European respiratory review : an official journal of the European Respiratory Society
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Pulmonary arterial hypertension (PAH) is a life-threatening complication of systemic sclerosis (SSc). However, PAH-specific treatments are available and can significantly improve survival of patients, especially those diagnosed in World Health Organization (WHO) functional class (FC) II. Registry data have shown that without screening, more than two-thirds of PAH-SSc patients are in WHO FC III or IV when diagnosed. ⋯ Data from bosentan trials have shown that nearly one-quarter of patients in WHO FC III at baseline can attain WHO FC II status with monotherapy. Maintenance of PAH-SSc patients in WHO FC II with monotherapy is unrealistic, and sequential goal-directed combination therapy is now becoming an accepted treatment strategy. It is hoped that earlier diagnosis, coupled both with regular assessments to ensure treatment goals are being met and timely, appropriate treatment, will further improve the survival rates of those PAH-SSc patients.
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Since the introduction of the flexible fibreoptic bronchoscope in the late 1960s there have been relatively few technological advances for three decades, aside from the development of a white light video bronchoscope with a miniature charge-coupled device built in its tip replacing the fibreoptics. White light flexible videobronchoscopy with its ancillary devices (forceps biopsy, bronchial brushing, bronchoalveolar lavage, bronchial washings and transbronchial needle aspiration) has long been the only established diagnostic bronchoscopic technique. With the advances in microtechnology over the past two decades, recent technical developments such as autofluorescence bronchoscopy and endoscopic ultrasound allow better evaluation of endobronchial, mediastinal and parenchymal lesions.
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Resectional lung volume reduction has proven to be superior to medical treatment in reducing dyspnoea and in increasing lung function, survival and quality of life in a very well selected, low risk group of hyperinflated patients with heterogeneous emphysema predominantly in the upper lobe. Nevertheless, this intervention is hampered by an important pulmonary (30%) and cardiovascular (20%) morbidity, mainly as a result of prolonged (>7 days) air leak, and a 5% risk of death as a result of the surgical intervention. Results from ongoing randomised trials are awaited in order to determine whether less invasive, non-resectional lung volume treatment of emphysema via the bronchoscope using endobronchial valves, airway bypass stents or biological adhesives/heated water vapour will yield similar improvement with less morbidity and reduced mortality, compared with surgical resection. Furthermore, it is hoped that endoscopic lung volume reduction techniques may help patients with homogeneous emphysema currently excluded by most teams for the resectional procedure.
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Mucus hypersecretion is a clinical feature of severe respiratory diseases such as asthma, cystic fibrosis and chronic obstructive pulmonary disease. Airway mucosal infection and/or inflammation associated with these diseases often gives rise to inflammatory products, including neutrophil-derived DNA and filamentous actin, in addition to bacteria, apoptotic cells and cellular debris, that may collectively increase mucus production and viscosity. Mucoactive agents have been the medication of choice for the treatment of respiratory diseases in which mucus hypersecretion is a clinical complication. ⋯ Mucoactive medications include expectorants, mucoregulators, mucolytics and mucokinetics. By developing our understanding of the specific effects of mucoactive agents, we may result in improved therapeutic use of these drugs. The present review provides a summary of the most clinically relevant mucoactive drugs in addition to their potential mechanism of action.
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Exacerbations of chronic obstructive pulmonary disease (COPD) determine disease-associated morbidity, mortality, resource burden and healthcare costs. Acute exacerbation care requirements range from unscheduled primary care visits to emergency room, inpatient or intensive care, generating significant costs in COPD. ⋯ Thus, patients with frequent exacerbations have a more rapid decline in lung function, worse quality of life and decreased exercise performance. Management of COPD directed to reduce incidence and severity of exacerbations improves long-term health status and conserves health care resources and costs.