Survival and causes of death for Takayasu's arteritis in Korea: A retrospective population-based study.

OBJECTIVE: Few studies have evaluated survival of Takayasu's arteritis (TAK; M31.4) in Korea. The purpose of this study was to assess the survival rate (SR) and causes of death for TAK. METHODS: Newly diagnosed TAK data (N = 2731) were collected from the National Health Insurance Service in Korea from 2006 through 2017. The Kaplan-Meier method was used. Korean death data was used from 2006 through 2018. RESULTS: The mean age was 48.1 (±16.9) years. The proportion of female patients was 74.4%. The most common cause of death in TAK was diseases of the circulatory system. The 1-, 3-, 5-, and 10-year SRs were 97.5%, 94.7%, 91.7%, and 84.7%, respectively. The 1-, 3-, 5-, and 10-year SRs by gender were 97.8%, 95.6%, 92.9%, and 86.3%, respectively, among females and 96.8%, 92.2%, 88.4%s and 79.7%, respectively, among males (P < .001). CONCLUSIONS: The overall 10-year SR was about 85%. The 10-year SR in males was lower than that in females. The most common cause of death in TAK was diseases of the circulatory system.

Morbidity and Mortality of Large-Vessel Vasculitides.

PURPOSE OF REVIEW: Giant cell arteritis (GCA) and Takayasu arteritis (TAK) comprise the primary systemic large-vessel vasculitides. In these conditions, arterial stenosis, occlusion, aneurysm, and dissection can lead to severe disease-related consequences. This review focuses on disease-related manifestations of GCA and TAK, emphasizing the impact of these findings on long-term morbidity and mortality. RECENT FINDINGS: Vision loss remains a main contributor of morbidity in GCA. Non-invasive imaging allows for recognition of aortic disease in GCA but monitoring and intervention guidelines require further development. TAK represents a severe disease of early-onset with high risk of morbidity due to aortic, pulmonary, cardiovascular, and neurologic involvement. Overall, patients with GCA have similar mortality rates to comparators but mortality is notably higher than the general population in TAK. A multidisciplinary approach of expert subspecialists is required to assist with the complex care of patients with GCA and TAK in order to appropriately surveil, identify, and address the multi-faceted co-morbidities of these diseases.

Association between the antineutrophil cytoplasmic antibody and late coronary arterial occlusive disease in patients with Takayasu arteritis.

BACKGROUND: No study has provided evidence of the clinical significance of the antineutrophil cytoplasmic antibody (ANCA) in patients with Takayasu arteritis (TAK). Therefore, we investigated the frequency of ANCA positivity and its clinical implications in patients with TAK. METHODS: We retrospectively reviewed the medical records of 121 patients with established TAK, who had results for ANCA status at diagnosis. We collected demographic and clinical data and the ANCA results at diagnosis. Additionally, we obtained information on patients' medications and complications during follow-up. Early coronary arterial occlusive disease (CAOD) and late CAOD were defined based on a 30-day interval after TAK classification. The chi-square test, Fisher's exact test, Mann-Whitney test, and Kaplan-Meier survival analysis were used to analyze the data. RESULTS: The patients' mean age was 44.6 years, and 21 patients were men (17.4%). ANCA was detected in 8 patients (6.6%), of which 2 had both the myeloperoxidase ANCA (or perinuclear ANCA) and proteinase 3 ANCA (or cytoplasmic ANCA). Early CAOD was observed in 10 patients (8.3%), and late CAOD was found in 9 patients (7.4%). In the comparative analysis, the proportion of late CAOD exhibited a tendency to increase in the ANCA-positive group compared to that in the ANCA-negative group. Kaplan-Meier analysis showed that patients with ANCA exhibited a lower cumulative late CAOD-free survival rate than those without ANCA (p=0.012). When the algorithm for the classification of ANCA-associated vasculitis (AAV) proposed by the European Medicine Agency in 2007 was applied to 8 patients with ANCA, all were not reclassified as having AAV. CONCLUSIONS: ANCA can be detected in a minority of patients with established TAK, and it may not contribute to the reclassification of AAV. Furthermore, ANCA positivity may be associated with late CAOD in patients with TAK.

