Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiag...Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiagnosed or missed.Approximately 50%of the patients having TA with pulmonary artery involvement develop pulmonary hypertension(PH).The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone.Early balloon pulmonary angioplasty(BPA)can improve the clinical symptoms and survival of patients with stable TA.To the best of our knowledge,this is the first case reported in the English literature in which a“Guidezilla”catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis(TA).展开更多
Dear Editor,Takayasu arteritis(TA)is a rare,idiopathic,granulomatous,large-vessel vasculitis,with preferential involvement of the aorta,its main branches,and pulmonary arteries[1].It has been reported that the ocular ...Dear Editor,Takayasu arteritis(TA)is a rare,idiopathic,granulomatous,large-vessel vasculitis,with preferential involvement of the aorta,its main branches,and pulmonary arteries[1].It has been reported that the ocular involvements were found in 8.1%–68%[2],non-specific arthralgia or myalgia in 13%–41%,aortic regurgitation in 5%–55%.展开更多
Takayasu arteritis (TA) is a chronic, nonspecific inflammatory disease of large and medium-sized vessels that primarily involves the aorta and its branches. TA involving the pulmonary arteries has a prevalence ranging...Takayasu arteritis (TA) is a chronic, nonspecific inflammatory disease of large and medium-sized vessels that primarily involves the aorta and its branches. TA involving the pulmonary arteries has a prevalence ranging from 14% to 86%, which can lead to pulmonary hypertension, a progressive increase in pulmonary artery pressure, and eventually death from right heart failure. The presentation of pulmonary arteritis (PA) is very nonspecific, with a reported misdiagnosis rate of up to 60% and a diagnosis time ranging from 1 month to more than 10 years. The clinical manifestation of pleural effusion is very rare in both TA and PA cases. Based on our literature review, this is the 6th reported case of TA with pleural effusion, and the specific mechanism of TA with pleural effusion is still unclear. The characteristics of this case and the previously reported cases are summarized in this article to improve the understanding of TA and PA and reduce the misdiagnosis rate.展开更多
Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large po...Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large population of TA patients. Methods We included a total of 1105 patients with TA, who were hospitalized between January 1992 and December 2017. Comparisons of clinical features were made according to the presence of TB. Results Among the 1105 patients, 109 (9.9%) had TB, including 53 patients (48.6%) diagnosed with TB before the onset of TA, 23 (21.1%) with a concurrent diagnosis of TB and TA, and 24 patients (22.0%) who developed TB after TA. Pulmonary TB was the most frequently identified (97 patients, 89.0%). Patients with TB had more frequent involvement of the pulmonary artery and experienced more chest discomfort and constitutional symptoms but had less interventional treatment. Demographic characteristics, comorbid diseases, and use of steroids were similar between patients with and without TB. Conclusions The proportion of Chinese TA patients with TB was not low, and about half of the patients had TB before TA. Pulmonary TB was the most common. Pulmonary artery involvement and pulmonary hypertension was more frequent in TA patients with TB.展开更多
BACKGROUND Takayasu arteritis is a rare but intractable chronic disease in young female patients. Percutaneous transluminal angioplasty of the involved renal arteries has been reported;however, few studies have report...BACKGROUND Takayasu arteritis is a rare but intractable chronic disease in young female patients. Percutaneous transluminal angioplasty of the involved renal arteries has been reported;however, few studies have reported the use of drug coated balloon angioplasty in the treatment of Takayasu arteritis. We aimed to demonstrate five young female patients who presented with a history of hypertension due to Takayasu arteritis. CASE SUMMARY From April 2017 to October 2018, five female patients were diagnosed with hypertension due to Takayasu arteritis by computed tomography angiography (CTA) and laboratory tests. Four patients had a complaint of headache with or without dizziness, and one patient showed no symptom. There was no significant family or past history of hypertension or kidney disease, and the physical examinations were almost normal on admission. We performed a treatment by drug coated balloon angioplasty. Blood pressure decreased dramatically in all patients after balloon angioplasty, and the patency of treated renal artery was demonstrated with CTA over 5 months after the angioplasty procedure. CONCLUSION Drug coated balloon angioplasty is safe and effective for renal artery stenosis due to Takayasu arteritis. A prospective study with a larger sample size is necessary to further demonstrate the effectiveness of the treatment.展开更多
Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospe...Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospective study included 533 consecutive patients hospitalized for TA from January 2009 to April 2018. Anemia was diagnosed on the basis of hemoglobin level, according to World Health Organization criteria. Results A total of 194 patients(36.4%) were diagnosed with anemia. Most had mild anemia(177, 91.2%). Female patients were predominant(92.8% of anemic patients). Normocytic anemia(62.9%) was the most common pattern. Anemic patients were more likely than non-anemic patients to have dizziness(29.4% vs. 21.2%), low body mass index(22.0 ± 3.6 vs. 22.9 ± 3.4 kg/m2), and active disease stage(64.9% vs. 50.1%);pulmonary involvement(12.4% vs. 26.8%), pulmonary hypertension(12.9% vs. 20.1%) and pulmonary hypertensive-target drugs(2.8% vs. 11.6%) were less common among anemic than non-anemic patients(all P < 0.05). Larger left ventricular end-diastolic diameter and lower left ventricular ejection fraction were observed in anemic patients. Over a median follow-up of four months, the increase of hemoglobin in anemic patients was associated with the use of iron supplementation. Conclusions Anemia is a very common concurrent condition in TA, especially in young, female patients. Patients with anemia are more likely to be in the active disease stage. Iron supplementation helps increase hemoglobin.展开更多
Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Metho...Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively.None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy,accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period,were analyzed.All of them were females with a mean age of 35.5 ± 10.0 years.Four cases presented with lower extremity edema.Laboratory tests showed that one was nephrotic syndrome,three were nephrotic range proteinuria,and two of them had mild renal dysfunction.The other two patients were asymptomatic microscopic hematuria and proteinuria.Renal pathology revealed mild immunoglobulin A nephropathy in two cases,mild mesangial proliferative glomerulonephritis(GN),membranoproliferative GN,minimal change disease,and fibrillary GN in one case respectively.Five cases received glucocorticoids and cyclophosphamide therapy.Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases.The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment.Conclusions TA may induce glomerular disease as a part of its histological spectrum.Apart from ischemic glomerular disease,glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria,that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.展开更多
Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital ...Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the American College of Rheumatology. Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging. Results Dizziness and visual disturbance were the most common symptoms, which occurred in 20 (74.1%) and 16 (59.3%) patients respectively. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had suffered from ischemic stroke; 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations. Conclusions Neurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some unusual ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA.展开更多
This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vasculitis and demonstrating several cases that we have encountered. Particular emphasis will be put on T...This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vasculitis and demonstrating several cases that we have encountered. Particular emphasis will be put on Takayasu arteritis (also known as aortitis syndrome) most commonly observed in Japan and other oriental countries. CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography. Importance of plain chest radiograph should also be kept in mind; it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.展开更多
Background Left ventricular dilatation and a decreased ejection fraction is one of the most serious complications in patients with Takayasu arteritis(TA).We aim to describe the prevalence,clinical features,treatment,a...Background Left ventricular dilatation and a decreased ejection fraction is one of the most serious complications in patients with Takayasu arteritis(TA).We aim to describe the prevalence,clinical features,treatment,and long-term prognosis of TA patients with this complication.展开更多
Objective Anemia as a concomitant disease of patients with Takayasu arteritis (TA) has been reported in several case reports, but epidemiological studies of large populations are lacking. This study evaluated the prev...Objective Anemia as a concomitant disease of patients with Takayasu arteritis (TA) has been reported in several case reports, but epidemiological studies of large populations are lacking. This study evaluated the prevalence, clinical characteristics, and medical treatment in Chinese TA population with anemia.展开更多
