Acute myocardial infarction with left main coronary artery ostial negative remodelling as the first manifestation of Takayasu arteritis: a case report.

BACKGROUND: Takayasu arteritis is a chronic inflammatory disease involving the aorta and its major branches. Acute myocardial infarction rarely but not so much presents in patients with Takayasu arteritis, and the preferable revascularization strategy is still under debate. CASE PRESENTATION: A 22-year-old female with Takayasu arteritis presented with acute myocardial infarction. Coronary angiography and intravenous ultrasound (IVUS) showed that the right coronary artery (RCA) was occluded and that there was severe negative remodelling at the ostium of the left main coronary artery (LMCA). The patient was treated by primary percutaneous transluminal coronary angioplasty (PTCA) with a scoring balloon in the LMCA, without stent implantation. After 3 months of immunosuppressive medication, the patient received RCA revascularization by stenting. There was progressive external elastic membrane (EEM) enlargement of the LMCA ostium demonstrated by IVUS at 3 and 15 months post-initial PTCA. CONCLUSION: Here, we report a case of Takayasu arteritis with involvement of the coronary artery ostium. Through PTCA and long-term immunosuppressive medication, we found that coronary negative remodelling might be reversible in patients with Takayasu arteritis.

Takayasu's Arteritis from the Patients' Perspectives: Measuring the Pulse to the Patient-Reported Outcomes.

BACKGROUND: Patients with Takayasu's arteritis (TA) experience important changes in lifestyle, quality of life, and functional status due to ischemic symptoms or treatment toxicity. PURPOSE: To describe the clinical characteristics and the patient-reported outcomes (PROs), such as quality of life, disability, fatigue, and perception/impact of the disease in Mexican patients with TA. METHODS: Cross-sectional study including patients with established diagnosis of TA recruited at a tertiary care center. Demographics, comorbidities, clinical characteristics, laboratory, imaging, and treatment were retrieved. Disease activity (the Indian Takayasu Clinical Activity Score (ITAS) 2010), damage (Vasculitis Damage Index (VDI)), quality of life (Short Form 36 (SF-36)), disability (Health Assessment Questionnaire Disability Index (HAQ-DI)), fatigue (Multidimensional Fatigue Inventory-20), and patient's disease perceptions were assessed. RESULTS: Fifteen women were included, with a median age of 41 years (interquartile range (IQR) 30-45) and disease duration of 108 months (IQR 55-197). Median ITAS 2010 and VDI scores were 0 (IQR 0-2) and 3 points (IQR 2-6), respectively. Mean SF-36 score was 71.38 ± 13.39, with mean physical and mental component summaries of 66.52 ± 13.37 and 76.24 ± 14.89, respectively. HAQ-DI mean score was 0.48 ± 0.62, being grip the most affected domain. Among fatigue subscales, the higher scores were present in the physical fatigue (16.3 ± 5.8). Correlations between the HAQ-DI and the VDI score (r = 0.64, P = 0.03); between the general fatigue, score, and disease duration (r = -0.71, P = 0.01); and between the SF-36 total score and the HAQ-DI (r = -0.87, P = 0.0004) were found. CONCLUSIONS: It is important to identify disease-specific outcomes of interest to the patients to develop tools that assess them with a holistic approach.

Typical angina in a patient with Takayasu arteritis.

Takayasu arteritis (TA) is a well-reported form of large-vessel vasculitis that primarily affects the aorta and its major branches. Cardiac manifestations of TA have been reported and can include typical angina secondary to coronary artery involvement; however, typical angina in the setting normal coronary arteries is uncommon. We describe a case of typical angina in a patient with TA with normal coronary arteries secondary to diastolic hypotension, in the absence of significant aortic regurgitation, likely from poor aortic distensibility and elevated left ventricular end-diastolic pressure.

Midaortic Syndrome due to Takayasu Arteritis in a Child with Acute Decompensated Cardiac Failure Managed by an Emergency Axillo-External Iliac Artery Bypass: A Follow-Up Case Report of Long-Term Outcomes.