Mortality and cardiovascular morbidity among patients diagnosed with Takayasu's arteritis: a Danish nationwide cohort study.

OBJECTIVES: To assess the long-term mortality and risk of cardiovascular events (CVE) among Danish patients with Takayasu's arteritis (TAK). METHODS: Administrative registers with nationwide coverage were used to identify patients diagnosed with TAK in Denmark during 1994-2014 and construct an age- and gender-matched cohort of population-controls. CVE were identified by means of hospital discharge diagnoses and categorised as major or minor, based on severity. Cox regression analyses were used to calculate hazard ratios (HRs) for death and first-time hospitalisations for CVE as a measure of relative risk. RESULTS: 79 patients with TAK were identified, corresponding to an incidence rate of 0.7 (95% confidence interval (CI): 0.6-0.9)/million/year. Median duration of follow-up in the TAK cohort was 6.4 (IQR: 3.7-11) years. Mortality was significantly higher among the TAK patients than among the population controls during the first 3 years of follow-up [HR for death: 8.0 (95% CI: 3.0-21)], but not after >3 years [HR for death: 0.5 (95% CI: 0.1-3.5)]. Risk of CVE was significantly increased among TAK patients after ≤3 years [HR for major CVE: 12 (95% CI: 3.8-37), HR for minor CVE: 19 (95% CI: 7.5-50)] as well as after >3 years [HR for major CVE: 7.6 (95% CI: 2.8-21), HR for minor CVE: 3.0 (95% CI: 1.01-9.0)]. CONCLUSIONS: Compared to the general population, patients with TAK experience markedly increased mortality during early follow-up periods. The long-term risk of CVE is high among patients affected by the disease.

Pulmonary Artery Involvement in Patients with Takayasu Arteritis.

OBJECTIVE: This study was performed to explore the clinical manifestations and longterm prognosis in patients with Takayasu arteritis (TA) with pulmonary artery involvement (PAI). METHODS: The medical records of 194 patients with TA who underwent traditional catheter angiography or computed tomography of pulmonary artery from 2009 to 2016 were retrospectively reviewed. The clinical manifestations, angiographic features, and mortality of 128 patients with TA with PAI were further analyzed. RESULTS: Patients with TA with PAI had a higher risk of pulmonary hypertension (PH) than patients with TA alone (61.7% vs 7.6%, p < 0.001). Patients with PAI and PH more frequently developed dyspnea, hemoptysis, and lower limbs edema (all p < 0.05) than those without PH. Patients with PH also had a higher incidence of bilateral PAI (84.8% vs 34.7%, p < 0.001) and a higher pulmonary artery obstruction index [23 (interquartile range 20-27) vs 10 (6-15), p < 0.001]. Left heart disease was presented in 39 (30.5%) patients with TA with PAI. During the median followup of 38 (21-58) months, 19 and 2 deaths occurred among patients with and without PH, respectively. Among patients with PAI, the mortality rate was 7 times higher in patients with than without PH (p = 0.009). Independent predictors of mortality were the disease duration (p = 0.047), New York Heart Association class III/IV (p = 0.019), right ventricular systolic dysfunction (p = 0.019), and respiratory failure (p = 0.007). CONCLUSION: Patients with TA with PAI have a higher risk of developing PH than patients with TA alone. The presence of PH in patients with PAI increases the risk of early mortality.

Surgery and Endovascular Management in Patients With Takayasu's Arteritis: A Ten-Year Retrospective Study.

