“Guidezilla”extension catheter combined with balloon technique for treating pulmonary artery stenosis caused by Takayasu arteritis

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).

Glomerular Disease Associated with Takayasu Arteritis:6 Cases Analysis and Review of the Literature

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.

Neurological Manifestations of Takayasu Arteritis

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.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 2

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.

Unilateral pleural effusion secondary to Takayasu arteritis: a case report and literature review

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.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis:Part 1

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.

Association of interleukin-18 gene polymorphisms with Takayasu arteritis in a Chinese Han population

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.

Surgical treatment of multivessel lesions in Takayasu arteritis

Takayasu arteritis （TA） is a chronic progressive nonspecific vasculitis that involves the aorta and itsmain 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. Upon admission, physical examination revealed that pulse in both arms were absent. Her blood pressure was 98/63 mmHg （1 mmHg=0.133 kPa） in the left arm,

Advancements in medical and surgical treatments of Takayasu arteritis-induced renal arteritis:a systematic review

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.

Single Nucleotide Polymorphism rs10919543 in FCGR2A/ FCGR3A Region Confers Susceptibility to Takayasu Arteritis in Chinese Population

Background： Takayasu arteritis （TA） is a rare inflammatory arteriopathy of unknown etiology. The aim of this study was to investigate the genetic susceptibility to TA in a Chinese population. Methods： Four single nucleotide polymorphisms （SNPs） those locate in the IL12B region （rs56167332）, the MLX region （rs665268）, the FCGR2A/FCGR3A locus （rsi0919543）, and the HLA-B/M1CA locus （rs12524487）, associated with TA in different population, were genotyped in 123 Chinese TA patients and 147 healthy controls from January 2013 to August 2014. A Chi-square test was used to test for genotype/allele frequencies variants. Results： Among the four SNPs, rs 10919543 was found to be significantly associated with TA in the studied population. The GG genotype of rs 10919543 at the FCGR2A/FCGR3A locus is a high risk factor （odds ratio [OR] = 6.532, 95% confidence interval [C1] = 2.402 - 17.763, P 〈 0.001 ） for TA. Among TA patients, the level of eosinophil granulocytes （Eos） in the peripheral blood was observed to be higher in the GG group of rs 10919543 （n = 23, Eos = 0. I 1 [0.08, 0.17] x 109/L） than the GA ＋ AA group （n = 100, Eos = 0.08 [0.05, 0.13] 10/L, P = 0.028）. No correlation between the genotypes of the other three SNPs and TA patients was observed. Conclusions： Our findings revealed unique genetic pattern in Chinese TA patients that may be partly responsible for the higher risk of TA in this population. FCGR2A/FCGR3A-related immune disorder might contribute to the etiology of TA.

Visceral and renal arteries stenosis associated with Takayasu arteritis

Takayasu arteritis （TA） is a nonspecific granulomatous inflammatory arteriopathy of unknown cause that results in occlusive obliteration or less commonly aneurysm degeneration of large and medium-sized elastic arteries. Most descriptions of this disease have emphasized the ＂pulseless＂ syndrome, however, less attention has been paid to involvement of other segments of the aorta, renal arteries, and in particular the visceral arteries. We reported a case of type Ⅱ TA, in which both the visceral and the bilateral renal arteries were involved.

Treatment of cerebral aneurysms associated with Takayasu arteritis

Takayasu arteritis （TA） is a chronic inflammatory disease of the large elastic arteries that primarily affects the aorta and its major branches, resulting in luminal stenosis and aneurysmal changes in the large vessels.1 Cerebral aneurysms and accompanying subarachnoid hemorrhage （SAH） are rare; no case from China has been reported. We herein report two cases of TA with cerebral aneurysms that were successfully treated with coil embolization.

