Takayasu arteritis is a rare large-vessel arteritis characterized by arterial fibrosis, granulomas, and stenoses. It displays a predilection for Asian women and primarily involves the aorta and related branches.
Presentation
Takayasu arteritis is a type of arteritis that causes inflammation of the large and medium-sized arteries, which further leads to aneurysms, stenoses, and fibrosis of the affected artery. The symptomatology elicited is dependent on the precise vessel affected, and in the majority of the cases, focal symptoms are experienced indicating an abnormally low perfusion of the affected organ or extremity. The disease exhibits a clear predilection for female patients [1] [2].
The aorta is the vessel most commonly affected by Takayasu arteritis; the most prominent symptoms resulting from the stenoses located in the descending thoracic aortic wall include hypertension, pain, and weakness of the arms, absent or asymmetric pulse in the extremities, leg claudication, and headaches. Renovascular hypertension may be induced in the case in which the abdominal aorta or renal arteries are involved in the inflammatory process.
If the carotid arteries are also affected by the disorder, symptoms related to cerebral hypoperfusion occur. These include strokes, visual impairment, temporary loss of consciousness, orthostatic hypotension, dizziness and headaches [3] [4]. On the other hand, stenoses in the subclavian arteries in the regions proximal to a patent vertebral artery can lead to the so-called subclavian steal syndrome; The backflow of blood from the vertebral to the subclavian artery, when the corresponding arm is set in motion, leads to a diminished flow of blood in the subclavian artery. Therefore, syncope or ischemic symptoms are elicited.
Finally, the involvement of the pulmonary arteries causes pulmonary hypertension and infarcts [5]. The former is associated with fatigue, cyanosis, leg edema, dyspnea, and syncope, as it progresses, and the latter may present with tachypnea, dyspnea, hemoptysis, pleuritic chest pain and cough [6]. If coronary arteries also partake in the inflammatory process, the clinical picture is further complicated by angina pectoris or symptoms related to a myocardial infarction and heart failure.
Entire Body System
- Anemia
Correction of anemia improves oxygen delivery to the hypoxic retina and relieves recurrent amaurosis. [ncbi.nlm.nih.gov]
There is often anemia with raised inflammatory markers. This phase gradually resolves with the initiation of the chronic phase which is characterized by inflammatory and obliterative changes in the aorta and its branches. [radiopaedia.org]
In fact, patients affected by Takayasu arteritis exhibit an elevated erythrocyte sedimentation rate (ESR), mild or no leucocytosis and normochromic anemia during periods of exacerbation. [symptoma.com]
- Coronary Atherosclerosis
[…] is unclear but some authors have suggested that as well as formation of stenoses, the inflammation itself accelerates coronary atherosclerosis.8 Hypertension is a common comorbidity, found in 33–83% of cases,4 linked to renal artery stenosis (observed [revportcardiol.org]
- Chills
However, many clinical findings may be similar between the two diseases, including the presence of constitutional symptoms (fatigue, fevers, chills, malaise, weight loss), muscle and joint pains, stroke-like symptoms, diminished or absent pulses, asymmetric [vasculitisfoundation.org]
- Raynaud Syndrome
’s syndrome (15%) .Pericarditis(< 8%) ,Congestive heart failure (< 7%), Myocardial infarction (< 3%). 12. [de.slideshare.net]
Cardiovascular
- Chest Pain
pain and Takayasu arteritis Key words: Takayasu arteritis, Persistent fever, Chest pain Takayasus arteritis (TA) is an extremely rare vasculitis that presents in paediatric age that typically affects adolescents. [medicoebambino.com]
Takayasu Disease Symptom Chest Pain There are many causes of chest pain. A serious form of chest pain is angina, which is a symptom of heart disease and results from inadequate oxygen supply to the heart muscle. [medicinenet.com]