Midaortic syndrome (MAS) is characterized by a diffuse narrowing of the distal thoracic or abdominal aorta and is concomitant with various etiologies. The common symptoms of MAS include severe hypertension or arterial insufficiency distal to the stenosis. This includes lower extremity claudication and heart failure due to afterload mismatch. We present the case of an 8-year-old girl who developed acute decompensated cardiac, respiratory, and renal failures because of the occlusion of the descending aorta secondary to Takayasu arteritis (TA). Although thoracoabdominal-aortic bypass is usually performed for patients with MAS, the procedure was considered too invasive, given the patient's condition. Therefore, we performed an emergency axillo-external iliac artery bypass for revascularization. Subsequently, organ failure improved and she was discharged. At postoperative 10 years, an asymptomatic pseudoaneurysm was detected at the distal anastomosis, for which revision surgery was performed. Overall, the long-term prognosis was satisfactory, suggesting that this procedure is less invasive and effective for treatment of MAS due to TA, in emergencies.

Staged Combined Endovascular and Open Surgical Approach in a Patient with Takayasu's Arteritis.

Takayasu's arteritis (TA) is a systemic large vessel vasculitis that affects the aorta and its branches. Most patients with TA respond to medical therapy with a minority of patients requiring surgical intervention. In our report, we describe the case of a 59-year-old Caucasian female with TA who underwent revascularization due to cerebrovascular symptoms refractory to medical therapy. She initially presented with amaurosis fugax and developed episodes of syncope and slurred speech during corticosteroid tapering. Vascular studies showed right common carotid artery (RCCA), left internal carotid artery (LICA), and left subclavian artery (LSA) occlusion with the right vertebral artery (RVA) ostium stenosis, and retrograde flow through the left vertebral artery (LVA). The sole source of cerebral perfusion flowed through her stenosed RVA, so it was decided to first stent the RVA to restore adequate posterior cerebral circulation before creating a right subclavian artery to RCCA bypass to restore anterior circulation. This case represents the successful management of TA utilizing a two-staged combined endovascular and surgical approach.

A retrospective cohort study to assess PET-CT findings and clinical outcomes in Takayasu arteritis: does 18F-fluorodeoxyglucose uptake in arteries predict relapses?

The objective of this study is to investigate the use of PET-CT scan with 18F-fluorodeoxyglucose (18F-FDG) as a method to predict outcomes in patients with Takayasu arteritis (TAK), as well as to analyze associations between 18F-FDG PET-CT findings with disease relapses, sustained remission, new angiographic lesions, ischemic events, and changes in therapy for TAK. At baseline assessment, 36 TAK patients underwent 18F-FDG PET-CT scan and maximal standardized uptake value (SUVmax) in arteries ≥ 1.3 was predictive for clinical disease activity. Thirty-two TAK patients were then followed-up for a median 83.5 months. Twenty-three (71.9%) patients developed clinical relapses and new arterial lesions were observed in 14 (43.8%) cases. Disease relapses [85.0% vs. 50.0%, p = 0.049; odds ratio (OR): 5.667; 95% confidence interval (95 CI): 1.067-30.085] and the need for changing immunosuppressive therapy (85.0% vs. 41.7%, p = 0.018; OR: 7.933; 95CI: 1.478-42.581) were more frequently found in patients with SUVmax ≥ 1.3 at baseline compared with those presenting SUVmax < 1.3. No associations were found between SUVmax ≥ or < 1.3 in large arteries at baseline and the development of ischemic events, sustained remission or new angiographic lesions. In multivariate analysis, associations between baseline SUVmax ≥ 1.3 and disease relapses were not independent (hazard ratio: 1.07; 95 CI 0.39-2.92; p = 0.892). In conclusion, arterial SUVmax is marginally associated with disease relapses and with the need to change therapy in TAK. 18F-FDG uptake in large arteries is not associated with the development of new arterial lesions in TAK.