Forty-two Patients with Takayasu Arteritis: Clinical,Laboratory and Angiographic Studies

This study was to analyze 42 cases of Takayasu arteritis, and to discuss its early diagnosis and treatment. The clinical differentiation, conventional laboratory study, immunology test and angiography of 42 patients dispersed in 9 clinic departments were analyzed. The first symptoms of these patients were pulseless (21.4%) and secondary hypertension (78.6%) were found. The murmur could be heard in the area corresponding to the narrowed artery in 70%. Conventional laboratory study and immunology tests were not valuable. The sensitivity for arterial stenosis by Doppler color Bultrasonography and radioisotopic nephrography was about 80%. Angiography could show the type of brachiocephalic trunk 8 cases, renal 29 cases and widespread 5 cases. Eighteen cases were treated with angioplasty, and 14 of them were successful. Nine cases in early stage were treated with medicine, and 7 cases of them got remission. Early diagnosis still remains a key problem for further study. Antiendothelial cell antibodies (AE CA) and thrombomodulin are suggested as a helpful basis for early diagnosis. Angiography could explicate the degree of the lesions and indicate angioplasty for them, and should be adopted earlier.

Clinical features of aortic dissection associated with Takayasu＇s arteritis

Takayasu＇s arteritis （TA） is a chronic and nonspecific in- flammatory vessel disease that involves the aorta and its major branches. TA results in stenosis, occlusion or aneu- rysmal degeneration of large arteries pathologically. Al- though TA is an unusual disease, it is more commonly ob- served to occur in Asian females compared to the general population worldwide. Aortic dissection is a very rare com- plication of TA, and only a limited number of TA cases presenting with aortic dissection have been published so far.

Large Vessel Vasculitis in a Patient with Systemic Lupus Erythematosus Presenting as Takayasu’s Pulseless Arteritis

Systemic lupus erythematosus (SLE) with an associated aortoarteritis presenting as an ischemic stroke is a rarity in the medical literature. We report the case of an 11-year-old male presenting with an acute ischemic stroke meeting the criteria for the diagnosis of SLE and findings consistent with an aortitis on imaging but mimicking the diagnosis of Takayasu’s pulseless arteritis. Blood and imaging investigations revealed the finding of SLE aortitis following an acute stroke presentation. Thus, it is imperative to note that even though it is infrequent, SLE can be associated with a large vessel vasculitis.

Syphilitic Aortitis Mimicking Takayasu’s Arteritis

SIR, Syphilis is a sexually-transmitted infectious disease caused by the bacteria Treponemapallidum. A characteristic manifestation of the third stage is arteritis of the aorta, which leads to necrosis and loss of tissue. Here, we report on a 48-year-old woman admitted with suspected Takayasu’s arteritis (TA) due to localized inflammation confined to the aortic root and valve, which could be diagnosed as syphilitic aortitis.

Transthoracic arterial bypass for the treatment of severe cerebral ischemia induced by brachiocephalic arteritis: A report of 15 cases

BACKGROUND： Carotid arterial reconstruction is hard to cure the extensive lesions of carotid arteries and subclavian arteries in patients with brachiocephalic arteritis; however, transthoracic arterial bypass surgery provides an opportunity for the treatment of brachiocephalic arteritis. OBJECTIVE： To report the improving effects of transthoracic arterial bypass surgery on the clinical symptoms of severe cerebral ischemia induced by brachiocephalic arteritis and observe the occurrence of complications after the intervention. DESIGN： Case observation. SETTING： Department of Vascular Surgery, Union Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology. PARTICIPANTS： Fifteen patients with Takayasu arteritis, including 1 male and 14 females, were selected from Department of Vascular Surgery, Wuhan Union Hospital from June 2003 to June 2007. Their ages ranged from 16 to 37 years. All patients were finally diagnosed by clinical histories, health examinations and color Doppler detection. All patients provided the confirmed consent. METHODS： All patients had received transthoracic artificial vascular bypass surgery. Among them, 6 patients had the bridge from ascending aorta to bilateral axillary arteries to lateral internal carotid artery; 3 from ascending aorta to bilateral axillary arteries; 5 from ascending aorta to lateral axillary artery to lateral internal carotid artery; In particular, one patient combining with abdominal aorta occlusion had received two step surgical interventions. In the first step, bridging surgery was performed from ascending aorta to bilateral axillary arteries to lateral internal carotid artery; in the second step, patients received left axillofemoral bypass. MAIN OUTCOME MEASURES：① Detecting blood velocity in variously intracranial-arterial systoles by using transcranial Doppler postoperatively; ② following-up the improve of clinical symptoms at 40 months after surgery; ③ observing postoperative complications. RESULTS： All 15 patients were involved in the final analysis. ① Intracranial-arterial blood velocity： Average intracranial-arterial blood velocity was significantly increased postoperatively （P 〈 0.01）. ② Following-up results of clinical symptoms： All patients did not have death and blindness; while, transient cerebral ischemia, dizzy, photophobia, giddiness, hemoptysis and other symptoms disappeared gradually; therefore, patients were able to live by themselves. ③ Postoperative complications： Among them, 3 patients had injury of hypoglossal nerve and 3 patients had injury of recurrent laryngeal nerve. All recovered after 1 - 3 months conservative treatment. One patient with injury of brachial plexus nerve on one side relieved gradually after a half-year treatment. One patient had occlusion in subclavian arterial bridge on the third day after surgery and once more embolism after arterial embolectomy on the next day; however, the limb did not have obvious ischemic symptoms but low skin temperature as compared with the contralateral side. CONCLUSION： Transthoracic arterial bypass surgery can relieve clinical symptoms of brachiocephalic arteritis postoperatively, complications are mild, and the effects are confirmed.

