Four-dimensional flow MR imaging showed abnormal helical systolic flow in the ascending aorta of patients with a BAV, including those without aneurysm or aortic stenosis. Identification and characterization of eccentric flow jets in these patients may help identify those at risk for development of ascending aortic aneurysm.

The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour.Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour.Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p50.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p50.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p50.001) and a radiological fibrosis score (p50.02).Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables. @ERSpublications Unclassifiable ILD has a heterogeneous clinical course that can be predicted using clinical and radiological variables

Blood flow imaging with 3-dimensional time-resolved, phase-contrast cardiac magnetic resonance (4-dimensional [4D] Flow) is an innovative and visually appealing method for studying cardiovascular disease that allows quantification of important secondary vascular parameters including wall shear stress. The hypothesis of this pilot study is that 4D Flow will become a powerful tool for characterizing the relationship of aortic valve-related flow dynamics, especially with bicuspid aortic valve (BAV), and progression of ascending aortic (AsAo) dilation. We identified 46 patients previously studied with 4D Flow: tricuspid aortic valve patients without valvular disease (n = 20), and BAV patients with either normal flow (n = 7) or eccentric systolic jets resulting in abnormal right-handed helical AsAo flow (n = 19). The subgroup of patients with BAV and eccentric systolic AsAo blood flow was found to have significantly and asymmetrically elevated wall shear stress. This increased hemodynamic burden may place them at risk for AsAo aneurysm.

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A well-accepted International Classification of Diseases, Ninth Revision, code-based IPF algorithm performs poorly, falsely classifying many non-IPF cases as IPF and missing a substantial proportion of IPF cases. A modification of the IPF algorithm may be useful for future population-based studies of IPF.

Purpose:To determine the accuracy of computed tomography (CT) in identifying the histopathologic usual interstitial pneumonia (UIP) pattern in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Materials and Methods:All patients were enrolled into institutional review boardapproved longitudinal cohorts at their respective institution, and informed consent was obtained at the time of enrollment. Images of patients with surgical lung biopsyproved RA-ILD (n = 69) were collected from three tertiary care centers. Two experienced thoracic radiologists independently reviewed the CT scans. The CT pattern was categorized as definite UIP, possible UIP, or inconsistent with UIP in accordance with published criteria. Findings of biopsies were reviewed by an experienced lung pathologist. The sensitivity and specificity of definite CT UIP pattern to histopathologic UIP pattern were determined. The agreement between radiologists was assessed by calculating a k score. Results:The histopathologic UIP pattern was present in 42 of 69 (61%) patients. Men were more likely than women to have a histopathologic UIP pattern (P = .02). Twenty patients (29%, 20 of 69) had a definite UIP pattern on CT scans. The specificity of CT UIP pattern was 96% (26 of 27; 95% confidence interval [CI]: 81%, 100%), with a negative predictive value of 53% (26 of 49). The sensitivity of CT UIP pattern was 45% (19 of 42; 95% CI: 30%, 61%), with a positive predictive value of 95% (19 of 20).The agreement between radiologists for definite UIP pattern versus not was 87% (k = 0.67, P , .0001). Conclusion:Definite UIP pattern on a CT scan in RA-ILD is highly specific and moderately sensitive for histopathologic UIP pattern. CT can therefore help accurately identify the UIP pattern in RA-ILD.q RSNA, 2013