Large vessel vasculitis

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures.     

A girl with Takayasu arteritis associated with possible systemic lupus erythematosus

Takayasu arteritis is an inflammatory disease that affects the aorta and its main branches. Its etiology is obscure. Its association with systemic lupus erythematosus has been reported in the English literature in about 20 cases worldwide, and a relationship with a positive tuberculin test, either with or without tuberculosis, has also been mentioned. We report a pediatric patient who presented with renovascular hypertension secondary to Takayasu arteritis associated with a strongly positive tuberculin test and who subsequently developed possible systemic lupus erythematosus 8 months later.     

Aortic stent and renal autotransplantation for the management of renovascular hypertension with Takayasu’s arteritis: report of a case

Takayasu’s arteritis (TA) is a rare inflammatory disease affecting the aorta and its major branches. In patients with TA, middle aortic syndrome with aortic and renal artery involvement causes severe hypertension that does not respond well to medical therapy. Currently, the optimal therapeutic options have not been established, and the reported results of different treatments vary widely. We herein present a case of middle aortic syndrome with renovascular hypertension caused by TA in a 12-year-old male treated by an aortic stent and renal autotransplantation as a two-staged procedure.     

Blood pressure measurements in patients with Takayasu arteritis: a work of caution

Takayasu arteritis is a large vessel vasculitis among young women that affects the aorta and its branches. Disease-related subclavian or brachial obstructions can lead to hypotensive brachial blood pressure values. By contrast, arterial hypertension is also frequent in this disease, possibly unrecognized in case of solitary brachial blood pressure measurement. We present the case of a 28-year-old woman with cerebral hypoperfusion related to Takayasu arteritis. Despite "pseudo-hypotensive" brachial blood pressure values of 70 mm Hg, Doppler measurement of the ankle revealed a systolic pressure of 220 mm Hg. In Takayasu vasculitis, additional ankle pressure measurement may represent the true cardiac load and blood pressure.     

A Rare Cause of Renovascular Hypertension: Takayasu Arteritis with Only Renal Artery Involvement

Takayasu arteritis is a chronic inflammatory disease that affects mainly the aorta, main branches of aorta, and pulmonary arteries with unknown etiology. Disease affecting solely the renal arteries is rare. We will present a case that had hypertension, hypokalemia, and metabolic alkalosis where the etiology was type 2 Takayasu arteritis, affecting renal arteries.     

Multiple isolated aneurysms in a case of "burned out" Takayasu aortitis

Takayasu aortitis (TA) is a chronic inflammatory disease predominantly seen in young Asian women. The disease is idiopathic and largely affects the aorta and its major branches. The basic pathologic changes in TA are fibrosis and subsequent occlusion of the large arteries. TA is classically termed "pulseless" disease, with manifestations during the occlusive stage including limb ischemia, renovascular hypertension, and heart failure. Arterial dilation and aneurysm are largely unappreciated manifestations of TA, but they occur in as many as 32% of affected patients. We report chronic "burned out" TA in a 23-year-old Hispanic woman with isolated aneurysms of the descending thoracic aorta, abdominal aorta, and common iliac arteries, without occlusive disease.     

Fulminant development of mega-aorta due to Takayasu's arteritis: case report and review of the literature

Takayasu's arteritis is a rare inflammatory arteriopathy characterized by segmental involvement of the aorta, its major branches, and, occasionally, the pulmonary arteries. Arterial inflammation generally results in occlusion, but Takayasu's arteritis occasionally presents as aneurysm formation. Takayasu's arteritis generally afflicts young women and is most often characterized by an acute episode of systemic illness and neurologic symptoms secondary to stenoses of the carotid and vertebral circulation. We report an unusual case of Takayasu's arteritis in a 43-year-old man who presented with severe back pain and provide a brief review of the literature.     

Coronary artery bypass grafting for Takayasu arteritis with severe coronary, carotid, subclavian, and renal artery involvement and subsequent pregnancy

Takayasu arteritis is a rare idiopathic large-vessel vasculitis that involves the aorta and its major branches. It affects young women in their child-bearing period. We present a case of a 32-year-old lady with a history of remittent fever. Magnetic resonance angiography and arch aortogram showed aortic involvement with critical stenosis of both carotid and subclavian arteries. She also had critical ostial left main and right coronary artery stenosis as well as severe renal arteries involvement. The patient underwent coronary artery bypass grafting. She did well and after 12 months she got pregnant. She had an uneventful pregnancy and delivery without flaring of symptoms.     

Mega-aorta syndrome development in giant cell arteritis. A same entity?