BACKGROUND: Takayasu's arteritis (TA) is an uncommon chronic vasculitis, and there is a lack of long-term large cohort studies regarding the optimal revascularization outcomes of patients with TA. METHODS: One hundred and sixteen patients with TA who underwent surgery or endovascular repair over a 10-year period were studied retrospectively. One hundred and fifty-four vascular procedures were performed consisting of 69 open and 85 endovascular repairs. RESULTS: After a mean follow-up period of 48.5 ± 38.5 months, three cases each of cerebrovascular accident (CVA) and death occurred in the open repair group while two cases of CVA and 4 deaths were observed in the endovascular repair group. At 1, 3, 5, and 10 years of follow-up, the primary patency rates were 97.3%, 86.2%, 70.5%, and 48.8% in the open repair group and 93.3%, 73.1%, 57.5%, and 31.8% in the endovascular repair group, respectively. The primary assisted patency rates were 98.2%, 90.3%, 73.2%, and 47.2% in the open repair group and 95%, 81%, 64.4%, and 35% in the endovascular repair group, respectively. The secondary patency rates were 98.2%, 96.1%, 83.5%, and 56.2% in the open repair group and 98.7%, 86%, 71.2%, and 44.9% in the endovascular repair group, respectively. The cumulative survival rates were 96.9%, 96.9%, 96.9%, and 90% in the open repair group and 97.4%, 97.4%, 88%, and 79.2% in the endovascular repair group, respectively. CONCLUSIONS: Both surgical repair and endovascular management are safe and efficient modes of treatment of TA. Surgical repair showed long-term durability, and it is suitable for complex lesions and failed cases of endovascular management.

Clinical Course, Management, and Outcomes of Pediatric Takayasu Arteritis Initially Presenting With Hypertension: A 16-year overview.

BACKGROUND: To investigate the clinical features, management, and outcomes of childhood Takayasu arteritis (c-TA) initially presenting with hypertension. METHODS: This study retrospectively reviewed medical charts of 96 inpatient c-TA cases from January 2002 to December 2016, with 5 additional patients being prospectively recruited from January 2017 to December 2017. Data were compared between c-TA groups initially presenting with and without hypertension. Blood pressure (BP) control, event-free survival, and associated risk factors were assessed by logistic regression, Kaplan-Meier survival curve, and COX regression models. RESULTS: The hypertensive cohort (N = 71, 28.2% males) as compared with non-hypertensive cohort had significantly fewer active diseases; fewer episodes of claudication, syncope, blurred vision, and myocardial ischemia; and fewer systemic symptoms (P < 0.05). The hypertensive group presented with more localized abdominal lesions (OR = 14.4, P = 0.001) and limited supradiaphragmatic arterial involvement. Renovascular disease (P = 0.001) and revascularization (P = 0.006) were associated with hypertension. At the median 3-year follow-up, 53% of hypertensive patients achieved BP control and 39% experienced events including vascular complications, flares, or death. The 1-, 3-, 5-, and 10-year event-free survival were 78.7% (95% CI: 65.7%-87.2%), 63.0% (95% CI: 48.1%-74.7%), 48.9% (95% CI: 32.0%-63.8%), and 31.6% (95% CI: 13.8%-51.2%), higher than in non-hypertensive group (P = 0.014). Heart failure, stroke, and body mass index <18.5 kg/m2 were prognostic factors for events. Intervention and baseline systolic BP were independent factors for BP control (P < 0.05). CONCLUSIONS: Majority of c-TA has hypertension, presenting with a more quiescent disease without typical systemic and/or ischemia symptoms, more localized abdominal lesions, higher proportion of revascularizations and better event-free survival. Three-year BP control is more than 50%. Intervention particularly on renal artery is beneficial for BP control and decreased events. CLINICAL TRIAL REGISTRATION: Trial Number: NCT03199183.

Overall survival and mortality risk factors in Takayasu's arteritis: A multicenter study of 318 patients.

