Current opinion in pulmonary medicine
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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.
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Idiopathic pulmonary fibrosis (IPF) is a progressive and deadly disease. The US Food and Drug Administration recently approved two medications for the treatment of IPF - pirfenidone and nintedanib. Given the limited clinical experience with these agents, a number of questions remain regarding their use. ⋯ Currently many questions remain regarding the use of pirfenidone and nintedanib in IPF, including which drug to prescribe, the optimal patient population to treat, the duration of therapy, and how to define treatment success or failure. It also remains to be seen whether combination therapy with both agents will result in improved outcomes. Hopefully, future randomized controlled trials will address these issues.
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The usual diagnostic criteria for sarcoidosis include the documentation of a compatible clinicoradiologic scenario, biopsy proof of granulomas, and exclusion of alternate causes for the findings. Establishing the presence of multisystem disease, and longitudinal assessment for the emergence of potential sarcoidosis mimics both strengthen the diagnosis. These principles have constituted a 'diagnostic modus operandi' for several decades, but the emergence of several new technologies has begun to transform their application. ⋯ The diagnosis of sarcoidosis and the assessment of its extent should be tailored to the clinical situation. The application of new technologies may permit some evolution of the diagnostic approach in many patients.
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Hypersensitivity pneumonitis is a complex syndrome characterized by a combination of inflammation and fibrosis located in both the airways and the lung parenchyma. Both diagnosis and treatment are a real challenge for physicians. This review will focus on recent developments in this emerging field; furthermore, we will emphasize major gaps in the current knowledge, to stimulate further research in this field. ⋯ Diagnosis and treatment of hypersensitivity pneumonitis remain a real challenge; this article provides an overview of our current understanding and points out new opportunities for further research.