Objective Takayasu arteritis(TA)is a kind of large vessel vasculitis of unknown reason.Autoimmune disorder was perhaps one of the risk factors.Cancer was related to autoimmune disease.This study aimed to describe the ...Objective Takayasu arteritis(TA)is a kind of large vessel vasculitis of unknown reason.Autoimmune disorder was perhaps one of the risk factors.Cancer was related to autoimmune disease.This study aimed to describe the type and incidence of malignancy in 1,105 TA patients.展开更多
In part 1 of this article, clinical features and imaging findings of Takayasu arteritis (also known as aortitis syndrome) were described in detail. In part 2, treatment of Takayasu arteritis will be first described an...In part 1 of this article, clinical features and imaging findings of Takayasu arteritis (also known as aortitis syndrome) were described in detail. In part 2, treatment of Takayasu arteritis will be first described and discussed. This will be followed by description of clinical features and imaging findings of other systemic vasculitis. Comments on interventional radiology for systemic vasculitis will also be made.展开更多
Takayasu vasculitis is a rare type of large vessel vasculitis that primarily affects the aorta and its main branches;signs and symptoms are usually due to systemic inflammation or ischemia of an organ or a limb, depen...Takayasu vasculitis is a rare type of large vessel vasculitis that primarily affects the aorta and its main branches;signs and symptoms are usually due to systemic inflammation or ischemia of an organ or a limb, depending on the group of blood vessels involved. In addition, Takayasu arteritis is associated with increased platelet and coagulation activity, leading to a hypercoagulable state and thrombus formation. We report a case of a 47-year-old male who presented with a history of complete anuria for 3 days and was found to have progressively worsening kidney function. Renal Doppler ultrasound confirmed the presence of bilateral renal artery thrombosis, while contrast-enhanced computed tomography (CECT) of the abdomen and pelvis showed extensive abdominal aortic thrombosis with radiological findings consistent with large vessel vacuities. After catheter-directed thrombolytic therapy of the renal arteries, the patient started producing urine and his kidney function significantly improved. Later, positron emission tomography scan (PET) confirmed large vessel Takayasu arteritis. Echocardiography showed no intracardiac thrombus, along with an extensive work-up for thrombophilia, as autoimmune and vasculitis serology came back negative. This is an extremely rare presentation of Takayasu arteritis, with an unusual recovery of acute renal failure after prolonged anuria due to bilateral renal artery thrombosis.展开更多
Takayasu arteritis is a large vessel vasculitis of the young women with giant cells and granuloma formation. The diagnosis and management of the disease are really not so easy because of the insidious onset and the di...Takayasu arteritis is a large vessel vasculitis of the young women with giant cells and granuloma formation. The diagnosis and management of the disease are really not so easy because of the insidious onset and the difficulties in assessment of disease activity. Nearly 60% of the patients are corticosteroid resistant or dependent and relapses are very frequent during taper of the dose [1]. The novel biologic agents as Anti-TNF, Rutiximab and Tocilizumab provide acceptable response rates with low toxicity. Herein, we reviewed the efficacy of Tocilizumab in Takayasu arteritis.展开更多
Background:Takayasu arteritis-induced renal arteritis (TARA), commonly seen in Takayasu arteritis (TA), has become one of the main causes of poor prognosis and early mortality in patients with TA. TARA progressing int...Background:Takayasu arteritis-induced renal arteritis (TARA), commonly seen in Takayasu arteritis (TA), has become one of the main causes of poor prognosis and early mortality in patients with TA. TARA progressing into Takayasu arteritis-induced renal artery stenosis (TARAS), could lead to severe complications including malignant hypertension, cardiac-cerebral vascular disease, and ischemic nephropathy. Since there existed no guidelines on treatments, this study aimed to review the comprehensive treatments for TARA.Methods:We searched systematically in databases including PubMed, Ovid-Medline, EMBASE, Web of Science, China National Knowledge Infrastructure, Wanfang, and SinoMed, from inception to May 2018. Literature selection, data extraction, and statistical analysis were performed.Results:Eighty-two literatures were recruited focusing on medical