Nuss procedure.

      • • 

        Careful pre-operative evaluation on the basis of clinical and psychological symptoms is required to select potential candidates for surgical remodelling.

      • • 

        The optimal timing of surgical repair would be after the main growth has stopped (late teens or early 20s).

  15. Page 451
    1. Page 451
      Abstract
      • • 

        Lung cancer prognosis is poor, most cases being surgically unresectable at the time of diagnosis.

      • • 

        Driver mutations, translocations or amplifications are involved in lung oncogenesis, and have led to a molecular classification of lung tumours.

    2. Page 455
      Abstract
      • • 

        The pulmonologist has a crucial role in obtaining tissue for diagnosis and molecular analyses.

      • • 

        Lung cancer staging is a stepwise process of more general tests for all, and more dedicated tests for patients with a prospect of radical treatment.

      • • 

        Functional assessment is key for patients with a prospect of radical treatment.

    3. Page 460
      Abstract
      • • 

        Due to the interdisciplinary nature of lung cancer treatment, decision-making should take place in structured tumour boards.

      • • 

        Performance status is an important parameter in treatment decision-making.

      • • 

        The side-effects of chemotherapy vary between agents and should be taken into account during treatment planning.

      • • 

        Endobronchial techniques are an important tool in the palliation of lung cancer patients.

      • • 

        First-line treatment of advanced NSCLC, adjuvant chemotherapy and chemotherapy for radiochemotherapy is mostly a platinum-based doublet.

      • • 

        The individualisation of treatment based on histology and molecular biology, in particular the EGFR mutation and EML4ALK fusion, is of increasing importance in NSCLC.

      • • 

        SCLC generally responds well to initial chemotherapy.

      • • 

        Prophylactic cranial irradiation has an important role in the treatment of SCLC.

    4. Page 466
      Abstract

      The following recommendations are evidence based.

      • • 

        Optimal results are obtained by specialised surgeons working in high-volume units.

      • • 

        Anatomical resection combined with a complete lymph node dissection is the gold standard. Increasingly VATS is becoming an alternative to open surgery in patients with small tumours.

      • • 

        Bronchoplastic and angioplastic lobectomies are viable alternatives to pneumonectomy, provided that a complete resection can be achieved.

      • • 

        Segmentectomies could be applied to high-risk patients with tumours <2 cm in diameter; wedge excisions may be recommended for very small bronchoalveolar carcinoma (ground-glass opacity).

    5. Page 472
      Abstract
      • • 

        For early-stage NSCLC patients, surgery (lobectomy or an anatomical segmentectomy with lymph node dissection) remains the standard treatment, while SBRT is indicated for medically inoperable patients.

      • • 

        In locally advanced NSCLC, definitive concurrent chemoradiotherapy is preferred while surgery is used for selected cases (with induction or adjuvant chemotherapy).

      • • 

        Although still controversial, post-operative radiotherapy is recommended for patients with positive surgical margins and/or pathologic N2 disease.

      • • 

        The current management of SCLC includes chemoradiotherapy with or without induction chemotherapy.

      • • 

        PCI is indicated for all stages of SCLC after response to primary therapy.

    6. Page 477
      Abstract
      • • 

        The thorax is a common site of metastasis from several cancers.

      • • 

        It is sometimes difficult to distinguish between primary lung cancer and metastases from other primaries.

      • • 

        Prognosis is linked to the underlying primary.

    7. Page 482
      Abstract
      • • 

        MPEs are much more frequent than primary pleural or chest wall tumours.

      • • 

        Diagnostic strategy includes pleural cytology, but a firm and reliable diagnosis of cancer is based on histology, usually best obtained by biopsies during thoracoscopy.

      • • 

        Talc pleurodesis by thoracoscopy is the best local treatment of recurrent or massive MPE, but indwelling pleural catheters represent an interesting alternative.

      • • 

        Figures 1 and 2 summarise a proposal for MPE and MPM management.

    8. Page 489
      Abstract
      • • 

        Mediastinal tumours are characterised by a wide variation in clinical presentation, histological features and treatment options.

