European respiratory review : an official journal of the European Respiratory Society
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Review
The need to move from 6-minute walk distance to outcome trials in pulmonary arterial hypertension.
Assessment of change in exercise capacity using the 6-min walk distance (6MWD) test has been the primary end-point in the majority of pulmonary arterial hypertension (PAH) clinical trials. The 6MWD has some advantages as an end-point in such studies. It is simple and inexpensive to perform, reproducible and validated. ⋯ While the 6MWD was initially considered to be a potentially reliable surrogate for disease progression in PAH, there is increasing evidence that this is not necessarily the case. Given this, there is a need to re-examine the role of 6MWD in PAH trials, and to evaluate the evidence supporting whether there is a need to move from 6MWD to more robust measures of clinical outcomes, such as morbidity and mortality. However, in the clinic the 6MWD test, alongside symptoms, haemodynamics and biomarkers, remains a useful tool in the assessment and management of PAH patients.
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Although the causal pathomechanisms contributing to remodelling of the pulmonary vascular bed in pulmonary arterial hypertension (PAH) are still unclear, several analogous features with carcinogenesis have led to the emergence of the cancer-like concept. The major similarities concern the altered crosstalk between cells from different tissue types, unexplained proliferation and survival of pulmonary smooth muscle and endothelial cells, the metabolic (glycolytic) shifts, and the association with the immune system. ⋯ It is clear that PAH is not a cancer, but this cancer-like concept has opened a new field of investigation and raises the possibility that antiproliferative and/or oncological drugs may exert therapeutic effects not only in cancer, but also in PAH. Such analogies and differences are discussed here.
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Review Historical Article
Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future.
During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, randomised, placebo-controlled trials in order to investigate agents with different mechanisms of action. ⋯ The main aim for the future should be the careful design of clinical trials, by choosing the most clinically meaningful end-point and keeping in mind that combination of various agents may be more effective. This approach has been used in the treatment of lung cancer with which IPF presents many similarities.
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Review
The importance of components of pulmonary rehabilitation, other than exercise training, in COPD.
Comprehensive pulmonary rehabilitation is an important component in the clinical management of people with chronic obstructive pulmonary disease (COPD). Although supervised exercise training is considered the cornerstone of effective pulmonary rehabilitation, there are many other components that should be considered to manage the impairments and symptom burden, as well as the psychosocial and lifestyle changes imposed by COPD. These include approaches designed to: 1) facilitate smoking cessation; 2) optimise pharmacotherapy; 3) assist with early identification and treatment of acute exacerbations; 4) manage acute dyspnoea; 5) increase physical activity; 6) improve body composition; 7) promote mental health; 8) facilitate advance care planning; and 9) establish social support networks. This article will describe these approaches, which may be incorporated within pulmonary rehabilitation, to optimise effective chronic disease self-management.
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While obstructive sleep apnoea syndrome dominates discussion of the prevalence of sleep disordered breathing, nocturnal hypoventilation remains extremely prevalent in those with chronic ventilatory disorders and in the natural history of these conditions pre-dates the development of daytime ventilatory failure. In this review the clinical management of chronic hypoventilation in neuromuscular disease will be considered and then compared with that in obesity hypoventilation syndrome. In simple terms these conditions illustrate the polar opposite ends of the spectrum, as in neuromuscular disease the reduced capacity of the respiratory system is unable to withstand a normal respiratory load, and in obesity hypoventilation syndrome the normal capacity of the respiratory system is unable to tolerate a substantially increased ventilatory load.