treatments ( n = 34) and surgical treatments ( n = 48). We found that combined medical treatments of glucocorticoids and conventional synthetic disease-modifying anti-rheumatic drugs could reach high rates of remission in patients with TARA, and biological disease-modifying anti-rheumatic drugs were preferred for refractory patients. After remission induction, surgical treatment could help reconstruct renal artery and recover renal function partly. Percutaneous transluminal angioplasty was the first choice for patients with TARAS, while open surgery showed a good longterm survival. Conclusions:Patients with TARA should benefit both from medical treatments and from surgical treatments comprehensively and sequentially. Multidisciplinary team coordination is recommended especially in patients with severe complications.展开更多
Background:Interleukin-18(IL18)gene polymorphisms are related to many inflammatory and autoimmune diseases.However,a correlation analysis between IL18-607C/A and-137G/C gene polymorphisms and Takayasu arteritis(TA)is ...Background:Interleukin-18(IL18)gene polymorphisms are related to many inflammatory and autoimmune diseases.However,a correlation analysis between IL18-607C/A and-137G/C gene polymorphisms and Takayasu arteritis(TA)is lacking.Methods:This study enrolled 200 patients with TA as the case group and 334 region-,age-,and sex-matched healthy subjects as the control group.We genotyped alleles and genotypes at positions-607 and-137 of the IL18 gene and analyzed the distribution frequencies.Mann-Whitney U test,t test,Chi-squared test and Hardy-Weinberg equilibrium were performed.Results:After adjusting for risk factors,the adjusted odds ratios and 95%confidence intervals at position-607C/A were 0.533,0.391 to 0.880(P=0.010);0.266,0.586 to 1.002(P=0.051);and 0.122,0.552 to 1.420(P=0.613)under the dominant,additive,and recessive models,respectively.For the-137G/C polymorphism,the adjusted odds ratios and 95%confidence intervals were 1.571,1.068 to 2.311(P=0.022);1.467,1.086 to 1.980(P=0.012);and 1.815,0.901 to 3.656(P=0.095)under the dominant,additive,and recessive models,respectively.Moreover,regardless of the model used,we found no statistical difference in distribution frequency between the active and quiescent states of TA for the-607C/A(P=0.355,0.631,and 0.705,respectively)and-137G/C polymorphisms(P=0.205,0.385,and 0.208,respectively).Conclusions:The IL18-607C/A gene polymorphism may decrease the risk of TA,and thus is a protective factor,whereas-137G/C may increase the risk of TA,and thus is a risk factor.However,neither polymorphism was related to activity(active vs.quiescent)of TA.展开更多
Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both d...Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.展开更多
Takayasu arteritis (TA) is a chronic progressive nonspecific vasculitis that involves the aorta and its main branches,resulting in luminal stenosis in the large vessels.Steroid therapy is the foundation for TA treatme...Takayasu arteritis (TA) is a chronic progressive nonspecific vasculitis that involves the aorta and its main branches,resulting in luminal stenosis in the large vessels.Steroid therapy is the foundation for TA treatment and the guarantee of surgery.When a patient presents with uncontrolled symptoms,surgical treatment is the only measure that may be adopted to prevent greater risk to life.However,surgical repair for extensive arteriopathy requires an ingenious design to avoid morbidity in response to the complexity of the pathophysiology of TA.A 41-year-old woman presented with pulselessness,recurrent dizziness,and visual disturbance for the past 23 years.The patient had been diagnosed with TA 13 years ago and had received steroid therapy.However,the symptoms of dizziness and visual disturbance had increased in intensity and duration over the last 2 years.展开更多
基金supported by fund from Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(2021-I2M-1–049)National High Level Hospital Clinical Research Funding(2022-NHLHCRF-LX-01-0203)
文摘Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiagnosed or missed.Approximately 50%of the patients having TA with pulmonary artery involvement develop pulmonary hypertension(PH).The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone.Early balloon pulmonary angioplasty(BPA)can improve the clinical symptoms and survival of patients with stable TA.To the best of our knowledge,this is the first case reported in the English literature in which a“Guidezilla”catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis(TA).
文摘Dear Editor,Takayasu arteritis(TA)is a rare,idiopathic,granulomatous,large-vessel vasculitis,with preferential involvement of the aorta,its main branches,and pulmonary arteries[1].It has been reported that the ocular involvements were found in 8.1%–68%[2],non-specific arthralgia or myalgia in 13%–41%,aortic regurgitation in 5%–55%.
