Bilateral pulmonary artery stenosis with pulmonary hypertension due to Takayasu's arteritis, examined using quantitative flow ratio during revascularization.
Bilateral pulmonary artery stenosis with pulmonary hypertension due to Takayasu's arteritis, examined using quantitative flow ratio during revascularization.
Project description:BACKGROUND:This study was performed to analyze the clinical manifestations, imaging features, and prognosis of Takayasu's arteritis (TA) with pulmonary arteritis (PA). METHODS:In total, 51 of 815 patients with TA were diagnosed with PA at the Peking Union Medical College Hospital from 1986 to 2015. The patients' medical records and radiographic data were retrospectively reviewed. RESULTS:The patients comprised 39 women and 12 men with a median age of 33 years (range, 14-67 years). The most common symptoms were dyspnea (70.6%), cough (66.7%), hemoptysis (47.1%), and chest pain (45.1%). Computed tomography (CT) pulmonary angiography, pulmonary arteriography, and pulmonary perfusion imaging showed pulmonary artery stenosis or occlusion in 44 patients. A total of 82.4% of patients had lung parenchyma lesions on CT scans, indirectly indicating pulmonary artery involvement. Additionally, 58.8% of patients had pulmonary hypertension (PH) by echocardiography. Compared with the PH group, the non-PH group was characterized by a shorter disease duration; more symptoms such as fever, chest pain, and hemoptysis; an increased erythrocyte sedimentation rate; and a higher incidence of subpleural wedge-shaped shadows on chest CT (P < 0.05). The median follow-up period was 48 months (range, 1-212 months), and all three deaths occurred in the PH group. CONCLUSIONS:The clinical manifestations of TA with PA are nonspecific. PH often complicates PA and is associated with a poor prognosis. Early clinical manifestations such as repeated fever, chest pain, hemoptysis, and recurrence of subpleural wedge-shaped shadows on chest CT should arouse suspicion of PA in patients with TA and prompt further investigations. This may allow PA to be diagnosed before the occurrence of PH. TRIAL REGISTRATION:ClinicalTrials, NCT03189602. Date of registration: June 16, 2017. Retrospectively registered.
Project description:Takayasu arteritis is a chronic vasculitis involving the aorta and its main branches, the pulmonary arteries, and the coronary tree, and needs to be considered in a young patient with angina, in particular when pulses are absent. This case illustrates the limitations of exercise testing in diagnosing the extent of coronary artery disease and the risks associated with coronary angiography in patients with inflammatory disease in the left main stem coronary artery. It also highlights the novel use of non-invasive scanning with positron emission tomography using 18-fluorodeoxyglucose in assessing remission from this disease. Revascularisation was performed with percutaneous transluminal coronary angioplasty and stenting as an emergency procedure, but treatment of the restenosis with directional atherectomy was based on a review of the available literature. The lymphocytic alveolitis seen in this patient has not been previously described in Takayasu's disease.
Project description:BackgroundVasculitides are commonly unrecognized causes of coronary stenosis and myocardial ischemia. We report on a 24-year old patient with Takayasu's arteritis who underwent urgent percutaneous coronary intervention, suffered from symptomatic restenosis of the left main coronary artery during standard immunosuppressive therapy.Case presentationA 24-year old woman was referred for coronary angiography because of typical progressive angina pectoris. On bicycle ergometry, there were both reproducible symptoms and deep ST segment depressions on precordial leads. Semi-selective angiography of the left coronary artery revealed high-grade ostial stenosis. Because of persistent angina pectoris and electrocardiographic signs of acute myocardial ischemia, immediate percutaneous coronary angioplasty with subsequent implantation of an everolimus-eluting stent was performed. This intervention was performed with excellent angiographic results. Because of several concomitant criteria including hypoechogenicity on postprocedural intravascular ultrasonography, the diagnosis of Takayasu's disease was made. The patient was treated with prednisolone and cyclophosphamide for 5 months. Because of recurrent angina pectoris, another coronary angiography was performed, which revealed high-grade in-stent-restenosis. Immunomodulatory therapy was switched to high-dose prednisolone and the anti-IL-6 receptor antagonist tocilizumab. The high-grade in-stent-restenosis persisted, and aortocoronary bypass graft surgery was performed with two saphenous vein grafts to the left anterior descending and circumflex artery. Since then, the patient has been doing well for 2 years.ConclusionIn cases of treatment refractoriness during standard immunosuppressive therapy, more recently developed biological compounds may offer an alternative strategy.