      • • 

        A multidisciplinary approach is necessary to determine optimal treatment.

      • • 

        Surgical treatment should aim at complete resection.

      • • 

        The mediastinum, which is defined as the anatomical compartment between both lungs, is a fascinating region due to its surprising complexity and variety.

  16. Page 491
    1. Page 491
      Abstract
      • • 

        OSAHS is characterised by recurrent episodes of partial or complete upper airway collapse during sleep.

      • • 

        Minimal diagnostic criteria exist for OSAHS.

      • • 

        Overnight polysomnography is the gold standard for OSAHS diagnosis.

    2. Page 498
      Abstract
      • • 

        CSA signifies the loss or reduction in ventilation due to a transient loss of neural output to the respiratory muscles.

      • • 

        A high prevalence of CSA is observed in association with conditions such as CHF, pulmonary hypertension, cerebral stroke, neuromuscular disease, obesity hypoventilation syndrome and opioid use.

      • • 

        Risk factors for CSA/CSR are age >60 years, male sex, severe heart failure, hypocapnia and atrial fibrillation.

      • • 

        Treatment includes oxygen, acetazolamide and positive pressure ventilation, in particular adaptive servoventilation.

    3. Page 503
      Abstract
      • • 

        Sleep-induced hypoventilation is characterised by increased PaCO2 levels of >45 mmHg.

      • • 

        Nocturnal hypoventilation is associated with decreased ventilatory drive, respiratory iatrogenic depression, alteration of respiratory nerve conductance, muscular disease, chest wall deformities or severe obesity.

      • • 

        OHS is the association of obesity and sleep-disordered breathing with daytime hypersomnolence and hypercapnia in the absence of other respiratory diseases.

      • • 

        OHS is nowadays the most common sleep-related hypoventilation syndrome.

      • • 

        Nocturnal polygraphy evaluation is needed in order to diagnose OHS.

      • • 

        In OHS, NIV is used as the first-line treatment with supplementary oxygen when PaCO2 ⩾50 mmHg; if PaCO2 <50 mmHg, nasal CPAP (nCPAP) plus oxygen may be discussed as a first-line treatment after a night trial of nCPAP plus oxygen.

  17. Page 509
    1. Page 509
      Abstract
      • • 

        PIDs include multiple genetic defects that belong to the group of rare diseases. The World Health Organization recognises >70 diseases classified as PID.

      • • 

        The risk and type of infections change according to the main defect of the immune system: they are classified as antibody deficiencies, combined immunodeficiencies, phagocytic disorders and innate immunity disorders.

      • • 

        In pulmonology, a PID diagnosis should be considered in patients presenting with: 1) severe and recurrent respiratory infections; 2) granulomatous diseases; and 3) life-threatening invasive pulmonary infections. In some cases, there are unique features of lung abnormalities in specific defects.

    2. Page 513
      Abstract
      • • 

        In populations with access to antiretroviral therapy, use of combination antiretroviral therapy (CART) has led to a marked reduction in the incidence of many HIV-associated pulmonary diseases and improved overall outcome following a severe respiratory event.

      • • 

        Despite CART, bacterial infections remain more common in HIV-infected people than in the general population.

      • • 

        In response to starting CART, there may be an overexuberant and uncontrolled immune response to exogenous antigen. This phenomenon of immune reconstitution disease can mimic a variety of other conditions and may be life threatening.

      • • 

        TB may occur at any stage of HIV infection. Cases should be managed in line with appropriate public health and infection control guidance.

      • • 

        Noninfectious respiratory complications of HIV are increasingly recognised in an ageing population. Many of these, such as COPD and lung cancer, are linked to smoking, and can run an accelerated course compared with the general population.

      • • 

        Quitting smoking is an important component of long-term respiratory health maintenance.

    3. Page 521
      Abstract
      • • 

        Graft versus host disease (GVHD) is the principal complication of allogeneic HSCT.

      • • 

        Vascular endothelial damage and increased secretion of pro-inflammatory cytokines are involved in the pathogenesis of lung disorders.