Clinical characteristics and long-term outcome of Takayasu arteritis in Iran: a multicentre study

Background/aim: This study aimed to evaluate the demographic, clinical, angiographic and prognostic characteristics of Takayasu arteritis (TA) in Iran. Materials and methods: A total of 75 patients with TA based on the American College of Rheumatology 1990 criteria for TA classification referred to the Rheumatology Centres, were followed-up from 1989 to 2019. Demographic, clinical, angiographic and prognostic characteristics were collected at baseline and last visit. Results: The mean age was 31.9 ± 9.8 years at the disease onset. Female to male ratio was 14. The median latency in diagnosis was 24 months. Pulse discrepancy in the arms, blood pressure discrepancy in the arms, limb claudication, hypertension and constitutional symptoms were the most common clinical features. The most common angiographic type at the time of diagnosis was Type I (42.7%). The most frequent arterial lesion was stenosis (89.4%). Subclavian, carotid and aortic arteries were the most commonly involved arteries. New lesions developed in 28.6% of patients during the 5.25-year follow-up. Vasculitis-induced chronic damage was observed in all patients. Disease activity decreased and vascular damage remained stable throughout the follow-up period. Conclusions: The clinical features and angiographic type of TA in Iran are different from most Asian countries. Differences in angiographic and clinical features may lead to delayed diagnosis. The issue of delay in diagnosis should create awareness among health care providers that TA is not a very rare disease in Iranians and failure to pay attention to warning symptoms may delay the diagnosis.

Nailfold videocapillaroscopy changes in Takayasu arteritis and their association with disease activity and subclavian artery involvement.

INTRODUCTION: Takayasu arteritis (TA) is a large vessel vasculitis characterized by the involvement of the aorta and its branches. In the present study, the nailfold videocapillaroscopy (NVC) findings of the TA patients were evaluated and compared to healthy individuals. Additionally, the association of NVC abnormalities with disease activity and subclavian artery involvement was also investigated. METHOD: In the present study, NVC changes of 15 TA patients and 15 age- and sex-matched healthy controls were compared. The frequency of hypertension, dyslipidemia, and smoking status was not different between the case and control group. Capillary density, avascular area, tortuosity, micro-hemorrhages, and ramification were investigated. Among capillaries' diameters; capillary length and width, arterial and venous limbs diameters were also compared between two groups. RESULTS: The capillary length and venous limb diameter were lower in TA patients compared to control cases (P-value: 0.026 and 0.049, respectively). Moreover, TA patients have more tortuous capillaries (P-value: 0.035). Among TA patients; capillary length, width, arterial and venous limbs diameter was lower in hands with subclavian involvement (P-value: 0.014, 0.034, 0.009 and 0.022, respectively). Furthermore, the arterial and venous limbs diameter were lower in TA patients with active disease compared to patients with inactive disease (P-value: 0.018 and 0.049, respectively). CONCLUSION: In the present study, we have shown that the hands with subclavian artery involvement have short, thin and tortuous capillaries which could be secondary to hypoperfusion. To the best of our knowledge, this is the first report describing NVC changes in TA.

The C-Reactive Protein/Albumin Ratio and Complete Blood Count Parameters as Indicators of Disease Activity in Patients with Takayasu Arteritis.

