Takayasu arteritis is a rare, chronic vasculitis of unknown etiology that predominantly affects young Asian women. The chronic inflammation causes progressive transmural fibrosis of the large arteries, primarily the aorta and its major branches. Most patients present with a constellation of systemic complaints that include fatigue, weight loss, and low-grade fever. However, it is rare for patients to initially present with vascular symptoms, as a hemodynamically significant stenosis is a late manifestation. We present a case of Takayasu arteritis that presented as subclavian steal syndrome.
A 50-year-old female presented to her primary care physician complaining of right arm pain, headaches, and dizziness. She had no medical history and her only surgical history was a right carpal tunnel release that did not improve her symptoms. The patient worked at a local restaurant and stated that when she wiped down counters with her right arm, she was forced to stop after about 5 minutes, secondary to right muscular arm and hand pain. At times, this pain would be accompanied by an intense sensation of dizziness with associated bilateral visual blurring and headache. The symptoms were precipitated only, with right arm activity and would resolve with rest.
On examination, the patient had a palpable decrease in her right radial pulse and had a brachial blood pressure discrepancy of > 30 mmHg, with her left arm measuring 176/92 and her right, 134/84. She was also found to have a loud supraclavicular bruit on the right. Adson’s maneuver and Wright test were negative. Upon initiation of antihypertensive medications, her symptoms were prohibitively exacerbated.
Given our clinical index of suspicion, computed tomographic (CT) imaging of the aortic arch was obtained and revealed a high-grade stenosis of her right subclavian artery (Figure 1).
The patient met the American College of Rheumatology criteria for clinical diagnosis of Takayasu arteritis. Inflammatory markers including C-reactive protein, erythrocyte sedimentation rate, and platelet count at that time were normal, and the patient was clinically without any other stigmata to suggest an active process. After consultation with rheumatology, it was felt that she was in the final “burnt out” phase of the disease and would not benefit from immune modulators. Due to her profound lifestyle-limiting symptoms, she was offered revascularization. Angiography confirmed the CT findings (Figure 2). Percutaneous transluminal angioplasty (PTA) was performed, but with suboptimal resolution due to elastic recoil. Accordingly, a stent was successfully delivered with excellent angiographic result (Figure 3). Our patient experienced complete resolution of her symptoms. Resolution of the pressure gradient enabled appropriate pharmacologic management of her systemic hypertension without precipitation of subclavian steal syndrome.
Subclavian artery involvement is common in Takayasu arteritis and a stenotic lesion proximal to the origin of the vertebral artery can lead not only to ischemic symptoms of the upper extremity, but also retrograde flow through the ipsilateral vertebral artery, a phenomenon referred to as subclavian steal.1 Subclavian steal is associated with ischemic symptoms of the ipsilateral upper extremity, including arm claudication, fatigue, coolness, paresthesias, or numbness. Neurologic symptoms secondary to vertebrobasilar ischemia are rare, but can include dizziness, vertigo, ataxia, disequilibrium, diplopia, nystagmus, and bilateral visual blurring. Takayasu arteritis presenting as subclavian steal syndrome has been rarely reported in the literature.2
Takayasu arteritis is diagnosed clinically using criteria published by The American College of Rheumatology.3 The criteria include age at disease onset less than or equal to 40, claudication of the extremities, decreased pulsation of one or both brachial arteries, difference of at least 10 mmHg in systolic blood pressure between the arms, bruit over one or both subclavian arteries or the abdominal aorta, and arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or other causes. Only three are needed for diagnosis. The disease has three phases that often overlap. The first is an early phase characterized by constitutional features. The second is a vascular phase characterized by progressive fibrosis and symptoms related to stenosis, occlusion, or aneurysms. The final phase is the “burnt out” or clinical remission phase.4 Patients in the active phases of the disease are aggressively treated with corticosteroids and cytotoxic agents targeting clinical remission. The extent of vascular involvement is most appropriately examined with either CT or magnetic resonance angiography, due to its ability to provide additional information regarding the vessel wall, as well as being less invasive.5,6 Percutaneous intervention has been shown to be very effective in focal lesions, while surgical intervention, including bypass, has been shown to increase survival in patients with several extensive lesions.7–9 There is a small body of evidence to suggest the initiation of immunomodulators after PTA to prevent restenosis.10 Our patient declined this option.
