Dataset Information

Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

ABSTRACT: A 23-year-old female patient with factor VII (FVII) deficiency was admitted with severe hypertension and renal failure. Brachial arterial pressures were 230/120 and 220/115 mm/Hg on the right and left arms, respectively. There was no blood pressure difference between the arms. Renal artery Doppler ultrasonography revealed bilateral severe renal artery stenosis (RAS). Contrast-enhanced magnetic resonance imaging angiography (CE-MRA) revealed severe mural irregularities, contrast enhancement in the aorta and its branches, and long-segment stenosis starting in the abdominal aorta and extending into the proximal renal arteries. The diagnosis of Takayasu arteritis (TA) complicated by RAS in a patient with FVII deficiency was established. This is the first case of concomitant TA and factor VII deficiency in the literature. In conclusion, TA complicated with RAS should be kept in mind in the etiology of secondary hypertension, even when there is no blood pressure difference between the arms in patients. CE-MRA is an accurate, sensitive, and safe imaging method for diagnosing vasculitis, even in the early phases of the disease, and should be considered for evaluating the activity and response to treatment in patients with TA.

SUBMITTER: Ozkok S

PROVIDER: S-EPMC6263223 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature

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Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

Ozkok Sercin S Aslan Ahmet A Gulseren Yildiz Y Aciksari Gonul Kutlu GK Agirbasli Mehmet Ali MA

The Eurasian journal of medicine 20181001 3

A 23-year-old female patient with factor VII (FVII) deficiency was admitted with severe hypertension and renal failure. Brachial arterial pressures were 230/120 and 220/115 mm/Hg on the right and left arms, respectively. There was no blood pressure difference between the arms. Renal artery Doppler ultrasonography revealed bilateral severe renal artery stenosis (RAS). Contrast-enhanced magnetic resonance imaging angiography (CE-MRA) revealed severe mural irregularities, contrast enhancement in th ...[more]

PMID: 30515046

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Project description:BackgroundHypertension occurred in 30-80% of Takayasu arteritis (TAK) patients around the world and the occurrence of hypertension might worsen the disease prognosis. This study aimed to investigate the clinical characteristics and imaging phenotypes, as well as their associations with events free survival (EFS) in Chinese TAK patients with hypertension.MethodsThis current research was based on a prospectively ongoing observational cohort-the East China Takayasu Arteritis (ECTA) cohort, centered in Zhongshan Hospital, Fudan University. Totally, 204 TAK patients with hypertension were enrolled between January 2013 and December 2019. Clinical characteristics and imaging phenotypes of each case were evaluated and their associations with the EFS by the end of August 30, 2020, were analyzed.ResultsSevere hypertension accounted for 46.1% of the entire population. Three specific imaging phenotypes were identified: Cluster 1: involvement of the abdominal aorta and/or renal artery (27.5%); Cluster 2: involvement of the ascending aorta, thoracic aorta, the aortic arch, and/or its branches (18.6%); and Cluster 3: combined involvement of Cluster 1 and 2 (53.9%). Clinical characteristics, especially hypertensive severity, differed greatly among the three imaging clusters. In all, 187 patients were followed up for a median of 46 (9-102) months; 72 events were observed in 60 patients (1-3 per person). The overall blood pressure control rate was 50.8%, and the EFS was 67.9% by the end of the follow-up. Multivariate Cox regression indicated that controlled blood pressure (HR = 2.13, 95% CI 1.32-3.74), Cluster 1 (HR = 0.69, 95% CI 0.48-0.92) and Cluster 3 (HR = 0.72, 95% CI 0.43-0.94) imaging phenotype was associated with the EFS. Kaplan-Meier curves showed that patients with controlled blood pressure showed better EFS (p = 0.043). Furthermore, using cases with Cluster 1 imaging phenotype and controlled blood pressure as reference, better EFS was observed in patients with Cluster 2 phenotype and controlled blood pressure (HR = 2.21, 95%CI 1.47-4.32), while the case with Cluster 1 phenotype plus uncontrolled blood pressure (HR = 0.64, 95%CI 0.52-0.89) and those with Cluster 3 phenotype and uncontrolled blood pressure (HR = 0.83, 95%CI 0.76-0.92) suffered worse EFS.ConclusionBlood pressure control status and imaging phenotypes showed significant effects on the EFS for TAK patients with hypertension.

