“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).

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.

Management of the occlusion and stenosis of the aortic arch branches in the course ofTakayasu＇s arteritis

Takayasu＇s arteritis （TA） is a chronic inflammatory disease of the aorta and its thoracic and abdominalparts, most commonly involving the aortic arch and the arteries that arise from it. TA is an autoimmune disease, although certain HLA-linked genetic predispositions have been observed. TA affects females 2 to 8 times more fi＇equently than males. The onset age is between 10 to 40 years old.1 TA is extremely uncommon in Poland. Corticosteroids are the choice of treatment for TA,

Tuberculosis in Takayasu arteritis: a retrospective study in 1105 Chinese patients

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.

Crohn's disease and Takayasu's arteritis: An uncommon association

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.

Anemia in patients with Takayasu arteritis: prevalence, clinical features, and treatment

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.

Drug coated balloon angioplasty for renal artery stenosis due to Takayasu arteritis: Report of five cases

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.

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.

A complicated case of innominate and right common arterial aneurysms due to Takayasu’s arteritis

BACKGROUND Innominate artery aneurysms (IAAs) are relatively rare. Endovascular therapy has been an alternative to open surgery in some IAA cases, but open repair is still necessary in complicated cases. CASE SUMMARY We report a 35-year-old female who suffered from Takayasu’s arteritis. The patient did not get regular treatment, and IAA and right common carotid artery aneurysm developed, which complicated with occlusion of the left carotid artery, subclavian artery, and the initial part of the left vertebral artery. The patient also had moderate aortic valve insufficiency. With inflammation being controlled well, the patient received the surgery for arterial aneurysms of innominate and right common carotid arteries and aortic valve insufficiency. The shunts for cerebral blood supply were designed to protect the brain and the surgery was conducted successfully under extracorporeal circulation. CONCLUSION The case illustrates that open surgery may be appropriate for some complicated IAAs, and brain protection is important.

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.

Mycobacterium tuberculosis in the aorta of a patient with takayasu’s arteritis. extra pulmonary tuberculosis

Takayasu’s arteritis (TA), of unknown etiology, has been related with Mycobacterium tuberculosis infection. The published cases are controversial. A 29- year-old man died, prolapse of the aortic valve and unspecific arteritis were demonstrated at necropsy. Our objective was to search in the fresh aortic tissues, the infectious agent of tuberculosis through culture, as well as by performing histopathology and searching by the molecular study, sequences of genes associated to micobacteria and Ziehl’s stain. Results. The bacillus of tuberculosis was observed by the Ziehl’s stain and the sequences of insertion IS6110 gene were identified. Our findings support the relation of TA with possible tuberculosis infection.Conclusion. The possibility that Takayasu’s arteritis is an extra pulmonary tu-berculosis must be explored.

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.

Prevalence and long-term prognosis in Takayasu arteritis patients with left ventricular dilatation and a decreased ejection fraction:data from 1,105 Chinese cases

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.

Anemia in Chinese patients with Takayasu arteritis

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.

Malignancy in Chinese patients with Takayasu arteritis

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.

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.

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.

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.