文摘Takayasu arteritis (TA) is a chronic, nonspecific inflammatory disease of large and medium-sized vessels that primarily involves the aorta and its branches. TA involving the pulmonary arteries has a prevalence ranging from 14% to 86%, which can lead to pulmonary hypertension, a progressive increase in pulmonary artery pressure, and eventually death from right heart failure. The presentation of pulmonary arteritis (PA) is very nonspecific, with a reported misdiagnosis rate of up to 60% and a diagnosis time ranging from 1 month to more than 10 years. The clinical manifestation of pleural effusion is very rare in both TA and PA cases. Based on our literature review, this is the 6th reported case of TA with pleural effusion, and the specific mechanism of TA with pleural effusion is still unclear. The characteristics of this case and the previously reported cases are summarized in this article to improve the understanding of TA and PA and reduce the misdiagnosis rate.
基金supported by the National Key Research and Development Program of China (2016YFC1300100)CAMS Innovation Fund for Medical Sciences (2016I2M-1-002)
文摘Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large population of TA patients. Methods We included a total of 1105 patients with TA, who were hospitalized between January 1992 and December 2017. Comparisons of clinical features were made according to the presence of TB. Results Among the 1105 patients, 109 (9.9%) had TB, including 53 patients (48.6%) diagnosed with TB before the onset of TA, 23 (21.1%) with a concurrent diagnosis of TB and TA, and 24 patients (22.0%) who developed TB after TA. Pulmonary TB was the most frequently identified (97 patients, 89.0%). Patients with TB had more frequent involvement of the pulmonary artery and experienced more chest discomfort and constitutional symptoms but had less interventional treatment. Demographic characteristics, comorbid diseases, and use of steroids were similar between patients with and without TB. Conclusions The proportion of Chinese TA patients with TB was not low, and about half of the patients had TB before TA. Pulmonary TB was the most common. Pulmonary artery involvement and pulmonary hypertension was more frequent in TA patients with TB.
基金Supported by the National Natural Science Foundation of China,No.81501569
文摘BACKGROUND Takayasu arteritis is a rare but intractable chronic disease in young female patients. Percutaneous transluminal angioplasty of the involved renal arteries has been reported;however, few studies have reported the use of drug coated balloon angioplasty in the treatment of Takayasu arteritis. We aimed to demonstrate five young female patients who presented with a history of hypertension due to Takayasu arteritis. CASE SUMMARY From April 2017 to October 2018, five female patients were diagnosed with hypertension due to Takayasu arteritis by computed tomography angiography (CTA) and laboratory tests. Four patients had a complaint of headache with or without dizziness, and one patient showed no symptom. There was no significant family or past history of hypertension or kidney disease, and the physical examinations were almost normal on admission. We performed a treatment by drug coated balloon angioplasty. Blood pressure decreased dramatically in all patients after balloon angioplasty, and the patency of treated renal artery was demonstrated with CTA over 5 months after the angioplasty procedure. CONCLUSION Drug coated balloon angioplasty is safe and effective for renal artery stenosis due to Takayasu arteritis. A prospective study with a larger sample size is necessary to further demonstrate the effectiveness of the treatment.
基金funded by the National Key Research and Development Program of China (2016YFC1300100)CAMS Innovation Fund for Medical Sciences (2016-I2M1-002)
文摘Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospective study included 533 consecutive patients hospitalized for TA from January 2009 to April 2018. Anemia was diagnosed on the basis of hemoglobin level, according to World Health Organization criteria. Results A total of 194 patients(36.4%) were diagnosed with anemia. Most had mild anemia(177, 91.2%). Female patients were predominant(92.8% of anemic patients). Normocytic anemia(62.9%) was the most common pattern. Anemic patients were more likely than non-anemic patients to have dizziness(29.4% vs. 21.2%), low body mass index(22.0 ± 3.6 vs. 22.9 ± 3.4 kg/m2), and active disease stage(64.9% vs. 50.1%);pulmonary involvement(12.4% vs. 26.8%), pulmonary hypertension(12.9% vs. 20.1%) and pulmonary hypertensive-target drugs(2.8% vs. 11.6%) were less common among anemic than non-anemic patients(all P < 0.05). Larger left ventricular end-diastolic diameter and lower left ventricular ejection fraction were observed in anemic patients. Over a median follow-up of four months, the increase of hemoglobin in anemic patients was associated with the use of iron supplementation. Conclusions Anemia is a very common concurrent condition in TA, especially in young, female patients. Patients with anemia are more likely to be in the active disease stage. Iron supplementation helps increase hemoglobin.