BACKGROUND This study aimed to evaluate the ratio of C-reactive protein (CRP) to albumin, inflammatory markers, and parameters from the complete blood count (CBC) in patients with Takayasu arteritis and the association with disease activity. MATERIAL AND METHODS A retrospective study included thirty-two patients with Takayasu arteritis and 32 healthy controls. Clinical and demographic characteristics of patients with Takayasu arteritis were recorded at baseline, before medication and on remission. Similar data were obtained for the controls at recruitment. Remission was defined as more than six months of stable disease without new vascular lesions in patients who previously had active disease. Kerr's criteria were used to define active Takayasu arteritis. RESULTS In patients with Takayasu arteritis, the erythrocyte sedimentation rate (ESR), CRP, CRP/albumin ratio, red cell distribution width (RDW), neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and monocyte-lymphocyte ratio (MLR) were significantly higher, and albumin and MPV were significantly lower compared with controls. The ESR, CRP, CRP/albumin ratio, NLR, PLR, and MLR were decreased in remission, whereas MPV was increased. CRP and the CRP/albumin ratio were positively correlated and albumin and MPV were negatively correlated with disease activity. The CRP/albumin ratio had the highest correlation with disease activity in Takayasu arteritis. The CRP/albumin ratio, RDW, NLR, PLR, and MLR were positively correlated with CRP and ESR. CONCLUSIONS The CRP/albumin ratio, RDW, NLR, PLR, MLR, and MPV were markers of remission of active disease, and the CRP/albumin ratio, total albumin, and MPV were markers of disease activity in Takayasu arteritis.

Increased Arterial Stiffness Adversely Affects Left Ventricular Mechanics in Patients With Pediatric Takayasu Arteritis From a Toronto Cohort.

BACKGROUND/OBJECTIVE: Takayasu arteritis (TA) is characterized by extensive aortic, large and midsize arterial wall inflammation. The aim of this study was to assess the morphological and elastic properties of the aorta and large arteries and the impact on left ventricular (LV) mechanics in children with TA. METHODS: Seven pediatric TA patients (6 female patients, 13.8 ± 3.2 years) were assessed with magnetic resonance imaging, vascular ultrasound, applanation tonometry, and echocardiography from February 2015 until July 2017 and compared with 7 age- and sex-matched controls. Takayasu arteritis disease activity was assessed clinically by the Pediatric Vasculitis Activity Score (PVAS). RESULTS: Pediatric TA patients showed increased carotid-to-radial artery pulse wave velocity (8.1 ± 1.8 vs. 6.4 ± 0.6 m/s, p = 0.03) and increased carotid-to-femoral artery pulse wave velocity (8.3 ± 1.9 vs. 5.1 ± 0.8 m/s, p < 0.01) when compared with controls. Patients demonstrated increased LV mass index (74.3 ± 18.8 vs. 56.3 ± 10.9 g/m, p = 0.04), altered myocardial deformation with increased basal rotation (-9.8 ± 4.5 vs. -4.0 ± 2.0 degrees, p = 0.01) and torsion (19.9 ± 8.1 vs. 9.1 ± 3.1 degrees, p = 0.01), and impaired LV diastolic function with decreased mitral valve E/A ratio (1.45 ± 0.17 vs. 2.40 ± 0.84, p = 0.01), increased mitral valve E/E' ratio (6.8 ± 1.4 vs. 4.9 ± 0.7, p < 0.01), and increased pulmonary vein A-wave velocity (26.7 ± 5.7 vs. 16.8 ± 3.3 cm/s, p = 0.03). Carotid-to-radial artery pulse wave velocity was associated with systolic (R = 0.94, p < 0.01), diastolic (R = 0.85, p = 0.02), and mean blood pressure (R = 0.91, p < 0.01), as well as disease activity by PVAS (R = 0.75, p = 0.05). The PVAS was associated with carotid-to-radial artery pulse wave velocity (R = 0.75, p = 0.05), as well as systolic (R = 0.84, p = 0.02), diastolic (R = 0.82, p = 0.03), and mean blood pressure (R = 0.84, p = 0.02). CONCLUSIONS: Increased arterial stiffness is present in pediatric TA patients and associated with increased blood pressure and TA disease activity. Pediatric TA patients demonstrate altered LV mechanics, LV hypertrophy, and impaired diastolic function.

Aggressive progression of Takayasu's arteritis in infancy: a case report.

