Respirology : official journal of the Asian Pacific Society of Respirology
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Treatment with pirfenidone may slow the decline in vital capacity and increase progression-free survival (PFS) in idiopathic pulmonary fibrosis (IPF). The effects of combination therapy with inhaled N-acetylcysteine (NAC) and pirfenidone are unclear. We assessed the effects of this combination therapy in patients with advanced IPF. ⋯ Combination treatment with inhaled NAC and oral pirfenidone reduced the rate of annual FVC decline and improved PFS in patients with advanced IPF.
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Exacerbations of chronic obstructive pulmonary disease (COPD) are one of the commonest causes of emergency hospital admission and are associated with high rates of readmission. Rehabilitation in the peri- and early post-hospitalization setting may counteract the deleterious consequences of an acute hospital admission and target modifiable risk factors for readmission such as physical inactivity, reduced exercise capacity and impaired physical function. Pulmonary rehabilitation in the peri-hospitalization period can improve exercise capacity and health-related quality of life and can also reduce rates of readmission. ⋯ However, recent trials showing less positive results and observational data questioning acceptability may challenge prevailing enthusiasm. This review examines the role of pulmonary rehabilitation in the peri- and early post-hospitalization setting, considering the modifiable risk factors for readmission, the latest evidence regarding rehabilitation in the acute setting, issues around acceptability and uptake, and alternative strategies to help deliver rehabilitation to more patients. The acceptability and effectiveness of pulmonary rehabilitation offered that post-exacerbation could be improved by overcoming issues around the setting, timing and format of rehabilitation approaches, including their integration with self-management interventions.
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Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. ⋯ Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population.
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A single nucleotide polymorphism (SNP) rs35705950 in the promoter of Mucin 5B (MUC5B) has been reported to be associated with idiopathic pulmonary fibrosis (IPF) mainly in Caucasian populations. This study was conducted to confirm the association between rs35705950 and IPF in a Japanese population. ⋯ The association between rs35705950 and IPF was also present in this Japanese cohort, but was not as strong as the German counterpart. To our knowledge, this is the first study to successfully validate the association between rs35705950 and IPF in a Japanese ethnicity.
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Recent data suggest that grey-scale textural analysis on endobronchial ultrasound (EBUS) imaging can differentiate benign from malignant lymphadenopathy. The objective of studies was to evaluate grey-scale textural analysis and examine its clinical utility. ⋯ These findings suggest that use of EBUS grey-scale textural analysis for differentiation of malignant from benign lymphadenopathy may not be accurate. Further studies are required.