European respiratory review : an official journal of the European Respiratory Society
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We are currently limited in our abilities to diagnose, monitor disease status and manage chronic airway disease like asthma and chronic obstructive pulmonary disease (COPD). Conventional lung function measures often poorly reflect patient symptoms or are insensitive to changes, particularly in the small airways where disease may originate or manifest. Novel pulmonary function tests are becoming available which help us better characterise and understand chronic airway disease, and their translation and adoption from the research arena would potentially enable individualised patient care. ⋯ With a particular focus on asthma and COPD, this article demonstrates how chronic airway disease mechanisms have been dismantled with the use of the FOT and MBNW. We describe their ability to assess detailed pulmonary mechanics for diagnostic and management purposes including response to bronchodilation and other treatments, relationship with symptoms, evaluation of acute exacerbations and recovery, and telemonitoring. The current limitations of both tests, as well as open questions/directions for further research, are also discussed.
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Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be "lumped" together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. ⋯ However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will provide insight into the role of antifibrotic treatment with nintedanib or pirfenidone in the management of fibrosing ILDs with a progressive phenotype.
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Obesity hypoventilation syndrome (OHS) is defined as a combination of obesity (body mass index ≥30 kg·m-2), daytime hypercapnia (arterial carbon dioxide tension ≥45 mmHg) and sleep disordered breathing, after ruling out other disorders that may cause alveolar hypoventilation. OHS prevalence has been estimated to be ∼0.4% of the adult population. OHS is typically diagnosed during an episode of acute-on-chronic hypercapnic respiratory failure or when symptoms lead to pulmonary or sleep consultation in stable conditions. ⋯ CPAP is considered the first-line treatment modality for OHS phenotype with concomitant severe obstructive sleep apnoea, whereas NIV is preferred in the minority of OHS patients with hypoventilation during sleep with no or milder forms of obstructive sleep apnoea (approximately <30% of OHS patients). Acute-on-chronic hypercapnic respiratory failure is habitually treated with NIV. Appropriate management of comorbidities including medications and rehabilitation programmes are key issues for improving prognosis.
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The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. ⋯ This review presents a broad overview of the epidemiological data available for ILDs that may present a progressive-fibrosing phenotype, collectively and stratified according to clinical classification. We also note where further data are needed in comparison to the well-studied IPF indication.
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Pulmonary arterial hypertension (PAH) is a condition characterised by increased pulmonary vascular resistance which can lead to right heart failure and premature death. It imposes a significant burden on patients' lives, affecting their physical, emotional and social wellbeing. Pharmacological therapies are the mainstay of treatment; while they are not curative, they can alleviate patient suffering, improve quality of life and delay disease progression. ⋯ Importantly, palliative care can be provided in concert with pharmacological treatment. Despite its potential benefits, palliative care is frequently underutilised. There is a paucity of clinical studies testing the impact of palliative care in PAH which prompted us to summarise the available evidence, recognise obstacles in its utilisation and identify areas for future research.