OBJECTIVE: To report the long term mortality in Takayasu arteritis (TA) and to identify prognosis factors. METHODS: We analyzed the causes of death and the factors associated with mortality in a cohort of 318 patients [median age at diagnosis was 36 [25-47] years and 276 (86%) patients were women] fulfilling American College of Rheumatology and/or Ishikawa criteria of TA. A prognostic score for death and vascular complications was elaborated based on a multivariate model. RESULTS: Among 318 TA patients, 16 (5%) died after a median [IQR] follow-up of 6.1 [2.8-13.0] years. The median age at death was 38 [25-47] years with 88% of women. Main causes of death included mesenteric ischemia (n = 4, 25%) and aortic aneurysm rupture (n = 4, 25%). The mortality rate at 5 and 10 years was of 1.9% and 3.9%, respectively. Caucasians (p = 0.049) and smokers (p = 0.002) TA patients were more likely to die. There was an increased mortality in TA (SMR with 95% confidence interval, 2.73 [1.69-4.22]) as compared to age and sex matched healthy controls. We defined high risk patients for death and vascular complications according to the presence of two of the following factors (i.e a progressive clinical course, thoracic aorta involvement and/or retinopathy). In the high risk TA group, the 5-year incidence of death and vascular complication was 48.5% compared to 21.6% (p = 0.001) in those with low risk. CONCLUSION: The overall mortality in our Takayasu cohort was 5% after a median follow-up of 6.1 years. We identified specific characteristics that distinguish TA patients at highest risk for death and vascular complications.

Clinical presentation, treatment and outcome of Takayasu's arteritis in southern Chinese: a multicenter retrospective study.

To study the clinical presentation, treatment and outcome of southern Chinese patients with Takayasu's arteritis (TA). This is a retrospective chart review study of 78 patients managed in 14 public hospitals in Hong Kong between the years 2000 and 2010. Patients were identified from the hospital registry using the ICD-10 diagnostic code of the disease. The classification of TA was based on the American College of Rheumatology (ACR) or modified Ichikawa's criteria. Demographic data, clinical presentation, angiographic findings, pattern of vascular involvement (Numano's classification), treatment and outcome of these patients were presented. 78 patients were studied (82% women, age at presentation 34.2 ± 14 years). The estimated point prevalence of TA was 11/million population. The commonest initial manifestations were hypertension (62%) and vascular ischemic symptoms (38%). Systemic symptoms occurred in nine (12%) patients only. The proportion of patients fulfilling the angiographic subtypes of the Numano's classification was: types I (13%), IIa (4%), IIb (12%), III (12%), IV (20%) and V (39%), respectively. Thirty-two patients (41%) were treated with high-dose glucocorticoids (GCs) and 22 patients (28%) received additional non-GC immunosuppressive drugs. Vascular complications occurred in 26 (33%) patients and revascularization surgery was performed in 23(29%) patients. Three (4%) patients died of vascular complication at a median of 8 years after disease onset. TA is rare in southern Chinese patients of Hong Kong. Most patients present with ischemic symptoms during the stenotic phase of the disease. Although mortality is low, a significant proportion of patients developed vascular stenosis that required surgical interventions. More awareness of TA as a differential diagnosis of non-specific systemic symptoms with elevated inflammatory markers in younger patients is needed for earlier diagnosis.

Efficacy of tocilizumab in Takayasu arteritis: Multicenter retrospective study of 46 patients.

OBJECTIVES: To assess the efficacy of tocilizumab in patients with Takayasu arteritis (TA). METHODS: We conducted a retrospective multicenter study in 46 TA patients treated with tocilizumab. We analyzed factors associated with response to tocilizumab (assessed using NIH score). RESULTS: Forty-six patients with TA were included, with a median age of 43 years [29-54], and 35 (76%) females. We observed a decrease in the median NIH scale (from 3 [2-3] at baseline to 0 [0-1] and 0 at 3 and 6 months, respectively; p < 0.0001). The daily prednisone dose also decreased from 15 mg [8-19] at baseline to 4 mg [5-21] and 5 mg [4.5-9] at 3 and 6 months, respectively (p < 0.0001) under tocilizumab. The overall tocilizumab failure free survival was 81% [CI 95%; 0.7-0.95], 72% [CI 95%; 0.55-0.95] and 48% [CI 95%; 0.2-0.1] at 12, 24 and 48 months, respectively. The presence of constitutional symptoms (HR 5.6 [CI 95%; 1.08-29], p = 0.041), and C-reactive protein level (HR 1.16 [CI 95%; 1.01-1.31], P = 0.003) at the time of tocilizumab initiation were significantly associated with tocilizumab event-free survival. The event-free survival was significantly better under tocilizumab therapy in comparison to DMARDs (p = 0.02). CONCLUSION: This large multicenter study shows that tocilizumab is efficient and may reduce the incidence of relapses in TA.