Takayasu's arteritis (TA) affects the aorta and its branches. Immunosuppressants are the usual course of therapy, while surgery has been used in acute cases. There is only scant information on TA in infancy, and the nonspecific symptoms in the initial stage of the disease make the diagnosis difficult and delayed, thus increasing the mortality rate. We describe a case of aggressive progression of TA in an infant. This child was the youngest to be affected with the disease as reported in the literature. A 3.5-month-old boy presented with cyanosis of both legs, tachycardia and antithrombin III deficiency. Computed tomography angiography (CTA) revealed thrombosis of distal aorta and both iliac arteries. Thrombectomy was performed at the level of both common femoral arteries. In addition, thigh amputation of the left leg had to be performed. TA was diagnosed postmortem with thrombosis of the distal aorta, its branches and upper mesenteric artery which was not occluded on previous CTA, glomerulonephritis and pulmonary parenchymal granulomatous infiltrations.

NMR-Based Serum Metabolomics of Patients with Takayasu Arteritis: Relationship with Disease Activity.

Takayasu arteritis (TA) is a large vessel vasculitis of unknown pathogenesis. Assessment of disease activity is a challenge, and there is an unmet need for relevant biomarker(s). In our previous study, NMR based serum metabolomics had revealed distinctive metabolic signatures in TA patients compared with age/sex matched healthy controls and systemic lupus erythematosus (SLE). In this study we investigate whether the metabolites correlate with disease activity. Patients with TA fulfilling American College of Rheumatology (ACR) criteria were enrolled, and disease activity was assessed using Indian Takayasu Clinical Activity Score using acute phase reactant-erythrocyte sedimentation rate [ITAS-A (ESR)]. Sera were analyzed using 800 MHz NMR spectrometer to identify metabolites [based on partial least squares discriminant analysis (PLS-DA) VIP (variable importance in projection) score > 1.0 and permutation test, p-value <0.01]. 45 active and 53 inactive TA patients with median age 27 [(IQR) 22-35 years] and 27 [(IQR) 23-37 years], female to male ratio 3.5:1 and 4.9:1, and median duration of illness 5 [(IQR) 2-9 years] and 3 [(IQR) 1-6 years], respectively, were enrolled. The key metabolites with highest discriminatory potential in active TA (ITAS-A ≥ 4) were glutamate and N-acetyl glycoprotein (NAG), both elevated, with area under the curve 0.775 and 0.769 ( p-value <0.001). On follow up assessment, metabolic spectra started to differ with change in disease activity. This large cohort of patients revealed metabolic profiles discriminating between clinically active and inactive TA patients. It suggests glutamate and NAG have strong potential as biomarkers for disease activity in TA and may serve as a guide to therapy. We are now working to further validate these results in longitudinal studies.

Accelerated atheromatosis and arteriosclerosis in primary systemic vasculitides: current evidence and future perspectives.

PURPOSE OF REVIEW: Primary systemic vasculitides (PSV) encompass a subset of autoimmune diseases, characterized by inflammation of blood vessels. Atheromatosis and arteriosclerosis may be accelerated in several PSV and account for the increased rate of cardiovascular morbidity that some exhibit. We aimed to summarize recent studies reporting on the acceleration of atheromatosis and/or arteriosclerosis in each type of PSV, using state-of-the-art noninvasive vascular biomarkers with clinical value as end points. RECENT FINDINGS: Limited number of PSV patients and methodology limitations reduce the value of many published studies. Accelerated atheromatosis, as measured by the use of carotid ultrasonagraphy (plaques and intimal-medial thickening) and increased arterial stiffening, as measured by the use of applanation tonometry (carotid to femoral pulse wave velocity), are currenly well established in Takayasu arteritis, Kawasaki disease and Behcet's disease. The association of atheromatosis and arteriosclerosis with polyarteritis nodosa and small vessel vasculitides remains less established and studied, so far. SUMMARY: Accelerated atheromatosis and arteriosclerosis or arteriosclerosis are established in some PSV. The potential clinical value of easy-to-measure and clinically useful noninvasive vascular biomarkes prompts the need for large prospective cohorts in order to provide useful future guidance regarding the prognosis and treatment of PSV patients.

mTOR pathway is activated in endothelial cells from patients with Takayasu arteritis and is modulated by serum immunoglobulin G.