A 34-year-old woman was taken to the Emergency Department of our hospital, presenting with a sudden onset and persistent retrosternal chest pain radiating to both upper extremities for 2 hours. [ncbi.nlm.nih.gov]
- Decreased Radial Pulse
Symptoms may include: Arm weakness or pain with use Chest pain Dizziness Fatigue Fever Lightheadedness Muscle or joint pain Skin rash Night sweats Vision changes Weight loss Decreased radial pulses (at the wrist) Difference in blood pressure between the [nlm.nih.gov]
- Subclavian Bruit
Examination demonstrated subclavian bruits, palpable epigastrium aortic pulsation, faint radial pulses, right retinal pallor, arteriolar narrowing, and bilateral boxcarring. [ncbi.nlm.nih.gov]
Musculoskeletal
- Muscle Weakness
Muscle weakness and pain during arm and leg movement. A weak pulse in the brachial artery, the major artery in the upper arm. A bruit, or a “whooshing” sound, which can be heard through a stethoscope, in the subclavian artery or the aorta. [vascular.surgery.ucsf.edu]
Physician’s use the following criteria to distinguish Takayasu’s arteritis from other forms of vasculitis : Symptoms beginning at age 40 or younger Muscle weakness and pain when you move your arms or legs A weak pulse in the brachial artery, the major [ucdmc.ucdavis.edu]
weakness and pain when you move your arms or legs A weak pulse in the brachial artery (major artery in the upper arm) A sound called a bruit, which indicates turbulent blood flow, when your doctor listens to your aorta or subclavian artery with a stethoscope [drugs.com]
Neurologic
- Headache
A 38-year-old Sri Lankan female presented with sudden onset severe headache, fixed dilated pupil, complete ptosis and ophthalmoplegia on the right side. [ncbi.nlm.nih.gov]
These include strokes, visual impairment, temporary loss of consciousness, orthostatic hypotension, dizziness and headaches. [symptoma.com]
Ramya from Salem district came in a few months ago, with severe headache, giddiness and leg pain. “I had gone to a private hospital at first, but they said they could not do anything,” said the 21-year-old. [thehindu.com]
Snapshot A 62-year-old woman presents to her primary care physician due to an unremitting headache. She says she has been having this headache for the last few days and is nonresponsive to ibuprofen. [step1.medbullets.com]
- Seizure
The neurological symptoms of the disease vary depending on the degree; the nature of the blood vessel obstruction; and can range from lightheadedness to seizures (in severe cases). [en.wikipedia.org]
Carotid and vertebral arteries (brain) – dizziness, fainting, headaches, visual disturbances, orthostatic hypotension, seizures, transient ischemic attacks (TIA’s) or a stroke. [healthhype.com]
They can vary depending on which artery is blocked, and may include problems such as: High blood pressure Heart attack Stroke Gastrointestinal bleeding Seizures Key points about Takayasu's arteritis Takayasu's arteritis is a rare autoimmune disease. [saintlukeshealthsystem.org]
They can vary depending on which artery is blocked, and may include problems such as: High blood pressure Heart attack Stroke Gastrointestinal bleeding Seizures Key points about Takayasu arteritis Takayasu arteritis is a rare autoimmune disease. [cedars-sinai.edu]
- Paresis
Presently, she remains aphasic with mild right upper extremity paresis, for which she is undergoing rehabilitation. Discussion This case highlights the ability of helical CT angiography to evaluate all portions of the vessels. [ajnr.org]
Workup
The fist step towards diagnosing Takayasu arteritis is a meticulous physical examination. Any symptom that indicates hypoperfusion of organs supplied with blood flow from the aorta or its branches raises suspicion about the condition. The absence of a pulse in the extremities, especially in patients who are unlikely candidates for other cardiovascular diseases, is also an indicator of Takayasu arteritis. Additionally, the physical examination may reveal arterial bruits and a difference in the blood pressure measured in the two arms or legs.