Endovascular Versus Open Surgical Intervention in Patients with Takayasu's Arteritis: A Meta-analysis.

OBJECTIVE/BACKGROUND: Although medical treatment has advanced, surgical treatment is needed to control symptoms of Takayasu's arteritis (TA), such as angina, stroke, hypertension, or claudication. Endovascular or open surgical intervention is performed; however, there are few comparative studies on these methods. This meta-analysis and systematic review aimed to examine the outcome of surgical treatment of TA. METHODS: A meta-analysis comparing outcomes of endovascular and open surgical intervention was performed using MEDLINE and Embase. This meta-analysis included only observational studies, and the evidence level was low to moderate. Data were pooled and analysed using a fixed or random effects model with the I2 statistic. RESULTS: The included studies involved a total of 770 patients and 1363 lesions, with 389 patients treated endovascularly and 420 treated by surgical revascularization. Restenosis was more common with endovascular than open surgical intervention (odds ratio [OR] 5.18, 95% confidence interval [CI] 2.78-9.62; p < .001). In subgroup analysis according to the involved lesions, endovascular intervention patients showed more restenosis than open surgical intervention patients in the coronary artery, supra-aortic branches, and renal artery. In both the active and inactive stages, restenosis was more common in those treated endovascularly than in those treated by open surgery. However, stroke occurred less often with endovascular intervention than with open surgical intervention (OR 0.33, 95% CI 0.12-0.90; p = .003). Mortality and complications other than stroke and mortality did not differ between endovascular and open surgical intervention. CONCLUSION: This meta-analysis has shown a lower risk of restenosis with open surgical intervention than with endovascular intervention. Stroke was generally more common with open surgical intervention than with endovascular intervention. However, there were differences according to the location of the lesion, and the risk of stroke in open surgery is higher when the supra-aortic branches are involved rather than the renal arteries.

Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis: A Multicenter Study of 318 Patients.

BACKGROUND: Because of the wide variation in the course of Takayasu arteritis (TA), predicting outcome is challenging. We assess long-term outcome and prognosis factors for vascular complications in patients with TA. METHODS: A retrospective multicenter study of characteristics and outcomes of 318 patients with TA fulfilling American College of Rheumatology and Ishikawa criteria was analyzed. Factors associated with event-free survival, relapse-free survival, and incidences of vascular complications were assessed. Risk factors for vascular complications were identified in a multivariable model. RESULTS: The median age at TA diagnosis was 36 [25-47] years, and 276 patients (86.8%) were women. After a median follow-up of 6.1 years, relapses were observed in 43%, vascular complications in 38%, and death in 5%. Progressive clinical course was observed in 45%, carotidodynia in 10%, and retinopathy in 4%. The 5- and 10-year event-free survival, relapse-free survival, and complication-free survival were 48.2% (42.2; 54.9) and 36.4% (30.3; 43.9), 58.6% (52.7; 65.1) and 47.7% (41.2; 55.1), and 69.9% (64.3; 76.0) and 53.7% (46.8; 61.7), respectively. Progressive disease course (P=0.018) and carotidynia (P=0.036) were independently associated with event-free survival. Male sex (P=0.048), elevated C-reactive protein (P=0.013), and carotidynia (P=0.003) were associated with relapse-free survival. Progressive disease course (P=0.017), thoracic aorta involvement (P=0.009), and retinopathy (P=0.002) were associated with complication-free survival. CONCLUSIONS: This nationwide study shows that 50% of patients with TA will relapse and experience a vascular complication ≤10 years from diagnosis. We identified specific characteristics that identified those at highest risk for subsequent vascular complications.

Incidence, prevalence, mortality and causes of death in Takayasu Arteritis in Korea - A nationwide, population-based study.