Giant cell arteritis (GCA) is the most common form of large vessel arteritis. GCA typically involves the branches of the external carotid artery, but is the leading cause of inflammatory aortitis. However, involvement of the aorta often goes undetected. We present a case of an 81-year-old man, with headache and intense chest pain, who was previously given a diagnosis of GCA with a temporal artery biopsy 6 years ago. Owing to the suspicion of acute aortic syndrome, an emergent computed tomography (CT) was performed. CT showed the development of mega-aorta syndrome, with a diameter of 75.2 mm in the ascending aorta, 61.8 mm in the aortic arch, 76.1 mm in the descending thoracic aorta, and 45.1 mm in the abdominal aorta, presenting a chronic type B aortic dissection. Although there are reported cases secondary to Takayasu arteritis, this is the first case reported in the literature of mega-aorta syndrome associated with GCA in a patient previously diagnosed using temporal artery biopsy.     

The Asia Pacific meeting on vasculitis and ANCA 2012 workshop on Takayasu arteritis: advances in diagnosis and medical treatment

Takayasu arteritis is a rare disease affecting especially young females. Nonspecific symptoms make the diagnosis difficult; cases in which a diagnosis has not been made for a long period are not rare. However, recent progress in imaging modalities including magnetic resonance angiography, computed tomography angiography, and positron emission tomography have allowed making specific diagnoses in the early stage. Although specific serological markers of this disease are not known, many biomarkers specific to arterial inflammation are being developed and applied for diagnosing this disease. Also, advances in immunosuppressive treatment including new biological agents could alter the clinical outcome of the disease. According to these changes in diagnosis and treatment, the prognosis of Takayasu arteritis has been improving.     

Carotid and coronary artery occlusion in a patient with Takayasu arteritis

Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its principal branches, leading to narrowing and occlusion of the vessels. Since the clinical manifestations depend on the location and severity of the lesions, stroke may be the initial presentation of the disease. Here we report the case of a young man with TA and complete thrombotic occlusion of the right common carotid artery who underwent surgical intervention at our institution. His postoperative computed tomography angiogram performed after 3 months revealed also total occlusion of the left anterior descending coronary artery from its proximal portion.     

Primary arteritis of the aorta and its branches

Primary arteritis of the aorta and its branches is a single clinicopathological entity affecting one or more segments of the aorta and resulting in a variety of symptom complexes. The prevalence is not known, but it is an important cause of renovascular hypertension in the black population of South Africa. Recently published articles on primary arteritis of the aorta and its branches are reviewed, and an attempt is made to draw conclusions regarding its cause, clinical features and treatment.     

Surgical procedures in the management of Takayasu's arteritis

Takayasu's arteritis is an inflammatory arteriopathy that often progresses to obliteration of multiple large arteries. Variable results have been reported after medical and surgical management. Twenty female patients with Takayasu's arteritis were treated from 1973 to 1989. Eleven (55%) patients had hypertension. Upper or lower extremity ischemia was present in 12 (60%) patients and cerebrovascular insufficiency in seven (35%). Nine patients initially managed with corticosteroids had no improvement in signs or symptoms of arterial insufficiency. Eleven patients had 16 vascular procedures for the following indications: renovascular hypertension (6), extremity ischemia (5), cerebrovascular insufficiency (2), dilation ascending aorta with aortic insufficiency (1), thoracic aortic aneurysm (1), abdominal aortic aneurysm (1). Procedures included aortorenal bypass (5), carotid-subclavian, axillary, or brachial bypass (4), aorto-carotid bypass (2), aneurysm resection (2), supra-celiac aorto-femoral bypass (1), ascending aorta/aortic valve replacement (1), and nephrectomy (1). Clinical improvement occurred in all patients. There were no operative deaths. All are alive at a mean follow-up of 5.75 years (6 months to 16 years). Revision of the initial reconstruction has been required for recurrent renovascular hypertension in one patient and extremity ischemia in another. The other nine patients remain symptomatically improved. Symptomatic Takayasu's arteritis frequently requires arterial reconstruction. Symptomatic improvement and excellent long-term graft patency can be expected after arterial reconstruction.     

Renovascular hypertension due to Takayasu arteritis--a case report

A 18-year old women with bilateral renal artery stenosis and renovascular hypertension as a part of Takayasu's disease is presented. Takayasu's disease is a chronic arteritis of unknown origin, primary affecting aorta and its branches. The disease is the most common in young women and is more common in the countries of eastern Asia in comparison with Western countries. Takayasu's disease can be severe and lifetreating disease. Mortality and morbidity depend on both the direct effect of the vascular lesions and the complications od disease. The mainstay of Takayasu's disease is based on the use of glucocorticoids alone or in association with cytotoxic drugs (cyclofosfamid, azatioprin, metotrexate). In the majority of cases, the partial control of disease is obtained.     

Takayasu's arteritis: surgical considerations.

Takayasu's arteritis, the nonspecific occlusive disease originally believed to affect young Oriental women exclusively, was identified in a 19-year-old man who had severe involvement of the aortic arch and its branches. Successful surgical treatment was accomplished with a bypass from the ascending aorta to the supraceliac abdominal aorta using a tube graft. The right vertebral artery, as the single patent vessel reaching the head, was revascularized distal to the stenosis with a tube graft that extended from the aortic graft. Revascularization of ischemic organ systems and body areas may be accomplished in most patients with Takayasu's arteritis using the bypass concept and fabric conduit grafts.     

