Current opinion in pulmonary medicine
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The use of noninvasive ventilatory support in patients with cystic fibrosis (CF) has increased exponentially over the past 2 decades. This review examines the current knowledge and considers potential future directions for use of noninvasive ventilation in CF patients. ⋯ The role of noninvasive ventilation in CF patients in chronic respiratory failure is established, but future prospective studies are needed to determine further indications and optimal timing of this intervention. Developments in both ventilator and interface design may enhance the efficacy of ventilation in CF patients but require careful individualized assessment and regular review. The implications on treatment burden and quality of life in CF also need to be studied.
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To review current approaches to the complex intersection of interstitial lung disease (ILD) with the spectrum of connective tissue disease (CTD). ⋯ A multidisciplinary approach can be helpful for CTD-ILD. Further research and controlled trials are needed to determine how to best manage the diverse spectrum of CTD-ILD.
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An overview over the role of lung transplantation in interstitial lung diseases will be given. ⋯ Lung transplantation offers a survival benefit in carefully selected patients with interstitial lung diseases.
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The purpose of this review is to provide an update on acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), with a specific focus on new data regarding the cause, clinical features, management and prognosis of AE-IPF. In addition, the limitations of the current definition of AE-IPF are discussed and a novel classification schema is proposed. ⋯ AE-IPF is associated with significant morbidity and mortality; however, there remains a paucity of clinical data. The current definition of AE-IPF has limitations and a new classification schema should be considered.
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Recent genetic findings have identified new targets of investigation in the field of pulmonary fibrosis and have the potential to change clinical care. ⋯ The inherited risk for pulmonary fibrosis is substantial, and recent data suggest that genetic risk for familial and sporadic forms of the disease are similar. Further characterizing this genetic risk will influence clinical practice in terms of categorization, diagnosis, and screening of individuals for this disease.