BACKGROUND: Studies investigating the epidemiology and mortality of Takayasu Arteritis (TA) are scarce. The objective of this study was to provide information regarding the epidemiology of TA, such as the incidence, prevalence, survival and cause of death in Korea. METHODS: We used a national, population-based database maintained by the Rare Intractable Disease registration program and the Health Insurance Review and Assessment Service, which includes information on all TA patients, diagnosed based on uniform criteria. We also used data from Statistics Korea linked with RID database to confirm survival and cause of death. We analyzed data for the period 2008-2012. RESULTS: During study period, there were 612 new patients with TA, with a female-to-male ratio of 4.3:1. The annual mean incidence was 0.24 per 105 people, and the prevalence was 2.82 per 105 people in 2012. The average annual mortality rate was 0.02 per 105 people, and the standardized mortality ratio was 3.1. The overall survival of patients with TA was 96.6%, which was significantly lower than that in the general population. Cardiovascular disease was the most common cause of death, accounting for 29 of the 64 deaths (45.3%), followed by neoplasms in 9 patients (14.1%). CONCLUSIONS: The incidence and prevalence presented in this study are the highest ever reported. The survival of patients with TA was significantly lower than that of the general population, and the main cause of death was cardiovascular disease. This study reflects the need for greater awareness of TA, especially in Asian countries.

Vascular surgery: the main risk factor for mortality in 146 Takayasu arteritis patients.

Takayasu arteritis (TA) is an idiopathic chronic inflammatory disease that affects the aorta and its main branches. According to disease involvement, patients may require surgical treatment mainly due ischemic lesions in association with medical therapy. We evaluated the impact of vascular interventions in a cohort of TA patients. Medical records from 146 TA patients were reviewed. Clinical features, medical, and surgical treatment were revised and disease activity was determined by clinical, laboratorial, and imaging parameters. Clinical parameters associated with mortality alongside vascular procedures were evaluated and their impact on mortality in our cohort was estimated. Ninety-four vascular interventions were performed in 61 patients (41.8%). A third of them were of endovascular procedures. The overall mortality was 4.1%, all due to early postoperative complications, which resulted in a rate of surgery-related mortality of 9.8%. All deaths occurred in patients with active disease. Clinical parameters known to be associated with mortality (aneurysm, secondary hypertension, aortic insufficiency, and cerebrovascular accident) were not found related with death. Patients whose disease began before age 20 years had an OR 3.54 of undergoing a vascular surgical intervention. The observed impact of vascular procedures on mortality in patients with Takayasu arteritis, especially during disease activity, supports the notion that such interventions should be performed with caution and preferably during periods of remission.

Cause of death in Chinese Takayasu arteritis patients.

To analyze the causes of death and the related risk factors for in-patients with Takayasu arteritis (TAK) admitted to a referral center of China during 1983 to 2014.The medical charts of 12 deceased TAK patients (10 women, 2 men) were reviewed by two senior rheumatologists. The demographic data, clinical manifestations, angiographic presentations, and the direct causes of death were analyzed retrospectively. Medical records of 40 TAK patients (32 women, 8 men) were selected as controls by age and sex matching method from 81 patients who were sampled isometrically from 810 successively admitted TAK in-patients of the same center during the same period. In addition to the comparison of clinical manifestations between the two groups, binary logistic regression was conducted to explore the related risk factors of mortality of TAK.Twelve patients died at the median age of 33.5 (ranging from 13 to 68 years old). The median survival time was 102.5(ranging from 6 to 567) months. The direct causes of death were heart failure in 5 (5/12, 41.7%), hemorrhage in 2 (2/12, 16.7%), pulmonary infection in 2 (2/12, 16.7%), sudden death in 1 (1/12, 8.3%), postoperative complication in 1 (1/12, 8.3%), and end-stage malignancy in 1 (1/12, 8.3%). Ischemia (4/12, 33.3%) and hemorrhage (4/12, 33.3%) were the two most common presentations in deceased patients. Eight patients had received surgical procedures related to TAK changes. Among them, 2 patients died after surgical procedure, the other 6 patients died later of non-operation-related causes. Compared with the control group (n = 40), patients in the deceased group had longer disease duration (P = 0.017), higher proportion of active disease (P = 0.020), secondary hypertension (P = 0.004), and congestive heart failure (P = 0.017). A model of binary logistic regression had revealed that secondary hypertension (odds ratio [OR] = 9.333, 95% confidence interval [CI]: 1.721 - 50.614, P = 0.010), congestive heart failure (OR = 5.667, 95% CI: 1.248 - 25.734, P = 0.025), and longer disease duration (OR = 1.007, 95% CI: 1.001 - 0.735, P = 0.027) were risk factors for TAK mortality. Active disease (OR = 0.167, 95% CI: 0.038 - 50.614, P = 0.018) was negatively associated with death of TAK.Heart failure is the leading cause of death in TAK patients, followed by ischemia and pulmonary infection. Early deaths occur postoperatively but become rare later after the procedure. Well-control of hypertension, and prevention of congestive heart failure may improve the long-term prognosis.

