Takayasu Arteritis


Article Author:
Brad Trinidad


Article Editor:
Vasimahmed Lala


Editors In Chief:
Sisira Reddy
Joseph Nahas
Chokkalingam Siva


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
4/2/2019 12:37:29 AM

Introduction

Takayasu arteritis, aka pulseless disease, is a systemic inflammatory condition which leads to damage of the medium and large arteries and their branches. It occurs predominantly in young Asian women. It usually involves the aorta and its major branches, particularly the renal arteries, carotid arteries, and subclavian arteries, and leads to stenosis, occlusions, or aneurysmal degeneration of these large arteries. An abnormality in cell-mediated immunity seems to be its main pathogenesis, but its etiology is still largely unknown. Diagnosis is based on suspicion as well arteriographic findings. Treatment usually begins with medical management using corticosteroids; however, surgical management has become more common recently due to findings of an overall lack of disease regression and high rates of relapse with just medical management alone.

Etiology

The etiology of Takayasu arteritis is largely unknown. At its core, it is an inflammatory disease, and it is thought that autoimmune cell-mediated immunity may be responsible for the disease. Eventual transmural fibrous thickening of the arterial walls is what ultimately leads to ischemic changes and formation of alternating areas of pseudoaneurysm.

Epidemiology

Takayasu arteritis is a rare disease, with a reported worldwide incidence rate of only 1 to 2 per million, the majority being in females (9:1). It is considered a disease of the young, with most being found in patients 40 to 50 years of age[1]. However, it has also been described in young children and the elderly. It is mostly found in patients of Asian or Mexican descent and is rare in North America.

Pathophysiology

There continues to be a substantial lack of understanding of the pathogenesis of Takayasu arteritis, and the etiology is largely unknown. At its core it is characterized as an inflammatory granulomatous vasculitis of medium and large arteries, which leads to transmural fibrous thickening of the arterial walls, leading to multiple vascular obstructions and eventual ischemic changes[2]. Degeneration of elastic fibers is also a feature, with the formation of aneurysms occurring when inflammation leads to loss of medial smooth muscle cells. Cell-mediated immunity involving CD4+ and CD8+ T cells may play a key role in the pathophysiology of Takayasu arteritis, as these cells support the formation of granulomas and potentially activate the activities of various proteases such as matrix metalloproteinase (MMP), as well as other cells which promote chronic inflammation and fibrosis formation. There has also been speculation about a potential link with certain human leukocyte antigens (HLA), although this has not fully been clarified.

Histopathology

Biopsy specimens are a rarely available for Takayasu arteritis but are most common in the unusual case of a potential biopsy of an excised segment, or from an autopsy. Inflammation targets areas around the vasa vasora and at the medio-adventitial junction. Mononuclear cell infiltration is common, and giant cell granulomatous reaction may be present. Reactive fibrosis and increased ground substance in the intima may also be present. In the aorta specifically, the arterial walls will feel extraordinarily stiff and rigid if palpated, with often strikingly high degrees of adventitial and periadventitial fibrosis.

History and Physical

Constitutional symptoms characterize a very nonspecific early acute inflammatory phase seen in Takayasu arteritis which includes fever, malaise, muscle aches, weight loss, and anorexia. Most patients do not seek treatment until signs of the “pulseless” phase occur, in which symptoms are usually secondary to arterial insufficiencies; these include hypertension in renovascular stenosis, neurologic manifestations as seen with carotid artery occlusion, or upper limb claudication as seen in upper extremity ischemia secondary to stenosis. Thus, delayed diagnosis is quite common. Severe debilitating upper or lower extremity ischemia often leads to diminished or absent pulses, hence the name "pulseless disease," or possibly a vascular bruit. Other potential late findings worth noting include accelerated atherosclerosis or heart failure.

Evaluation

Clinical suspicion is needed to diagnose Takayasu arteritis, and thus a good, complete history and physical exam is a necessity. Imaging is vital, and CTA has recently become the standard for initial staging of disease distribution (previously it was conventional angiography) in Takayasu arteritis. This standard is based on the 1994 Tokyo International Conference Classification of Takayasu arteritis. CTA will allow for visualization of vessel wall thickening and luminal narrowing. MRI and MRA may also be used for identification and provide excellent multiplanar imaging without ionizing radiation use. However, like with all MRA, it tends to overestimate the degree if stenotic disease and access to MRI or MRA are much more difficult.

Treatment / Management

Initial treatment of symptomatic Takayasu arteritis begins with corticosteroids. Immunosuppressive medications have also been used in place, or in combination with corticosteroids. However, studies have not conferred an overall advantage of these medications.

The benefits of revascularization for these conditions are well recognized, but the uncertainty of this disease process had tempered enthusiasm for primary surgical approaches. Thus, traditionally, surgical therapy had been reserved for symptomatic manifestations of arterial occlusive disease refractory to corticosteroids and/or immunosuppressive therapy. However, this experience seems to be changing recently as the efficacy and ability of medical management to arrest disease has been called into question. A recent nationwide study revealed that 50% of patients with Takayasu arteritis will relapse and experience a vascular complication less than 10 years from diagnosis. Those most likely to relapse include males and those with elevated C-reactive protein.

Surgical revascularization is now rapidly evolving into a primary treatment choice. Transluminal angioplasty has been studied in the past. However, its overall role is limited in the treatment of patients with Takayasu arteritis. The fibrous nature of the arterial obliteration mitigates any durable long-term benefit[3]. A study at the Cleveland Clinic revealed 78% of their patient cohort treated with angioplasty in various anatomic locations experienced restenosis during a median 3-year follow-up, and 93% required reintervention at some point. Thus, PTA should be viewed a short-term strategy for symptomatic relief if other medical needs must be attended to first in a given patient.