文摘Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively.None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy,accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period,were analyzed.All of them were females with a mean age of 35.5 ± 10.0 years.Four cases presented with lower extremity edema.Laboratory tests showed that one was nephrotic syndrome,three were nephrotic range proteinuria,and two of them had mild renal dysfunction.The other two patients were asymptomatic microscopic hematuria and proteinuria.Renal pathology revealed mild immunoglobulin A nephropathy in two cases,mild mesangial proliferative glomerulonephritis(GN),membranoproliferative GN,minimal change disease,and fibrillary GN in one case respectively.Five cases received glucocorticoids and cyclophosphamide therapy.Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases.The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment.Conclusions TA may induce glomerular disease as a part of its histological spectrum.Apart from ischemic glomerular disease,glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria,that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.
文摘Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the American College of Rheumatology. Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging. Results Dizziness and visual disturbance were the most common symptoms, which occurred in 20 (74.1%) and 16 (59.3%) patients respectively. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had suffered from ischemic stroke; 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations. Conclusions Neurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some unusual ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA.
文摘This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vasculitis and demonstrating several cases that we have encountered. Particular emphasis will be put on Takayasu arteritis (also known as aortitis syndrome) most commonly observed in Japan and other oriental countries. CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography. Importance of plain chest radiograph should also be kept in mind; it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.
文摘Background Left ventricular dilatation and a decreased ejection fraction is one of the most serious complications in patients with Takayasu arteritis(TA).We aim to describe the prevalence,clinical features,treatment,and long-term prognosis of TA patients with this complication.
文摘Objective Anemia as a concomitant disease of patients with Takayasu arteritis (TA) has been reported in several case reports, but epidemiological studies of large populations are lacking. This study evaluated the prevalence, clinical characteristics, and medical treatment in Chinese TA population with anemia.
文摘Objective Takayasu arteritis(TA)is a kind of large vessel vasculitis of unknown reason.Autoimmune disorder was perhaps one of the risk factors.Cancer was related to autoimmune disease.This study aimed to describe the type and incidence of malignancy in 1,105 TA patients.
文摘In part 1 of this article, clinical features and imaging findings of Takayasu arteritis (also known as aortitis syndrome) were described in detail. In part 2, treatment of Takayasu arteritis will be first described and discussed. This will be followed by description of clinical features and imaging findings of other systemic vasculitis. Comments on interventional radiology for systemic vasculitis will also be made.
文摘Takayasu vasculitis is a rare type of large vessel vasculitis that primarily affects the aorta and its main branches;signs and symptoms are usually due to systemic inflammation or ischemia of an organ or a limb, depending on the group of blood vessels involved. In addition, Takayasu arteritis is associated with increased platelet and coagulation activity, leading to a hypercoagulable state and thrombus formation. We report a case of a 47-year-old male who presented with a history of complete anuria for 3 days and was found to have progressively worsening kidney function. Renal Doppler ultrasound confirmed the presence of bilateral renal artery thrombosis, while contrast-enhanced computed tomography (CECT) of the abdomen and pelvis showed extensive abdominal aortic thrombosis with radiological findings consistent with large vessel vacuities. After catheter-directed thrombolytic therapy of the renal arteries, the patient started producing urine and his kidney function significantly improved. Later, positron emission tomography scan (PET) confirmed large vessel Takayasu arteritis. Echocardiography showed no intracardiac thrombus, along with an extensive work-up for thrombophilia, as autoimmune and vasculitis serology came back negative. This is an extremely rare presentation of Takayasu arteritis, with an unusual recovery of acute renal failure after prolonged anuria due to bilateral renal artery thrombosis.
文摘Takayasu arteritis is a large vessel vasculitis of the young women with giant cells and granuloma formation. The diagnosis and management of the disease are really not so easy because of the insidious onset and the difficulties in assessment of disease activity. Nearly 60% of the patients are corticosteroid resistant or dependent and relapses are very frequent during taper of the dose [1]. The novel biologic agents as Anti-TNF, Rutiximab and Tocilizumab provide acceptable response rates with low toxicity. Herein, we reviewed the efficacy of Tocilizumab in Takayasu arteritis.