Patterns of aortic involvement in Takayasu arteritis and its clinical implications: evaluation with spiral computed tomography angiography

OBJECTIVE: Although the luminal changes of Takayasu arteritis are well depicted with conventional angiography, its mural changes can be best evaluated with spiral computed tomography (CT) angiography. Here, the authors investigated the patterns of aortic involvement in Takayasu arteritis by using CT angiography. METHODS: CT angiography was performed from the carotid bifurcation to the iliac bifurcation in a consecutive 85 patients (M:F = 10:75, mean age: 37 years) with Takayasu arteritis. Two radiologists interpreted axial images and three-dimensional reconstructed images by consensus with respect to disease extent, lesion continuity, and disease activity based on mural and luminal changes on CT angiography. RESULTS: Eighty-one (95%) patients had aortic involvement with or without aortic branch involvement, and the other four (5%) patients had only aortic branch involvement. In terms of aortic branches, the left common carotid artery (77%) and the left subclavian artery (76%) were most commonly involved. Extent of disease involvement assessed by mural change was wider than that assessed by luminal change in 52 (61%) patients. Although arterial involvement was contiguous in 69 (81%) patients, skipped lesions were identified in 16 (19%) patients. An analysis of mural findings revealed the coexistence of active and inactive lesions in nine (11%) patients. CONCLUSIONS: Aortic involvement in Takayasu arteritis can occur from the aortic root to below the iliac bifurcation, and isolated branch vessel involvement is also possible. In most patients, aortic involvement occurs in a contiguous, synchronous fashion. However, skipped involvement and the coexistence of active and inactive lesions also occur.     

Aortic Arch Branches are Occluded: Which Artery Nourishes the Brain?

Takayasu arteritis is an inflammatory vascular disease that primarily affects the aorta and its major branches. In this report, we present a 47-year-old woman whose aortic arch branches were totally occluded and the cerebral circulation is dependent on a dilated right vertebral artery.     

Large renal artery aneurysm in Takayasu arteritis

Takayasu arteritis (TA), a primary arteritis of unknown cause, commonly affects the aorta and its main branches. Most patients with TA have stenotic or occlusive changes; aneurysmal degeneration is relatively rare, and renal artery aneurysms are extremely rare. We observed a fusiform, 7-cm renal artery aneurysm in a 57-year-old man with TA who had been followed up for 17 years. The patient underwent nephrectomy and resection of the aneurysm. Histologic examinations of resected specimens of the lesion showed characteristics typical of TA.     

Anesthetic Management of a Patient With Takayasu Arteritis

Takayasu arteritis is a rare chronic progressive panendarteritis involving the aorta and its main branches. Anesthesia in patients with this disease can be complicated by severe uncontrolled hypertension, end-organ dysfunction, and stenosis of major blood vessels. In this case, general anesthesia was induced with sevoflurane and remifentanil without complications. To prevent intraoperative complications, we conducted intubation with a rigid video laryngoscope with careful consideration of the concentrations of analgesics and sedatives used. This case demonstrates the importance of anesthetic techniques for maintaining adequate tissue perfusion without hemodynamic changes in the anesthetic management of patients with Takayasu arteritis.Key Words: Takayasu arteritis, Anesthetic management, General anesthesia, DentistryTakayasu arteritis is an idiopathic and chronic inflammatory disease characterized by the formation of stenoses or abnormal aneurysms in large vessels, resulting in life-threatening ischemia in major organs. Takayasu arteritis predominantly affects young females under 40. The etiology of this disease has not been defined.^1,2Some reports have emphasized problems in the anesthetic management of a patient with Takayasu arteritis.^3–6 However, limited information is available. We present a case of general anesthesia in a patient with Takayasu arteritis and discuss specific anesthetic management and considerations required in such cases.     

A case of pulmonary artery bypass surgery for a patient with isolated Takayasu pulmonary arteritis and a review of the literature.

A 45-year-old female was presented with progressive dyspnea and bilateral leg edema. Pulmonary angiography revealed total occlusion of the right pulmonary artery and significant stenosis of the left pulmonary artery. The inferior lobar artery as well as the segmental arteries were well patent. No pathology was detected elsewhere at the aorta and its branches. The diagnosis of chronic pulmonary arterial occlusion by isolated Takayasu arteritis was made because of the characteristic pattern of angiographic findings and the presence of unusual shunt formation from the coronary artery to the peripheral portion of the pulmonary artery, as well as a characteristic presentation of HLA typing in blood analysis, which strongly suggested the diagnosis of Takayasu arteritis. To restore the pulmonary blood flow, we employed reconstructive surgery by means of bypass procedure, using PTFE graft. Postoperatively there was marked improvement in cardiopulmonary function and the quality of life of the patient. The graft was proved to be patent at long-term follow-up study. An extremely rare case of chronic occlusive pulmonary arteritis, which was surgically treated by means of bypass procedure, is reported herein, and a brief review of previous reports on this subject was attempted.