clinicaltrials.gov Indentifier: NCT01174550.

Objectives:IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined. We describe the clinical features, radiology findings, and treatment response in a cohort of 36 patients with IgG4-RD affecting large blood vessels.Methods:Clinical records of all patients diagnosed with IgG4-RD in our center were reviewed. All radiologic studies were reviewed. We distinguished between primary large blood vessel inflammation and secondary vascular involvement. Primary involvement was defined as inflammation in the blood vessel wall as a principal focus of disease. Secondary vascular involvement was defined as disease caused by the effects of adjacent inflammation on the blood vessel wall.Results:Of the 160 IgG4-RD patients in this cohort, 36 (22.5%) had large-vessel involvement. The mean age at disease onset of the patients with large-vessel IgG4-RD was 54.6 years. Twenty-eight patients (78%) were male and 8 (22%) were female. Thirteen patients (36%) had primary IgG4-related vasculitis and aortitis with aneurysm formation comprised the most common manifestation. This affected 5.6% of the entire IgG4-RD cohort and was observed in the thoracic aorta in 8 patients, the abdominal aorta in 4, and both the thoracic and abdominal aorta in 3. Three of these aneurysms were complicated by aortic dissection or contained perforation. Periaortitis secondary to RPF accounted for 27 of 29 patients (93%) of secondary vascular involvement by IgG4-RD. Only 5 patients demonstrated evidence of both primary and secondary blood vessel involvement. Of those treated with rituximab, a majority responded positively.Conclusions:IgG4-RD is a distinctive, unique, and treatable cause of large-vessel vasculitis. It can also involve blood vessels secondary to perivascular tumefactive lesions. The most common manifestation of IgG4-related vasculitis is aortitis with aneurysm formation. The most common secondary vascular manifestation is periaortitis with relative sparing of the aortic wall. Both primary vasculitis and secondary vascular involvement respond well to B cell depletion therapy.

To update pretest probabilities (PTP) for obstructive coronary artery disease (CAD > _ 50%) across age, sex, and clinical symptom strata, using coronary computed tomography angiography (CTA) in a large contemporary population of patients with stable chest pain referred to non-invasive testing.

Our data support the hypothesis that the proton RBE for lung-density changes exceeds 1.1. This RBE elevation could be attributable to (1) the late, normal tissue endpoint that we consider or (2) end-of-range proton linear energy transfer elevation-or a combination of the two. Regardless, our results suggest that variations in proton RBE prove important in vivo as well as in vitro.

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Purpose To evaluate the frequency and implications of perivascular fat stranding on coronary computed tomography (CT) angiograms obtained for suspected acute coronary syndrome (ACS). Materials and Methods This retrospective registry study was approved by the institutional review board. The authors reviewed the medical records and images of 1403 consecutive patients (796 men, 607 women; mean age, 52.8 years) who underwent coronary CT angiography at the emergency department from February 2012 to March 2016. Fat attenuation, length and number of circumferential quadrants of the affected segment, and attenuation values in the unaffected epicardial and subcutaneous fat were measured. "Cases" were defined as patients with perivascular fat stranding. Patients with significant stenosis but without fat stranding were considered control subjects. Baseline imaging characteristics, ACS frequency, and results of subsequent downstream testing were compared between cases and control subjects by using two-sample t, Mann-Whitney U, and Fisher tests. Results Perivascular fat stranding was seen in 11 subjects, nine with atherosclerotic lesions and two with spontaneous coronary artery dissections, with a mean fat stranding length of 19.2 mm and circumferential extent averaging 2.9 quadrants. The mean attenuation of perivascular fat stranding, normal epicardial fat, and normal subcutaneous fat was 17, -93.2, and -109.3 HU, respectively (P < .001). Significant differences (P < .05) between cases and control subjects included lower Agatston score, presence of wall motion abnormality, and initial elevation of serum troponin level. ACS frequency was 45.4% in cases and 3.8% in control subjects (P = .001). Conclusion Recognition of perivascular fat stranding may be a helpful additional predictor of culprit lesion and marker of risk for ACS in patients with significant stenosis or spontaneous coronary artery dissection. RSNA, 2018 Online supplemental material is available for this article.

Purpose To assess concordance and relative prognostic utility between central core laboratory and local site interpretation for significant coronary artery disease (CAD) and cardiovascular events. Materials and Methods In the Prospective Multicenter Imaging Study for Evaluation of Chest Pain (PROMISE) trial, readers at 193 North American sites interpreted coronary computed tomographic (CT) angiography as part of the clinical evaluation of stable chest pain. Readers at a central core laboratory also interpreted CT angiography blinded to clinical data, site interpretation, and outcomes. Significant CAD was defined as stenosis greater than or equal to 50%; cardiovascular events were defined as a composite of cardiovascular death or myocardial infarction. Results In 4347 patients (51.8% women; mean age ± standard deviation, 60.4 years ± 8.2), core laboratory and site interpretations were discordant in 16% (683 of 4347), most commonly because of a finding of significant CAD by site but not by core laboratory interpretation (80%, 544 of 683). Overall, core laboratory interpretation resulted in 41% fewer patients being reported as having significant CAD (14%, 595 of 4347 vs 23%, 1000 of 4347; P < .001). Over a median follow-up period of 25 months, 1.3% (57 of 4347) sustained myocardial infarction or cardiovascular death. The C statistic for future myocardial infarction or cardiovascular death was 0.61 (95% confidence interval [CI]: 0.54, 0.68) for the core laboratory and 0.63 (95% CI: 0.56, 0.70) for the sites. Conclusion Compared with interpretation by readers at 193 North American sites, standardized core laboratory interpretation classified 41% fewer patients as having significant CAD. RSNA, 2017 Online supplemental material is available for this article. Clinical trial registration no. NCT01174550.

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