基金This work was supported by a grant from the National Natural Science Foundation of China(No.81771730)。
文摘Background:Takayasu arteritis-induced renal arteritis (TARA), commonly seen in Takayasu arteritis (TA), has become one of the main causes of poor prognosis and early mortality in patients with TA. TARA progressing into Takayasu arteritis-induced renal artery stenosis (TARAS), could lead to severe complications including malignant hypertension, cardiac-cerebral vascular disease, and ischemic nephropathy. Since there existed no guidelines on treatments, this study aimed to review the comprehensive treatments for TARA.Methods:We searched systematically in databases including PubMed, Ovid-Medline, EMBASE, Web of Science, China National Knowledge Infrastructure, Wanfang, and SinoMed, from inception to May 2018. Literature selection, data extraction, and statistical analysis were performed.Results:Eighty-two literatures were recruited focusing on medical treatments ( n = 34) and surgical treatments ( n = 48). We found that combined medical treatments of glucocorticoids and conventional synthetic disease-modifying anti-rheumatic drugs could reach high rates of remission in patients with TARA, and biological disease-modifying anti-rheumatic drugs were preferred for refractory patients. After remission induction, surgical treatment could help reconstruct renal artery and recover renal function partly. Percutaneous transluminal angioplasty was the first choice for patients with TARAS, while open surgery showed a good longterm survival. Conclusions:Patients with TARA should benefit both from medical treatments and from surgical treatments comprehensively and sequentially. Multidisciplinary team coordination is recommended especially in patients with severe complications.
基金grants from the National Key Research and Development Program of China(No.2016YFC1301002 and No.2020YFC2004803)National Natural Science Foundation of China Grant(No.81900449).
文摘Background:Interleukin-18(IL18)gene polymorphisms are related to many inflammatory and autoimmune diseases.However,a correlation analysis between IL18-607C/A and-137G/C gene polymorphisms and Takayasu arteritis(TA)is lacking.Methods:This study enrolled 200 patients with TA as the case group and 334 region-,age-,and sex-matched healthy subjects as the control group.We genotyped alleles and genotypes at positions-607 and-137 of the IL18 gene and analyzed the distribution frequencies.Mann-Whitney U test,t test,Chi-squared test and Hardy-Weinberg equilibrium were performed.Results:After adjusting for risk factors,the adjusted odds ratios and 95%confidence intervals at position-607C/A were 0.533,0.391 to 0.880(P=0.010);0.266,0.586 to 1.002(P=0.051);and 0.122,0.552 to 1.420(P=0.613)under the dominant,additive,and recessive models,respectively.For the-137G/C polymorphism,the adjusted odds ratios and 95%confidence intervals were 1.571,1.068 to 2.311(P=0.022);1.467,1.086 to 1.980(P=0.012);and 1.815,0.901 to 3.656(P=0.095)under the dominant,additive,and recessive models,respectively.Moreover,regardless of the model used,we found no statistical difference in distribution frequency between the active and quiescent states of TA for the-607C/A(P=0.355,0.631,and 0.705,respectively)and-137G/C polymorphisms(P=0.205,0.385,and 0.208,respectively).Conclusions:The IL18-607C/A gene polymorphism may decrease the risk of TA,and thus is a protective factor,whereas-137G/C may increase the risk of TA,and thus is a risk factor.However,neither polymorphism was related to activity(active vs.quiescent)of TA.
文摘Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.
文摘Takayasu arteritis (TA) is a chronic progressive nonspecific vasculitis that involves the aorta and its main branches,resulting in luminal stenosis in the large vessels.Steroid therapy is the foundation for TA treatment and the guarantee of surgery.When a patient presents with uncontrolled symptoms,surgical treatment is the only measure that may be adopted to prevent greater risk to life.However,surgical repair for extensive arteriopathy requires an ingenious design to avoid morbidity in response to the complexity of the pathophysiology of TA.A 41-year-old woman presented with pulselessness,recurrent dizziness,and visual disturbance for the past 23 years.The patient had been diagnosed with TA 13 years ago and had received steroid therapy.However,the symptoms of dizziness and visual disturbance had increased in intensity and duration over the last 2 years.