Objectives: Takayasu arteritis (TA) and GCA are large-vessel vasculitides characterized by vascular remodelling involving endothelial cells (ECs) and vascular smooth muscle cells. Mammalian target of rapamycin (mTOR) pathway has been involved in vascular remodelling. We hypothesized that the mTOR pathway was involved in the pathogenesis of large-vessel vasculitis. Methods: We used IF analysis on aortic and temporal artery biopsies from patients with TA and GCA to assess the involvement of the mTOR pathway and searched for antibodies targeting ECs in serum by IIF and cellular ELISA. We evaluated in vitro the effect of purified IgG from patients on mTOR pathway activation and cell proliferation. Results: IF analyses on tissues revealed that both mTORC1 and mTORC2 are activated specifically in ECs from TA patients but not in ECs from GCA patients and healthy controls (HCs). Using IIF and ELISA, we observed higher levels of antibodies binding to ECs in TA patients compared with GCA patients and HCs. Using western blot, we demonstrated that purified IgG from TA patients caused mTORC1 activation in ECs, whereas this effect was not observed with purified IgG from GCA patients or HCs. Purified IgG from TA patients induced a significant EC proliferation compared with to GCA and HC IgG, and this effect was decreased after EC exposure with sirolimus, a specific mTOR inhibitor and PI3K inhibitor. Conclusion: Our results suggest that antibodies targeting ECs drive endothelial remodelling in TA through activation of the mTOR pathway, but not in GCA. Inhibition of the mTOR pathway could represent a therapeutic option in TA.

Simultaneous Multi-Supra-Aortic Artery Bypass Successfully Implemented in 17 Patients with Type I Takayasu Arteritis.

OBJECTIVE: Type I Takayasu arteritis is common in Chinese patients and usually leads to cerebral ischaemia. There are limited data regarding simultaneous multi-supra-aortic artery (SMSAA) bypass in patients with type I Takayasu arteritis. The present study shares experience of using this method. METHODS: Seventeen patients with Takayasu arteritis who received SMSAA bypass from June 2012 to March 2016 were included in this analysis. Demographic features and basic information were recorded. Follow up results, including major complications, patency rates, and survival rates, were also obtained. The EQ-5D questionnaire was also administered to evaluate the quality of life of patients. RESULTS: Among these 17 patients, there were 77 supra-aortic arteries affected by TA and 37 arteries were reconstructed, 10 with great saphenous vein and 27 with artificial blood vessels. All the operations were technical successes and the mean operation time was 396.6 ± 93.4 min. The patients were followed for a mean of 25.2 ± 12.3 months. Two graft restenoses were found, and both were successfully recanalised by balloon dilatation. One patient suffered a minor stroke but was free from obvious sequelae. No major complications, recurrent symptoms, or deaths occurred during the follow up period. The EQ-5D questionnaire scores were significantly improved after the operation compared with the pre-operative scores (87.14 ± 8.25 vs. 58.93 ± 14.4, p < .01), and all the patients enjoyed a satisfactory quality of life. CONCLUSION: SMSAA bypass in type I Takayasu arteritis can be effectively and safely performed with rigorous peri-operative management. Maintaining the patency of the bypass graft relieves the symptoms of cerebral ischaemia and helps sustain patients' quality of life.

Health-related outcomes of importance to patients with Takayasu's arteritis.