Arterial reconstruction is the other surgical option in patients with Takayasu arteritis. The type of reconstruction depends on where known lesions lie, as well as the patient’s surgical anatomy. In general, Dacron or polytetrafluoroethylene (PTFE) would be used for major aortic reconstructions, while autogenous saphenous vein would be used for extremity or isolated renal or mesenteric revascularization. Caution should be used when using autogenous saphenous vein secondary to its known propensity for aneurysmal degeneration. It is important that both proximal and distal anastomotic sites are free of any inflammatory involvement. Anastomosis onto involved arterial segments is a setup for early graft failure. In a study of 40 patients with TA at the University of Southern California, renovascular procedures were most common[4]. Other procedures included various aortic reconstructions, aorta-carotid bypasses for cerebrovascular insufficiency, and bypasses to distal subclavian, axillary, or brachial arteries for upper extremity ischemia.

Surgical therapy is usually well tolerated due to the young age of most patients with Takayasu arteritis. Most have dramatic improvements in their symptoms following surgery, whether it be decreases, or even curing, of hypertension following renovascular reconstruction, or resolving of upper limb ischemia following upper extremity bypass for example. These findings suggest that surgical correction of symptomatic Takayasu arteritis will continue to rise.[5][6][7][8]

Enhancing Healthcare Team Outcomes

TA is a complex vascular disorder that has no cure. It is best managed with an interprofessional team that includes a vascular surgeon, radiologist, cardiologist, internist, neurologist, and nephrologist. A nurse should provide assistance with and coordination of patient education. While isolated lesions may be stented, long lesions may require a bypass. Some patients may benefit from steroids and other immunosuppressive agents. These patients will need long term monitoring for adverse side effects of the drugs by the nurse practitioner and primary care provider. The prognosis of patients with TA is guarded.[9][10]


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Takayasu Arteritis - Questions

Take a quiz of the questions on this article.

Take Quiz
A 45-year-old Asian female presents to your office with concerns over not being able to feel either one of her radial artery pulses. She notes this is a new occurrence. She says that she has felt "feverish" for the last few weeks, with associated fatigue, weight loss, and arthralgias. On examination, her radial pulses are indeed non-palpable. What is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 48-year-old Asian female presents with severe left upper extremity claudication. The extremity is colder to the touch than the right. Radial, ulnar, and brachial pulses are absent on the left but are readily palpable on the right. She denies a history of irregular heart rate or any other cardiovascular problems. She is otherwise asymptomatic and denies associated rash or erythema. What is the most likely etiology of her symptoms?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Select the finding not expected with Takayasu arteritis.



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following findings best correlates with the diagnosis of Takayasu arteritis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which is not seen with Takayasu arteritis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What has become the standard for initial imaging and staging of Takayasu arteritis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is the least likely symptom of Takayasu arteritis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Takayasu Arteritis - References

References

Takayasu arteritis: a review., Johnston SL,Lock RJ,Gompels MM,, Journal of clinical pathology, 2002 Jul     [PubMed]
Pathogenesis of Takayasu's arteritis: a 2011 update., Arnaud L,Haroche J,Mathian A,Gorochov G,Amoura Z,, Autoimmunity reviews, 2011 Nov     [PubMed]
Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis: A Multicenter Study of 318 Patients., Comarmond C,Biard L,Lambert M,Mekinian A,Ferfar Y,Kahn JE,Benhamou Y,Chiche L,Koskas F,Cluzel P,Hachulla E,Messas E,Resche-Rigon M,Cacoub P,Mirault T,Saadoun D,, Circulation, 2017 Sep 19     [PubMed]
Mason JC, Surgical intervention and its role in Takayasu arteritis. Best practice     [PubMed]
Shah B,Chhetri R, Malignant Ischemic Stroke in a Young Female: A Rare Primary Manifestation of Takayasu Arteritis. Case reports in neurological medicine. 2019;     [PubMed]
Vargas-Hitos JA,Jiménez-Jáimez J,Molina Navarro E,Salmerón Ruiz A,López Milena G,Jiménez-Alonso J, Takayasu arteritis, malignant hypertension and severe left ventricular hypertrophy. International journal of rheumatic diseases. 2019 Mar 12;     [PubMed]
Kwon OC,Oh JS,Park MC,Hong S,Lee CK,Yoo B,Kim YG, Statins reduce relapse rate in Takayasu arteritis. International journal of cardiology. 2019 Feb 20;     [PubMed]
Chauhan R,Luthra A,Mahajan S, Takayasu Arteritis with Cerebral Hypoperfusion: An Unusual Case. Journal of neurosciences in rural practice. 2019 Jan-Mar;     [PubMed]
Fan L,Zhang H,Cai J,Yang L,Liu B,Wei D,Yu J,Fan J,Song L,Ma W,Zhou X,Wu H,Lou Y, Clinical course and prognostic factors of childhood Takayasu's arteritis: over 15-year comprehensive analysis of 101 patients. Arthritis research     [PubMed]
Berti A,Dejaco C, Update on the epidemiology, risk factors, and outcomes of systemic vasculitides. Best practice     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Rheumatology. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Rheumatology, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Rheumatology, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Rheumatology. When it is time for the Rheumatology board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Rheumatology.