Project description:BackgroundDelayed contrast-enhanced magnetic resonance imaging (DE-MRI) is a useful technique to identify arterial wall inflammation. The aim of this study was to explore the value of DE-MRI in the evaluation of pulmonary artery (PA) lesions in Takayasu's arteritis (TAK) compared with 18F-fuorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT).MethodsPatients with TAK were recruited for this prospective, observational study. Imaging and clinical assessments were performed concurrently. Only thoracic arteries were evaluated, and they were divided into 18 segments per person. All arterial lesions were evaluated using both PET/CT and DE-MRI. Correlations between both methods were assessed in the PA and thoracic aorta. A receiver operating characteristic (ROC) curve was used to analyze the value of imaging features in detecting disease activity based on National Institutes of Health (NIH) criteria.ResultsA total of 24 patients contributed 432 arterial segments. Using PET/CT, correlations between arterial wall DE, thickening, and edema in the PA were 84.52%, 67.92%, and 58.33%, respectively, with Cohen's kappa =0.69, 0.30, and 0.13, respectively; for the thoracic aorta, the values were 86.38%, 80.00%, and 75.92%, respectively, with Cohen's kappa =0.71, 0.52, and 0.372, respectively. There was a significant difference in the incidence of wall DE between the PA and thoracic aorta in patients with clinically active TAK (χ2=6.85, P=0.009). DE-MRI presented a higher area under the curve [area under the curve (AUC); 0.729, P=0.047] than wall thickening and edema in the detection of TAK activity. The wall DE combined with erythrocyte sedimentation rate (ESR) showed improved efficiency (AUC: 0.858, P=0.003).ConclusionsDE-MRI displays appreciable correlations with PET/CT findings and allows for the detection of PA inflammation in patients with TAK; it shows higher values in the thoracic aorta than in the PA. The combination of wall DE and ESR can improve the efficiency of assessing disease status.
Project description:Takayasu's Arteritis (TA) is a rare, chronic large-vessel vasculitis that can lead to severe cardiac complications and life-threatening outcomes. Early diagnosis is essential for improving patient prognosis, but its nonspecific clinical presentation and laboratory findings often cause delays. We present a 34-year-old woman with a history of heart murmur who presented with chest pain but no additional symptoms. Imaging revealed aortic regurgitation, ventricular septal defect, myocardial ischemia, pericarditis, aortic wall thickening, and multivessel stenoses, leading to a diagnosis of Takayasu's Arteritis, treated with coronary bypass and aortic tube graft surgery. Takayasu's Arteritis should be included in the differential diagnosis of patients presenting with atypical clinical features and cardiac involvement, particularly in cases with valvular disease. This case highlights the essential role of multimodal imaging in the detection and management of TA.
Project description:BackgroundRandomized controlled trials (RCT) have shown no overall benefit of renal revascularization in atherosclerotic renovascular disease (ARVD). However, 25% of patients demonstrate improvement in renal function. We used the ratio of magnetic resonance parenchymal volume (PV) to isotopic single kidney glomerular filtration rate (isoSKGFR) ratio as our method to prospectively identify "improvers" before revascularization.MethodsPatients with renal artery stenosis who were due revascularization were recruited alongside non-ARVD hypertensive CKD controls. Using the controls, 95% CI were calculated for expected PV:isoSK-GFR at given renal volumes. For ARVD patients, "improvers" were defined as having both >15% and >1ml/min increase in isoSK-GFR at 4 months after revascularization. Sensitivity and specificity of PV:isoSK-GFR for predicting improvers was calculated.Results30 patients (mean age 68 ±8 years), underwent revascularization, of whom 10 patients had intervention for bilateral RAS. Stented kidneys which manifested >15% improvement in function had larger PV:isoSK-GFR compared to controls (19±16 vs. 6±4ml/ml/min, p = 0.002). The sensitivity and specificity of this equation in predicting a positive renal functional outcome were 64% and 88% respectively. Use of PV:isoSK-GFR increased prediction of functional improvement (area under curve 0.93). Of note, non-RAS contralateral kidneys which improved (n = 5) also demonstrated larger PV:isoSK-GFR (15.2±16.2 ml/ml/min, p = 0.006).ConclusionThis study offers early indicators that the ratio of PV:isoSK-GFR may help identify patients with kidneys suitable for renal revascularization which could improve patient selection for a procedure associated with risks. Calculation of the PV:isoSK-GFR ratio is easy, does not require MRI contrast agent.