Retrospective analysis of 95 patients with large vessel vasculitis: a single center experience.

AIM: Although Takayasu arteritis (TAK) and giant cell arteritis (GCA) have been considered as distinct disease entities, similarities of these diseases have been recently reported. However, little data is available regarding this issue in Japanese patients with TAK and GCA. In addition, the classification criteria for TAK established in 1990 by the American College of Rheumatology (ACR) have been criticized due to the age restriction for disease onset (≤ 40 years). Thus, we aimed to compare the clinical characteristics of Japanese patients with TAK and those with GCA and to clarify whether clinical differences existed between patients with early-onset (≤ 40 years) and late-onset (> 40 years) TAK. METHODS: We enrolled 86 patients with TAK and nine with GCA who visited our department from 1990 to 2014. The diagnoses of TAK and GCA were based on the criteria of the Japanese Circulation Society and the ACR, respectively. RESULTS: Mean ages at onset for TAK and GCA were 36.4 and 71.0 years, respectively. Patients with TAK had significantly higher incidences of aortic regurgitation and carotid and subclavian arterial involvement, lower frequencies of polymyalgia rheumatica, and better prognoses than those with GCA. In contrast, the clinical characteristics, distribution of arterial lesions, treatments administered, and prognoses of patients with early- and late-onset TAK were comparable. CONCLUSIONS: These results suggested that TAK and GCA differed substantially, and that the age restriction (≤ 40 years) may not be necessary for the diagnosis of TAK.

Clinical Features and Outcomes of Takayasu Arteritis with Neurological Symptoms in China: A Retrospective Study.

OBJECTIVE: To describe the clinical features and longterm outcomes of patients with Takayasu arteritis (TA) in China who experienced neurological symptoms. METHODS: A retrospective study was undertaken of patients with TA who attended a single study center from 2002 to 2013, who also exhibited neurological symptoms (n = 274). Clinical and imaging features were analyzed, as well as longterm outcomes. RESULTS: The mean age at disease onset was 28.2 ± 11.2 years, with a female-to-male ratio of 4.3:1. The most common neurological manifestation was dizziness (214, 78.1%), the most frequent type of TA was type III (112, 40.9%), and the most common affected artery was the left subclavian (147, 53.6%). Involvement of 3 or 4 branches of the aortic arch was observed in 28% of patients. Among 30 patients experiencing a stroke (10.9%), steno-occlusive lesions of the subclavian artery and common carotid artery were frequently observed in patients with ischemic stroke, while steno-occlusive lesions of the descending aorta, abdominal aorta, and/or renal arteries were more frequently observed with hemorrhagic stroke. Heart failure was the most common cardiovascular event in those who died (n = 6) and in surviving cohorts. CONCLUSION: Neurological features in patients with TA were variable, and correlated with the number of arteries and the site of artery involvement. Resistant hypertension was one of the most important risk factors for hemorrhagic stroke in patients with TA.

Surgery for aortic regurgitation and aortic root dilatation in Takayasu arteritis.