Urological manifestations and treatment of the primary systemic vasculitides

The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.

Surgical treatment of brachiocephalic vessel involvement in Takayasu＇s arteritis

Background Takayasu＇s arteritis （TA） is a chronic idiopathic inflammatory disease that affects large and medium size arteries. The brachiocephalic trunk is the most frequently involved site in TA, and multi-vessel lesions are common. Surgical treatment includes vessel reconstruction surgery and percutaneous transluminal angioplasty （PTA）. Herein, we report our preliminary experience with surgical treatment of cerebral ischemia caused by cervical arterial lesions due to TA.Methods From January 2000 to December 2007, 38 patients with cerebral ischemia caused by cervical arterial occlusive lesions due to TA were treated surgically. There were three males and 35 females, with an age range of 15-42 years （mean 26.5 years）. All patients had operative repairs undertaken. Twenty eight patients received bypass operation and 10 patients received percutaneous transluminal angioplasty. One case with coronary stenosis received coronary artery bypass simultaneously. Patients were followed up for 11 months to eight years.Results There were no peri-operative deaths in cerebrovascular reconstruction patients. Symptoms of cerebral ischemia were improved or cured in 25 of 38 patients. There was a low incidence of cerebral reperfusion syndrome. Two patients died at five and seven years after surgery due to heart failure. Another 8 patients （20%） required further surgery for stenosis （5 patients） or anastomotic aneurysms （3 patients）. Percutaneous transluminal angioplasty was performed successfully for treatment of aortic and renal lesions. Repeated angioplasty for revascularization was performed in six PTA cases with restenosis after 5-24 months.Conclusions When cerebral perfusion has potential to be affected by TA, a definitive corrective procedure is advised when the patient is relatively stable. Although the recurrence rate is very high, percutaneous transluminal angioplasty is the first choice procedure. Bypass operation is optimal for brachiocephalic-vessel involvement in TA. Cerebral reperfusion syndrome can be avoided by careful selection of the operation method and improved post-operative treatment.

Acupuncture Effects on Endocranial Hemodynamics of Patients with Takayasus Arteritis （Brachiocephalic Artery Type）

Objective： To discuss the action mechanisms of Takayasus arteritis （brachiocephalic artery type） of acupuncture. Methods： We applied acupuncture therapy with the principle of “warming Yang and supplementing Qi, removing obstruction in the meridians and recovering pulse”, to treat patients with takayasus arteritis （brachiocephalic artery type）. Before and after treatment, we detected the TCD （Transcranial Doppler） changes of the average velocity of blood flow （AVBF） and arterial pulsatility index （PI） of related arteris. Results： After treatment, acupuncture and moxibustion can regulate the abnormal blood flow rate in endocranial correlated arteries by two sides, in the AVBF while, elevate the elasticity of endocranial vessels. Conclusion： Acupuncture and moxibustion can regulate cerebrovascular function of patients with takayasus arteritis （brachiocephalic artery type）, increase the perfusion of cerebral blood flow, and adjust abnormal state of endocranial hemodynamics. This is possibly the important action mechanisms acupuncture in treating this disorder.