      • • 

        Acute and subacute patterns of lung injury include: idiopathic interstitial pneumonia, bronchiolitis obliterans syndrome, organising pneumonia, alveolar haemorrhage, capillaritis, post-transplant lymphoproliferative disorders.

      • • 

        CMV infection is the most frequent viral complication in patients undergoing HSCT and acute GVHD significantly affects active CMV infection recurrence.

      • • 

        GVHD has beneficial effect of on the incidence of leukaemia relapse and increase the overall survival of patients with leukaemia: this phenomenon is known as the graft-versus-tumour effect.

      • • 

        New insights from basic immunology, preclinical models and clinical studies have led to novel approaches for prevention and treatment.

    4. Page 526
      Abstract
      • • 

        Amyloidosis is a protein deposition disease.

      • • 

        Diagnosis is by biopsy and Congo red staining.

      • • 

        Systemic amyloidosis is a life threatening condition that usually affects several organs.

      • • 

        Localised amyloidosis can present with obstructive symptoms, haemoptysis or as an incidental finding on imaging.

      • • 

        Treatment depends on the type and distribution of amyloid deposits.

    5. Page 529
      Abstract
      • • 

        PAP is a rare syndrome caused by surfactant clearance impairment.

      • • 

        >90% of PAP cases are associated with the presence of neutralising autoantibodies against GM-CSF (GMAb; primary autoimmune PAP).

      • • 

        Diagnosis of primary autoimmune PAP is based on the following triad: 1) crazy paving pattern on HRCT; 2) milky appearance and cytology of BAL fluid; and 3) elevated serum level of GMAb.

      • • 

        WLL is the current standard of care of PAP but alternative therapies (especially GM-CSF administration) are under active investigation.

    6. Page 532
      Abstract
      • • 

        Pulmonary LCH is characterised by cough, dyspnoea on exercise, and diffuse pulmonary nodules and cysts on chest imaging in smokers that may evolve into respiratory failure.

      • • 

        Smoking cessation should be attained. No medical therapy has demonstrated efficacy.

    7. Page 535
      Abstract
      • • 

        LAM is a rare disease occurring in women of child-bearing age, characterised by dyspnoea on exertion, relapsing pneumothorax and numerous thin-walled cysts on chest imaging.

      • • 

        Diagnostic criteria have been proposed recently.

      • • 

        The disease may slowly progress to respiratory insufficiency.

      • • 

        No effective therapy is available.

  18. Page 539
    1. Page 539
      Abstract

      Physiotherapy is indicated in most respiratory conditions, both for groups and individuals, for:

      • • 

        self-management advice and education on lifestyle modifications,

      • • 

        breathlessness management,

      • • 

        improvement or maintenance of mobility and function,

      • • 

        airway clearance in well-defined cases,

      • • 

        prescription of exercise and exercise training,

      • • 

        prescription of walking aids.

    2. Page 543
      Abstract
      • • 

        Pulmonary rehabilitation is an evidence-based treatment that improves health-related quality of life and symptoms in COPD.

      • • 

        Programmes should be tailored to the patient in terms of content, location, duration, frequency and exercise training.

      • • 

        In order for the effects to be durable, patients’ everyday activity should be higher after rehabilitation than before.

    3. Page 552
      Abstract

      Palliative care is a multidisciplinary approach and needs teams of specialists with experience and training in palliative care.

  19. Page 554
    1. Page 554
      Abstract
      • • 

        Occurrence of a health outcome is estimated by prevalence (i.e. the proportion of subjects affected by the health outcome in the considered population) and/or incidence (i.e. the proportion of new cases of the health outcome in the considered population).

      • • 

        The effect of exposure to a risk factor on the health outcome and the associated risk (i.e. the probability that the health outcome will occur following the exposure) is quantified through two measures: the ratio of the measures of disease frequency according to the presence or absence of the exposure to the factor, and the difference between these two measures.

      • • 

        The existence of a statistically significant association between the exposure to a factor and the health outcome does not imply that the factor is a cause of the health outcome; causation must meet several criteria introduced by Austin Bradford Hill.

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