Current opinion in pulmonary medicine
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Idiopathic pulmonary fibrosis is a progressively fatal interstitial lung disease associated with pathological findings of usual interstitial pneumonia. Its pathogenesis is unknown, and there are no proven effective therapies. Familial cases account for 0.5-2% of idiopathic pulmonary fibrosis. Familial idiopathic pulmonary fibrosis occurs as an autosomal dominant disorder with variable penetrance. The clinical characteristics of familial idiopathic pulmonary fibrosis are indistinguishable from sporadic idiopathic pulmonary fibrosis. These marked similarities support the hypothesis there may, in part, be a genetic basis for idiopathic pulmonary fibrosis. ⋯ The pathogenesis of both idiopathic pulmonary fibrosis and familial idiopathic pulmonary fibrosis remains unclear. A consistent genetic basis has not yet been demonstrated in all cases. Other factors, including variable gene expression, co-carriage of other modifying genes and environmental stimuli, particularly cigarette smoke, significantly contribute to disease expression.
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Our goal is to update advances in the use of surgical lung biopsy in the idiopathic interstitial pneumonias. We discuss an approach for identifying patients with idiopathic interstitial pneumonias who may benefit from surgical lung biopsy, newer surgical approaches and complications and risks of surgery. ⋯ Standard video-assisted thoracoscopic lung biopsy should be considered in patients with interstitial lung diseases of unknown cause who have a subacute course, ground-glass opacities on high-resolution computed tomography or features atypical for idiopathic pulmonary fibrosis, as these patients may respond to therapy. A step-wise process for selection of patients for surgical lung biopsy is recommended.
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To summarize currently available literature regarding indications for inferior vena cava filters, potential problems associated with the different filters available, and the efficacy and safety of retrievable inferior vena cava filters. ⋯ Retrievable filters are a very attractive alternative to either permanent or temporary filters when inferior vena cava interruption becomes necessary, thanks to the advantages of very easy management and the possibility of their being left in place for a long time and removed when they become unnecessary.
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To describe the most recent epidemiologic, molecular and immunologic literature related to the role of infectious antigens in sarcoidosis pathogenesis, with a focus upon Mycobacterium and Proprionibacterium species. ⋯ While the studies to date do not fulfill Koch's postulates, they do add further support to the hypothesis that infectious antigens, particularly those from mycobacteria, may have a causal role in some sarcoidosis cases. In future studies that purport to show an association of microbial antigen(s) with sarcoidosis, investigation of genetic risk factors contributing to risk will be important, in order to explain why some patients are found to have an association with microbial antigens and others are not.
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High-resolution computed tomography (HRCT) has been the major advance in the diagnosis of idiopathic interstitial pneumonias in the last two decades. In diffuse lung diseases, HRCT now has a central role in routine diagnostic evaluation, and a major impact on the utility of other diagnostic tests, especially bronchoalveolar lavage and surgical lung biopsy. ⋯ When HRCT and clinical findings are both typical of an individual diffuse lung disease, i.e. 'pathognomonic', it is generally appropriate to institute management based on a confident noninvasive diagnosis. When clinical and HRCT data are divergent, or when HRCT features are 'indeterminate', however, histologic evaluation continues to play an essential role. Integration of histology with radiologic and clinical data is the best way to formulate the final diagnosis in these cases.