How Would You Treat This Patient?
Daniel Clair, MD Chairman/Program Director,
Department of Vascular Surgery, Cleveland Clinic Foundation, Cleveland, Ohio
The authors have presented an interesting case of a complex problem, which we rarely see in the United States. The rare chronic inflammatory disease known as Takayasu’s arteritis affects large arteries, primarily the aorta and its primary branches. The incidence in this country has been recorded at 1–3 cases per million, while in Japan it has been reported as frequently as 1 in 3,000 autopsies. With limited experience in managing a patient’s suffering from this disease, it is important that information from multiple centers or centers of excellence with extensive experience be utilized in making decisions. Several points regarding the medical evaluation of this patient, as well as the management of this patient, deserve further mention. Some of these issues are highlighted in the history of this patient and some, while not mentioned, are of significance to these patients and their care.
In evaluating patients with Takayasu’s disease, there is currently limited ability to assess the extent of the inflammatory state. The serologic inflammatory markers used to give us a sense of the acuity of the disease are crude measures, at best, of the acuity of the disease state. It is clear that most individuals who suffer from this disease chronically have varying degrees of correlation between their serologic markers and the activity of the disease. In light of this, patients who present with this diagnosis at the Cleveland Clinic are routinely evaluated with a complete aortic scan, usually done with MRI imaging, to assess the degree of inflammation within the aortic wall and the primary branch vessel walls. Even this represents a very limited ability to assess the degree of activity of the disease. It is important to also remember that it is the minority of these patients (20%) who will have a self-limited monophasic inflammatory episode. The vast majority will have a progressive, relapsing/remitting course that will require continued follow-up and treatment with immunosuppressive therapy that intermittently may need to be elevated for increased activity of the disease.
In the patient presented, there has clearly been significant activity of the disease. In this patient, as in most who present with this disease, there is already significant restriction of flow through at least one of the major arch branches. The full evaluation with either computed tomography (CT) angiography or more commonly magnetic resonance (MR) angiography allows an assessment of all of the arch vessels and in this case in particular, the status of the contralateral subclavian and vertebral arteries to assess their adequacy in supplying the posterior circulation of the brain. In most cases, since this patient presented with activity enough to have narrowed a subclavian artery, immunosuppressive therapy would likely be initiated. The fact that the disease often progresses along a continuum over a period of time also mandates that this patient have close follow-up to the revascularization done for this problem.
Neurologic and coronary ischemic syndromes are the most common reasons for acute treatment of occlusive lesions in patients affected by Takayasu’s arteritis. The presentation of this patient with subclavian steal syndrome is one of the indications commonly reported needing revascularization therapy.
Both percutaneous and surgical intervention are safe procedures in these patients, however, it must be remembered that there is significant risk of recurrent stenosis in patients treated by any method. The majority of experience in this country involves surgical revascularization. Recurrence rates in treated patients range from 20–30% over five years time. Angioplasty and stenting has extremely variable results, depending upon which vessel is treated, the size the angioplasty and stent procedure and the activity of the disease. In reports evaluating angioplasty and stent treatment for subclavian stenosis, recurrence rates have varied from 10 to as high as 45%. The problem with many of these studies, however, is their limited number of patients and follow up. In addition, many of these series report interventions in multiple vascular beds without separation of identification of exclusive vascular territories treated.
The group of patients known to do very well with surgical revascularization is the group of patients who have autogenous tissue reconstructions via bypass. This patient in particular provides the opportunity for an autogenous tissue reconstruction via carotid subclavian transposition. This procedure can be performed with minimal morbidity and an improved outlook for long-term patency.