OBJECTIVES: The need to include patients' perspectives as key outcomes in clinical trials is widely accepted. No disease-specific patient-reported outcomes have been developed in Takayasu's arteritis. This project was designed to identify outcomes of importance to patients with Takayasu's arteritis during active disease and remission across 2 different cultures. METHODS: Patients with Takayasu's arteritis from the US and Turkey were recruited to participate in semi-structured, one-on-one interviews or focus groups. The interviews and group sessions were recorded, transcribed, and entered into an Nvivo database. A line-by-line review of narrative data was used to develop themes describing the impact of Takayasu's arteritis on patients' life. US Patients were invited to freelist terms that they associated with disease states (active disease and remission). The Smith's Salience Index (SSI) was used to identify the most salient terms. RESULTS: Results. A total of 31 patients with Takayasu's arteritis participated in this study. Interviews and focus groups identified pain, fatigue, and emotional impact as common themes. Outcomes did not differ between the 2 countries. The most salient terms identified through freelisting were pain/discomfort and fatigue/low energy levels (SSI=0.56 and 0.33, respectively) during active disease and pain/discomfort and emotional impact (SSI=0.51 and 0.37, respectively) during remission. CONCLUSIONS: Patients with Takayasu's arteritis report a range of disease-specific symptoms across different cultures and disease states that are generally not specifically captured by generic patient-reported outcome tools currently used in research. Identifying disease-specific outcomes would advance clinical trials methodology to best capture the full spectrum of disease activity in Takayasu's arteritis.

Cardiopulmonary involvement in Takayasu's arteritis.

OBJECTIVES: To evaluate cardiopulmonary (CP) involvement in patients with Takayasu's arteritis (TAK) and assess the impact on disease outcomes. METHODS: A retrospective cohort of patients with newly diagnosed TAK from 1984 to 2009 was assembled. Demographics, baseline disease characteristics, relapse events, surgeries and mortality were abstracted from direct medical record review. Angiograms, advanced imaging and cardiac studies were reviewed for evidence of CP involvement. Cox models with time-dependent covariates were used to assess the association between CP involvement and outcomes. RESULTS: A total of 124 patients with TAK were identified. Forty-five (36%) patients had at least one objective CP abnormality observed within 6 months of TAK diagnosis. Age at diagnosis was higher in those with CP involvement than those without (34.6 vs 30.1 yrs; p=0.04). Baseline characteristics and symptoms were similar, except shortness of breath, which was more frequently observed at TAK diagnosis in patients with CP involvement compared to those without (53% vs 21%; p=0.001). Composite CP involvement was not associated with risk of first surgery [Hazard ratio (95% CI): 1.21 (0.64-2.30); p=0.56]. However, pulmonary hypertension (PH) on echocardiogram was significantly associated with risk of first surgery [HR (95% CI): 12.9 (1.86- 89.14); p=0.01]. CP involvement was not significantly associated with mortality [HR (95% CI): 2.51 (0.45- 14.02); p=0.29]. CONCLUSIONS: Cardiopulmonary abnormalities in TAK are common at the time of initial presentation. In this population, the presence of PH predicted a 13-fold increased risk for vascular or valvular surgery. In this cohort, the presence of CP involvement did not increase mortality.

Concomitant Hybrid Arch Vessel Reconstruction and Intracranial Clot Retrieval in Takayasu's Arteritis Presenting with Acute Cerebral Ischemia: A Case Report.

BACKGROUND: Type 1 Takayasu's arteritis (TA) predominantly affects the supra-aortic branches. Occlusive disease can cause catastrophic complications such as stroke and death. Open and endovascular techniques of revascularization are described for extracranial disease. There is currently no default choice of surgical intervention. Furthermore, the management of distal intracranial complications has not been described. METHODS: A 25-year-old woman with known TA on immunosuppression was presented with acute left middle cerebral artery (MCA) stroke. Computed tomography angiogram and formal cerebral digital subtraction angiography demonstrated critical stenoses or occlusion of all arch vessels except for the left subclavian artery (SCA), which was also stenosed at the origin. The left vertebral artery was the sole inflow to the brain. Furthermore, there was acute embolus in M1 of MCA and A1 of the anterior cerebral artery (ACA). Surgical access was achieved by bilateral common carotid artery (CCA) and left SCA exposures. Extracranial revascularization was performed using a hybrid technique: (i) left SCA covered stent; (ii) left SCA to right CCA bypass; (iii) left CCA thrombectomy; (iv) left CCA covered stent; and (v) left CCA interposition bypass. This was immediately followed by clot retrieval in the MCA and ACA territory for intracranial revascularization. RESULTS: There were no major postoperative complications and the patient made an excellent neurologic recovery. CONCLUSIONS: Hybrid vascular reconstruction for extracranial disease with concomitant clot retrieval for intracranial disease is a novel approach to achieving emergency revascularization in active TA.