Project description:Purpose of reviewTakayasu's arteritis (TAK) is a large-vessel vasculitis with a chronic, indolent course affecting the aorta and its main branches. This review will describe the recent studies to develop validated outcome measures to assess TAK.Recent findingsTAK is traditionally assessed with a physician's global assessment including symptoms and signs of inflammation and vascular insufficiency, acute-phase reactants (APRs), and imaging including conventional digital subtraction angiography, computerized tomographic, and magnetic resonance angiography, and recently 18-FDG-PET. Recent attempts to develop a validated tool for disease assessment include the Indian Takayasu Clinical Activity Score (ITAS2010), which incorporates clinical signs and symptoms with APRs in a simplified and weighted adoption of the Birmingham Vasculitis Activity Score. Among biomarkers to assess clinical activity, pentraxin-3 is perhaps the most promising, but its validity and superiority against APRs in clinical practice need to be demonstrated. Patient-reported outcomes (PROs) are increasingly recognized as of substantial importance to measure in clinical trials; in addition to so-called 'generic' tools such as the SF-36 or measures of fatigue, disease-specific instruments would likely help capture aspects of TAK not measured by generic quality-of-life assessments or physician-based tools.SummaryAlthough outcome measures for TAK are not sufficiently validated, progress in the assessment of TAK is reflected in recent studies with new tools such as ITAS2010, new biomarkers, and a variety of PROs.
Project description:We present the first case report of coronary-carotid artery collateral formation in Takayasu's arteritis. There was a vasculitic involvement of both subclavian and carotid arteries with critical stenosis; cerebral perfusion was supported with collaterals arising from the mesenteric arteries and coronary artery. <Learning objective: A case of coronary-carotid artery collateral formation in Takayasu's arteritis is presented. Such an angiographic finding has not been reported previously, and this case could be the first description of coronary-carotid artery collaterals development. The learning objective of the article is to evaluate collateral circulation in Takayasu's arteritis from different vasculature including the coronary system.>.
Project description:Recent studies have provided evidence of a close link between specific microbiota and inflammatory disorders. While the vessel wall microbiota has been recently described in large vessel vasculitis (LVV) and controls, the blood microbiome in these diseases has not been previously reported (LVV). We aimed to analyse the blood microbiome profile of LVV patients (Takayasu's arteritis [TAK], giant cell arteritis [GCA]) and healthy blood donors (HD). We studied the blood samples of 13 patients with TAK (20 samples), 9 patients with GCA (11 samples) and 15 HD patients. We assessed the blood microbiome profile by sequencing the 16S rDNA blood bacterial DNA. We used linear discriminant analysis (LDA) coupled with linear discriminant effect size measurement (LEfSe) to investigate the differences in the blood microbiome profile between TAK and GCA patients. An increase in the levels of Clostridia, Cytophagia and Deltaproteobacteria and a decrease in Bacilli at the class level were found in TAK patients compared with HD patients (LDA > 2, p < 0.05). Active TAK patients had significantly lower levels of Staphylococcus compared with inactive TAK patients. Samples of GCA patients had an increased abundance of Rhodococcus and an unidentified member of the Cytophagaceae family. Microbiota of TAK compared with GCA patients was found to show higher levels of Candidatus Aquiluna and Cloacibacterium (LDA > 2; p < 0.05). Differences highlighted in the blood microbiome were also associated with a shift of bacterial predicted metabolic functions in TAK in comparison with HD. Similar results were also found in patients with active versus inactive TAK. In conclusion, patients with TAK were found to present a specific blood microbiome profile in comparison with healthy donors and GCA subjects. Significant changes in the blood microbiome profiles of TAK patients were associated with specific metabolic functions.