- Department of Neurology, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
- Department of Radiology, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
- Department of Rheumatology, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
- Department of Pathology, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
- Department of Neurosurgery, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
- Department of Neuroscience, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
Correspondence Address:
Michael F. Waters
1Department of Neurology, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
6Department of Neuroscience, University of Florida, Archer Road, Gainesville, Florida, 32611, USA
DOI:10.4103/2152-7806.102947
Copyright: © 2012 Hedna VS. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Hedna VS, Patel A, Bidari S, Elder M, Hoh BL, Yachnis A, Waters MF. Takayasu's arteritis: Is it a reversible disease? Case Report and Literature Review. Surg Neurol Int 27-Oct-2012;3:132
How to cite this URL: Hedna VS, Patel A, Bidari S, Elder M, Hoh BL, Yachnis A, Waters MF. Takayasu's arteritis: Is it a reversible disease? Case Report and Literature Review. Surg Neurol Int 27-Oct-2012;3:132. Available from: http://sni.wpengine.com/surgicalint_articles/takayasus-arteritis-is-it-a-reversible-disease-case-report-and-literature-review/
Abstract
Background:Takayasu's arteritis (TA) is a rare and potentially devastating condition leading to prolonged morbidity and even death.
Case Description:We report an 18-year-old female presenting with an acute ischemic stroke treated with intravenous thrombolysis and subsequent endovascular therapy (ET) with excellent results followed by chronic treatment with immunosuppressants after a formal diagnosis of TA. Following immunosupression, improvement was noted in critical stenoses of the extracranial large vessels.
Conclusion:These observations underscore the importance of early initiation of therapy to halt or even reverse vascular pathology, though frequent follow up is mandatory as relapse is common. In this article we provide brief review of the current literature on TA related to pathophysiology, criterion for diagnosis, therapy, and follow up.
BACKGROUND
Takayasu's Arteritis, (TA) also known as pulseless disease, is an idiopathic large vessel vasculitis affecting the aorta and its major branches. Although most commonly seen in Asia, TA is reported in the United States with an incidence of 2.6 cases per million annually[
CASE REPORT
An 18-year-old Hispanic female with no significant past medical history presented with acute onset left side weakness, left hemi neglect, and an national institutes of health stroke scale (NIHSS) stroke scale of 15. The admission computed tomography (CT) angiogram (CTA) of head and neck revealed hyper dense right middle cerebral artery (MCA) with intraluminal clot in the right internal carotid artery (ICA) at the level of the ophthalmic artery extending into the M1 and M2 segment of the MCA. Also observed was near-total stenosis of the right common carotid artery (CCA) [Figures
Figure 1
Pre and Post t-PA/Angioplasty Middle cerebral artery (MCA). (a) There is an acute thrombus (arrow) in the M1 MCA segment (right). The intravenous t-PA has dissolved the supraclinoid ICA thrombus, but no effect on the MCA thrombosis which warranted mechanical endovascular therapy. (b) Postpenumbra device and angioplasty there is TIMI 3 flow in the MCA (left)
She was electively readmitted shortly after discharge for evaluation of her underlying large vessel occlusions and stenoses. This included acute phase reactants and CTA of the chest to inspect the aorta and its branches. ESR (83) and CRP (28) were elevated.
CTA of the aortic arch [Figures
Figure 4
Pretreatment/Post ET CTA of the neck (axial view). (a) CTA axial view show nearly occluded right CCA (thin blue arrow) with minimal cresenteric flow and extensive soft tissue thickening (thick blue arrow) in and around the wall of the vessel. Also seen is the left CCA (thick red arrow). (b) Right (red) and Left (blue) CCA
According to the recent criteria for large vessel vasculitis;[
Figure 5
Posttreatment CTA of the neck (axial view). (a) CTA showing improvement in the caliber and soft tissue deposition of the CCA. Thin blue arrow: improvement in the soft tissue thickening in the wall. Thick red and blue arrows denote Right and Left CCA, respectively. (b) Again shown is the reduced soft tissue deposition in the vessel wall. Arrow denotes Right and Left CCA
DISCUSSION
TA is an idiopathic chronic arteritis with predilection for the aorta and its major branches. Pathophysiology of TA can be divided into early, intermediate and chronic stages for clinical purposes.
In the early stage, there is mononuclear cell infiltration[
In the Intermediate stage, there is secondary deposition of mucopolysaccharides, and fibroblasts along with smooth muscle cell proliferation.[
Finally, in the chronic stage there is further thickening of tunica intima, media, and adventitia along with neovascularization where fibrosis replaces the elastic tissue resulting in luminal narrowing. At this stage, the opportunity for reversibility of vessel pathology is decreased. In this setting, immunomodulators play a role in preventing relapses and new lesions.
Vessel pathology of TA can be varied. Rapid disease progression may lead to aneurysm formation secondary to inadequate fibrosis. Aneurysms may also form secondary to inflammation mediated mural stress. This commonly affects the aorta, subclavian, renal, carotid, and vertebral arteries.[
Aneurysms are common and clinically significant in the aortic root, where they can cause valvular regurgitation. Hypertension is most often caused by renal artery stenosis, but can also be associated with suprarenal aortic stenosis or a chronically damaged, rigid aorta.
The diagnosis of TA involves combination of clinical, laboratory and imaging features. Various validated criteria have been developed over the years which can help diagnose TA with 91% sensitivity and 98% specificity [
Imaging modalities such as conventional angiography, MRI, MRA, CT angiography, or ultrasonography can be used in the diagnosis each having advantages and disadvantages. One of the earliest abnormalities in TA is increased vessel wall thickness which can be detected with ultrasonography. Conventional angiography is the gold standard given its sensitivity for detecting aneurysms and local areas of stenosis. The common carotid and proximal subclavian arteries are assessed using non-invasive color duplex high-resolution ultrasound.[
Both medical and surgical modalities play an important role in disease management [
There have been several studies looking at various immunosuppressants such as Cyclophosphamide, methotrexate, mycophenolate mofetil, and Azathioprine. However, no cytotoxic drug has been shown to have superior efficacy; thus treatment choice depends on weighing each patient's ability to tolerate side effect profiles of particular drugs.[
Recently anti-TNF therapy and Intereukin-6 receptor inhibitor (Tocilizumab) have been tried especially in patient refractory to standard therapy. Following treatment with an anti-TNF agent steroid-free remission was seen. In one study, patients treated with anti- TNF therapy for 28 months resulted in prednisone-free remission in 60% patients with 28% patients achieving remission with daily prednisone therapy <10 mg/day.[
Early surgical intervention in conjunction with institution of appropriate immunosuppressive therapy is key in the management. However, if inflammation is not in remission outcomes maybe less favorable.[
Various clinical and diagnostic factors will be taken into consideration while monitoring the disease activity.[
The sequelae of TA which includes aortic regurgitation, Cardiomyopathy, left ventricular systolic dysfunction, uncontrolled HTN, renal failure, myocarditis, and central nervous manifestations are the principal causes of severe morbidity and mortality. Mortality ranges from 3%-35% at 5 years follow-up depending on the severity of the disease, patient demographics, and geographical distribution.
In summary, See
ACKNOWLEDGEMENTS
Publication of this article was funded in part by the University of Florida Open-Access Publishing Fund.
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