It is again important to remember that any revascularization procedure done in patients with Takayasu’s runs significant risk of intimal hyperplasia and recurrent stenosis and therefore, these patients need to be followed closely. The authors here have chosen the ideal lesion to be treated with interventional therapy, which is a short focal stenosis, and in most series, outcomes with interventional therapy rival outcomes with surgical reconstruction.
Ideally, revascularization procedures in this patient population are best performed when the patient is not in an acute period of inflammation. There are, however, some situations which mandate treatment because of the risk related to the vascular bed supplied by the arteries involved. Most commonly, these issues involve the coronary or cerebral circulation. Both interventional and surgical revascularization methods are safe in this patient population and can be done with low morbidity and mortality. It is fairly clear, however, that the best long-term results are obtained with surgical revascularization with recurrence rates that appear to be nearly half of those performed with percutaneous methods. The ideal lesion for percutaneous therapy is a short focal stenosis, as long and occlusive lesions clearly fair less well with interventional therapies. This patient has a lesion, which would be the best in terms of its setting for interventional therapy. The fact is that even in these areas, there can be as high as a 20–30% recurrence rate over 2 to 3 years. It is important to remember that any form of revascularization is subject to the risk of neo-intimal hyperplasia, leading to restenosis of the revascularization procedure. In the setting of surgical reconstruction, the ideal procedure is an autogenous reconstruction to revascularize the vessel at risk. In the setting of this patient, an additional option would be a carotid subclavian bypass or more ideally, a subclavian artery transposition to the carotid artery, which would allow a completely autogenous reconstruction and offer the best long-term durability for this patient. This procedure can be done with minimal morbidity and low risk to the patient and often requires no more than a single night of hospitalization.
While the authors have chosen a therapy that can be safely done and offers this patient the potential of a relatively good result, it is clear that this patient needs to be followed over time and does have a risk of recurrent stenosis, which is not insignificant. In addition, continued follow up for assessment of the level of activity of the disease is mandatory for this patient. Some have argued for aggressive immunosuppressive therapy following interventional and surgical revascularizations for these patients to try and reduce the risk of restenosis, as the authors noted in the paper. There is currently little evidence regarding medicated stents or covered stents to treat these lesions, which may potentially be an alternate strategy in the future.
How Would You Treat This Patient?
Frank J Criado, MD Editor-in-Chief, VDM; Vascular Surgery and Endovascular Intervention;
Union Memorial Hospital/MedStar Health, Baltimore, Maryland
We are indebted to the authors for bringing to our consideration an interesting case that presents certain diagnostic and management challenges. In this country, Takayasu’s disease is an uncommon cause of subclavian artery stenosis and subclavian steal. Most patients we see with subclavian artery stenosis have atherosclerotic disease, often with focal stenotic lesions in the proximal portion of one or both subclavian arteries, the left much more frequent than the right.
Intervention (or surgery) for subclavian steal can only be justified in the presence of significant arm claudication symptoms or manifest vertebro-basilar insufficiency. An asymptomatic patient need not be considered for revascularization because these lesions tend to have a rather benign natural history. The risk of stroke is low, if not nil. An exception may be made on some asymptomatic patients with severe subclavian artery stenosis where “preservation of the left internal mammary artery (LIMA)” is felt to be important for possible future use for myocardial revascularization. However, many cardiac surgeons are reluctant to use a LIMA that is dependent on a stented vessel exposed to restenosis and hemodynamic failure over time, so the argument is weak at best.
I find little, if anything, to disagree with regarding the authors’ approach and choice of interventional therapy. Procedural details are not provided, but it does not hurt to remind the readers of potential difficulties and challenges in performing the procedure on the right subclavian artery. Fluoroscopic and angiographic visualization often requires a right anterior oblique projection with slight cranial tilt. It is paramount to achieve clear “separation” of the subclavian and carotid arteries, with precise assessment of the location of the lesion vis-à-vis the junction with the left common carotid artery. Balloon angioplasty and stenting can usually be performed using a standard transfemoral antegrade approach. However, the retrograde transbrachial access option must be kept in mind, for it can prove valuable, especially in case of a totally occlusive subclavian artery lesion.