Current opinion in pulmonary medicine
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MRI has emerged as radiation-free imaging modality for assessment of cystic fibrosis lung disease. Here, we review the current status and new developments of MRI in cystic fibrosis. ⋯ MRI is sensitive to detect hallmarks of cystic fibrosis lung disease such as bronchial wall thickening, bronchiectasis, mucus plugging and abnormal lung perfusion. A morpho-functional MRI score has been established for semiquantitative assessment of these characteristic abnormalities over a broad range of disease severity. Recent studies demonstrated that MRI is sensitive to detect changes in mucus plugging and lung perfusion in response to antibiotic therapy for pulmonary exacerbations. These results suggest that MRI may be suitable for noninvasive monitoring and as a quantitative endpoint in clinical trials for cystic fibrosis. Technical advances including ultra-short echo time and Fourier decomposition imaging are expected to enhance morphological and functional MRI of cystic fibrosis lung disease without the need of contrast medium in the near future.
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With our growing understanding of the pathophysiology of cystic fibrosis, the pace of drug discovery is accelerating. Newer agents and therapies have traditionally been added to available medications, given the urgency in treating the disease. As the cystic fibrosis population ages, the number of associated comorbidities increases, requiring additional therapeutic approaches. Thus, while current management strategies have dramatically extended projected life expectancy, the treatment burden of the disease in adulthood has become onerous, and there is increasing concern over unintended effects and drug-drug interactions of new and existing therapies. ⋯ As the cystic fibrosis formulary grows, a primary emphasis will be for providers to develop personalized treatment plans, with a goal to reduce unnecessary treatment burden and an awareness of potential unanticipated effects of medications.
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About one out of 10 patients with idiopathic pulmonary fibrosis (IPF) develop lung cancer. This review provides an epidemiology and clinical update of the association of these two lethal diseases. In addition, we focus on the emerging overlapping epigenetic mechanisms in both diseases. ⋯ The two novel drugs approved for IPF, pirfenidone and nintedanib, open a new scenario in which treated patients with fibrosis will live longer, and possibly have a lower incidence of lung cancer. However, prospective studies are urgently needed to definitively clarify the role of lung cancer treatment in the management of IPF patients. Furthermore, common epigenetic alterations may represent a promising target for therapeutic approaches in the near future.
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Idiopathic pulmonary fibrosis (IPF) is a fatal disease with limited treatment options and extensive gene expression changes identified in the lung parenchyma. Multiple lines of evidence suggest that epigenetic factors contribute to dysregulation of gene expression in IPF lung. Most importantly, risk factors that predispose to IPF - age, sex, cigarette smoke, and genetic variants - all influence epigenetic marks. This review summarizes recent findings of association of DNA methylation and histone modifications with the presence of disease and fibroproliferation. ⋯ Although we are in very early stages of understanding the role of epigenetics in IPF, the potential for the use of epigenetic marks as biomarkers and therapeutic targets is high and discoveries made in this field will likely bring us closer to better prognosticating and treating this fatal disease.
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To highlight recent advances of imaging modalities with focus on interstitial lung disease, pulmonary vascular disease and cardiac involvement of sarcoidosis. The contribution of key imaging features to the assessment of disease activity and their impact in the prognostic evaluation and management of sarcoidosis are also described. ⋯ In many patients with pulmonary sarcoidosis, a confident diagnosis can be made based on clinical and imaging features, without the need for histological sampling. In cardiac sarcoidosis, advanced imaging modalities have an increasing role in the identification of active disease, risk stratification and optimal management.