Current opinion in pulmonary medicine
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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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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.
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To describe the methods for derivation of clinical phenotypes in general and how they should be applied in sarcoidosis, taking into account the dimensions of the disease. The results from the small number of studies in this area are summarized. ⋯ Clinical phenotypes, by examining relationships between several domains in sarcoidosis, are likely to better delineate the underlying processes and pathways. Larger studies with appropriate methods should be performed to better delineate clinical phenotypes in sarcoidosis.