Project description:IntroductionWe conducted a systematic review of patient-reported outcome measures (PROMs) regarding quality of life, disability, mood abnormalities (anxiety, depression), fatigue, illness perceptions and fibromyalgia in Takayasu arteritis (TAK). Wherever available, comparisons with healthy controls, disease controls or longitudinal changes in PROMs were noted.MethodsMEDLINE, EMBASE, Scopus, Web of Science and Pubmed Central databases, major recent international rheumatology conference abstracts, clinical trial databases and the Cochrane library were searched for relevant articles. Wherever possible, outcome measures across studies were pooled using the restricted maximum likelihood model. Inter-group differences were pooled and compared using standardized mean differences (SMD) with 95% confidence intervals (95% CI). Heterogeneity was assessed using the I2 statistic. Quality of randomized controlled trials was assessed using the Cochrane risk of bias tool. For cross-sectional and cohort studies, the Joana Briggs Institute checklist and Newcastle-Ottawa scale were used, respectively. GRADE methodology was used to determine the certainty of evidence for outcomes.ResultsTwenty-one studies (all but one observational) involving 1311 patients with TAK and 308 healthy controls were identified. Ten studies (559 TAK patients, 182 healthy controls were synthesized in a meta-analysis. Patients with TAK had worse quality of life (pooled SMD - 6.66, 95% CI - 10.08 to - 3.23 for individual domains; - 0.64, 95% CI - 1.19 to - 0.09 for pooled physical and mental component scores of 36-item Short Form Survey), depression (SMD 0.26, 95% 0.05-0.47) and anxiety (SMD 0.34, 95% CI - 0.06 to 0.75) scores and higher disability (SMD 0.64, 95% CI 0.43-0.84) than healthy controls. Patients with active TAK had worse quality of life, depression and work impairment when compared with those with inactive disease. Included studies were of moderate to high quality. Certainty of evidence for individual outcomes was low to very low.ConclusionLiterature on PROMs in TAK, albeit sparse, appears to indicate worse scores in patients with TAK compared to healthy individuals. These results, however, require cautious interpretation. Development of a TAK-specific PROM is an important focus of the research agenda.

Project description:Atherosclerotic renovascular disease (ARVD) is associated with high rates of coronary events and predicts mortality among patients with coronary artery disease (CAD). However, the impact of coronary atherosclerosis on renal outcomes after revascularization of ARVD is unclear. We hypothesized that CAD negatively impacts renal functional outcomes among patients with ARVD undergoing renal artery revascularization.Patients with ARVD who underwent echocardiography at Mayo Clinic, Rochester, Minnesota, USA between 2004 and 2012 were identified retrospectively and included if they had ejection fraction more than 50%. Renal and overall outcomes were compared among atherosclerotic renovascular disease patients with coronary artery disease (ARVD-C, n?=?75) and without coronary artery disease (ARVD, n?=?56), within 1 year from initial revascularization and included blood pressure control, renal function, and incident cardiovascular/cerebrovascular events.Degree of renal artery stenosis was similar in both groups. ARVD-C had higher prevalence of diabetes, peripheral artery disease (PAD), and cerebrovascular disease, and lower baseline renal function. Risk of developing end-stage renal disease was higher in ARVD-C (11 vs. 2%, P?=?0.05). Despite better control of blood pressure and cholesterol levels, renal function postrevascularization worsened in 15% of ARVD-C compared with 2% of ARVD (P?=?0.01). Differences in clinical outcomes remained statistically significant after adjustment for covariables, including sex, baseline blood pressure, renal function, underlying diabetes, cholesterol levels, and medications. Similar differences in clinical outcomes were also associated with PAD and cerebrovascular disease.CAD in patients with ARVD is a predictor of worse outcomes after renal revascularization, likely reflecting diffuse atherosclerotic disease. Further studies are needed to develop strategies to manage patients with vascular comorbidities and improve their outcomes.

Dataset Information

Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

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Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

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