BACKGROUND: Special consideration may be required for surgical treatment in Takayasu arteritis because inflammation may cause serious complications such as valve detachment or dilatation of the residual aorta. We evaluated our surgical outcome of treatment for aortic regurgitation and aortic root dilatation in Takayasu arteritis. METHODS: Between December 1983 and January 2013, 22 cardiac operations were performed in 20 patients with aortic regurgitation due to Takayasu arteritis. Aortic valve replacement was carried out in 6 patients, and aortic root replacement in 16. Of these 16 patients, composite graft replacement was undertaken in 6, composite graft replacement plus coronary artery bypass grafting in 2, composite graft replacement plus total or partial arch replacement in 7, and valve-sparing aortic root replacement with hemiarch replacement in one. RESULTS: The operative mortality (within 30 days) was 4.5% and the 5-year survival rate was 90.9%. Early surgical reintervention was not required in any patient. Neither valve detachment nor composite graft detachment was noted. Two patients required redo aortic root replacement due to pseudoaneurysm formation and severe aortic regurgitation during follow-up. Late dilatation of the residual thoracoabdominal or abdominal aorta was observed in 2 patients, and both were treated surgically. CONCLUSIONS: The early surgical outcome was acceptable but surgical reintervention was required because of late dilatation of the residual aorta or recurrent aortic regurgitation due to annular dilatation, and longstanding careful follow-up will be needed.

Inflammatory disease of the aorta: patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis.

OBJECTIVE: Inflammatory diseases of the aorta, other than those of known infective etiology, are poorly understood. We analyzed a large series of affected patients who had histologic diagnoses with a view to improving the classification of the extent of aortitis to enable a more targeted approach of treatment. METHODS: Between 1996 and 2012, we operated on 7551 patients with ascending or aortic arch disease, of whom 279 had clinically diagnosed inflammatory disease. Of these, 156 (2%) were found to have aortitis on histologic examination. RESULTS: Patients were divided into 4 histologically based groups: giant cell aortitis, 31% (49/156); Takayasu arteritis, 5.1% (8/156); isolated aortitis, 59% (92/156); and other, 4.5% (7/156). Patterns of anatomic extent were also analyzed, and in particular it was noted that giant cell aortitis and isolated aortitis had more extensive disease. In addition, specimen analysis suggested early indications of unrecognized preexistent infections. Death after surgery occurred in 3.2% (5/156), and stroke in 1.9% (3/156). Kaplan-Meier survival at 8 years was 55%. We present a classification for disease extent and management. CONCLUSIONS: Aortitis continues to be a conundrum; however, good results are achievable with surgery. Intervention should be based on a clearer understanding of the histologic pattern and extent of disease, which helps in subsequent targeted disease management.

Clinical manifestations and longterm outcome for patients with Takayasu arteritis in China.

OBJECTIVE: To describe a large cohort of patients with Takayasu arteritis in China. METHODS: We retrospectively analyzed 566 patients hospitalized in Fuwai Hospital between 2002 and 2013. Data collected were clinical characteristics, laboratory findings, angiographic features, treatment, and longterm outcome. RESULTS: The female to male ratio was 3.8 to 1, and the mean age of onset was 28.9 ± 12.0 years. The most common inflammatory symptom, initial symptom, and coexisting disease were fever (52, 9.2%), dizziness (214, 37.8%), and hypertension (HTN; 392, 69.3%), respectively. Pulmonary artery, coronary artery involvement, and aortic regurgitation were found in 83 (14.7%), 66 (11.7%), and 181 (36.7%) patients, respectively. Elevation of the erythrocyte sedimentation rate was observed in 131 patients (23.1%). Treatment included drugs, interventional therapy, autologous blood vessel transplant, artificial blood vessel transplant, and aortic valve replacement. During a mean followup of 5.0 ± 0.2 years, 32 patients died, including 1 patient who died suddenly during coronary angiography. HTN, major complications, and a progressive disease course were significant prognostic markers. CONCLUSION: HTN, rather than fever, is the leading reason for patients with Takayasu arteritis to see a doctor in China. HTN, major complications, and a progressive disease course are statistically significant predictors of survival. Because of cardiovascular events associated with the disease, early diagnosis and treatment are urgent to improve prognosis.