The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps. ⋯ Updates are provided on issues related to utility of the previous classification system to reflect paediatric-specific aetiologies and approaches to medical and interventional management of PAH, including the Potts shunt. Although a lack of clinical trial data for the use of PAH-targeted therapy persists, emerging data are improving the identification of appropriate targets for goal-oriented therapy in children. Such data will likely improve future clinical trial design to enhance outcomes in paediatric PAH.
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Safety concerns are a barrier to prescribing benzodiazepines (BDZs) and opioids in interstitial lung disease (ILD). We therefore examined the association of BDZs and opioids on risk of admission to hospital and death. We conducted a population-based longitudinal cohort study of fibrotic ILD patients starting long-term oxygen therapy in Sweden between October 2005 and December 2014. ⋯ Opioids showed no association with increased admission. Neither low-dose opioids (≤30 mg·day-1 oral morphine equivalent) (SHR 1.18, 95% CI 0.96-1.45) nor high-dose opioids (>30 mg·day-1 oral morphine equivalent) (SHR 1.11, 95% CI 0.89-1.39) showed association with increased mortality. This first ever study to examine associations between BDZ and opioid use and harm in ILD supports the use of opioids and low-dose BDZs in severely ill patients with respiratory compromise.
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The efficacy, safety and positioning of inhaled corticosteroids (ICS) in the treatment of patients with chronic obstructive pulmonary disease (COPD) is much debated, since it can result in clear clinical benefits in some patients ("friend") but can be ineffective or even associated with undesired side effects, e.g. pneumonia, in others ("foe"). After critically reviewing the evidence for and against ICS treatment in patients with COPD, we propose that: 1) ICS should not be used as a single, stand-alone therapy in COPD; 2) patients most likely to benefit from the addition of ICS to long-acting bronchodilators include those with history of multiple or severe exacerbations despite appropriate maintenance bronchodilator use, particularly if blood eosinophils are >300 cells·µL-1, and those with a history of and/or concomitant asthma; and 3) the risk of pneumonia in COPD patients using ICS is higher in those with older age, lower body mass index (BMI), greater overall fragility, receiving higher ICS doses and those with blood eosinophils <100 cells·µL-1 All these factors must be carefully considered and balanced in any individual COPD patient before adding ICS to her/his maintenance bronchodilator treatment. Further research is needed to clarify some of these issues and firmly establish these recommendations.
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Meta Analysis
Triple therapy versus single and dual long-acting bronchodilator therapy in COPD: a systematic review and meta-analysis.
We performed a meta-analysis to compare the impact of triple combination therapy with inhaled corticosteroids (ICS), long-acting β2-agonists (LABAs) and long-acting muscarinic receptor antagonists (LAMAs) versus LABA/LAMA combination therapy or single long-acting bronchodilator therapy in chronic obstructive pulmonary disease. The ICS/LABA/LAMA combination reduced the risk of exacerbation (relative risk 0.70, 95% CI 0.53-0.94) and improved trough forced expiratory volume in 1 s (mean difference in mL +37.94, 95% CI 18.83-53.89) versus LABA/LAMA combination therapy. The protective effect of triple combination therapy versus LABA/LAMA combination therapy against risk of exacerbation was greater in patients with blood eosinophil counts ≥300 cells·µL-1 (relative risk 0.57, 95% CI 0.48-0.68). ⋯ The risk of pneumonia did not differ between ICS/LABA/LAMA combination therapy and its comparators. The number needed to harm was ∼195. This meta-analysis suggests that patients on single long-acting bronchodilator therapy or LABA/LAMA combination therapy, who still have exacerbations and have blood eosinophil counts ≥300 cells·µL-1, could benefit from ICS/LABA/LAMA combination therapy.