Takayasu arteritis: an update

Takayasu arteritis (TAK) is a challenging chronic, granulomatous, large-vessel systemic vasculitis, mostly due to difficulties in early diagnosis and assessing actual disease activity. Since there are no specific diagnostic laboratory tests, biomarkers, or autoantibodies, many patients experience considerable delay in diagnosis. Remembering the possibility of TAK together with the use of acute phase responses and appropriate imaging studies may be helpful for early diagnosis. Since there may be discrepancies between systemic and vascular wall inflammation, using only acute phase responses is not reliable in assessing current disease activity. Therefore, physical examination and new imaging findings should also be used to assess current disease activity. Despite its limitations, the Indian Takayasu Clinical Activity Score (ITAS2010) may also be helpful for this purpose. The rationale of medical treatment is to suppress both vascular and systemic inflammation with appropriate systemic immunosuppression, including corticosteroids and conventional immunosuppressive agents. In cases of refractory disease activity, leflunomide and biologic agents such as TNF inhibitors and tocilizumab may be tried. In selected cases with persistent lesions that cannot be reversed with medical treatment, endovascular interventions including balloon angioplasty, stent and stent graft replacement, or surgery may be tried. However, such procedures should be performed after suppression of inflammation, i.e. during inactive disease. Prognosis of TAK is probably getting better with lower mortality rates reported in recent years, probably due to the use of more effective medical treatments as well as the use of endovascular interventions when necessary and available.

[Pulmonary cavities with Takayasu arteritis: report of 3 cases and literature review].

Objective: To analyze the clinical features of 3 cases of Takayasu arteritis(TA) with pulmonary cavities on chest computed tomography(CT). Methods: The clinical data of 3 TA patients with cavities on the chest CT who were admitted into Beijing Chaoyang Hospital were retrospectively analyzed. A literature search was performed with "Takayasu arteritis" and "pulmonary" as the key words in China Knowledge Resource Intergrated Database (CNKI) and Pubmed Database for publications from Jan 1, 2000 to Dec. 31,2017. The relevant literatures were reviewed. Results: Among the 3 patients, 2 were males and 1 was female, aging 49, 28 and 28 years, respectively. They presented with cough, fever and chest pain, and chest CT showed cavities, single or multiple, either with thick or thin wall, or wedge-shaped consolidation, residual stripes after being absorbed, and one case had pulmonary biopsy results which showed hemorrhagic infarction. They were all misdiagnosed before as pneumonia, pulmonary tuberculosis, pulmonary thromboembolism. After being treated by combination therapy of glucocorticoids and immunosuppressive agents, the disease improved significantly. A total of 777 cases with TA involving pulmonary arteries were reported, from which 13 cases with involvement of pulmonary parenchyma were described. Therefore total 16 cases including the 3 cases in this article were included for analysis. Twelve cases showed patchy or wedge-shaped ground-glass opacity and consolidation, and peripheral lung stripes remained after being absorbed. Two cases showed pleural effusion, and 4 cases showed cavities, 3 cases were misdiagnosed as pulmonary tuberculosis, 7 as pulmonary infection, and 5 as pulmonary thromboembolism. Conclusions: TA with pulmonary arteries involved is susceptible to be misdiagnosed and missed, and therefore, in patients with cough, hemoptysis, chest pain and cavities in pulmonary parenchyma, TA should be suspected. Early diagnosis and appropriate treatment can lead to a better prognosis.