Imaging modalities that help to establish a definitive diagnosis include a magnetic resonance angiography (MRA) or a computerized tomography angiography (CTA), which can illustrate Takayasu arteritis-related vascular pathologies, such as stenoses with post-stenotic dilatation, obstruction of blood flow, collateral circulation or aneurysms [7]. Laboratory tests are also usually carried out but contribute little to the differential diagnosis, due to their extremely low specificity. In fact, patients affected by Takayasu arteritis exhibit an elevated erythrocyte sedimentation rate (ESR), mild or no leucocytosis and normochromic anemia during periods of exacerbation [8] [9]. Other laboratory findings include anti-endothelial antibodies, which also do not assist in the final diagnosis, given the fact that they are detected in multiple other autoimmune diseases, such as connective tissue diseases. In recent years, it has been proposed that soluble vascular cell adhesion molecule-1 (VCAM-1) levels are considerably elevated in the serum of patients affected by Takayasu arteritis.
Blood pressure measurement has to be measured meticulously in such patients. In cases where both the subclavian arteries display abnormalities associated with Takayasu arteritis, blood pressure can accurately be measured solely in the lower extremities. Should the iliac or femoral arteries be affected as well, only a central arterial pressure measurement provides accurate results. The latter needs to be evaluated because the inability to measure peripheral pressure could mask hypertension.
Laboratory
- Leukocytosis
Abdomen examination revealed no abnormal findings Laboratory investigations: leukocytosis, hemoglobin was 10.9 gm/dl, erythrocytes sedimentation rate was 70 mm and C-reactive protein 75.8 g/l (normal < 6g/l). [jneuro.com]
Typical laboratory findings are indicative of a generalized inflammatory process and include elevations in erythrocyte sedimentation rate and C-reactive protein as well as findings on complete blood counts that include leukocytosis, anemia, and/or anemia [rheumatologyadvisor.com]
Treatment
One month later, the second treatment was performed. [ajnr.org]
She was started on a drug treatment for vasculitis with deflazacort at 30 mg/day. Figure 1. [bjn.org.br]
Balloon dilatation of stenosed segments has revolutionised the treatment of Takayasu's arteritis. [ncbi.nlm.nih.gov]
In this article, the author highlights the potential for vascular wall imaging methods to detect and monitor vascular inflammation before occlusion occurs and reviews the evidence for effective therapy with biological agents in the treatment of this uncommon [medlink.com]
Prognosis
Takayasu arteritis is an uncommon inflammatory disease with usually a good prognosis. However, sometimes, the evolution can be fatal essentially by a coronary arteries involvement. [ncbi.nlm.nih.gov]
Prognosis tends to be variable ranging from a rapidly progressive disease in some reaching a quiescent stage in others. History and etymology The condition is named after Dr. [radiopaedia.org]
Etiology
Takayasu arteritis (TA) is a systemic vasculitis of unknown etiology that affects the aorta and its primary branches or large arteries in the proximal upper or lower extremities. [ncbi.nlm.nih.gov]
Etiology The etiology of Takayasu arteritis is unknown. [emedicine.com]
Epidemiology
BACKGROUND: Studies investigating the epidemiology and mortality of Takayasu Arteritis (TA) are scarce. [ncbi.nlm.nih.gov]
Pathophysiology
Serum IL-6 seems to be the best biomarker for disease state and disease activity in TA and increased Th1 and Th17 responses are predominant in the pathophysiology of TA. Copyright © 2017 Elsevier B.V. All rights reserved. [ncbi.nlm.nih.gov]
Conclusion Our findings indicate novel genetic susceptibility loci for Takayasu arteritis and uncover potentially important aspects of the pathophysiology of this form of vasculitis. [doi.org]
Prevention
Hence, prevention with control of perioperative hemodynamic changes during the pregnancy is an effective method to prevent heart failure, embolism, and thrombosis. [ncbi.nlm.nih.gov]
References
- American College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology. Systemic Vasculitis. 2009; 65–67.
- Shikino Kiyoshi, Takako Masuyama, Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014 Jul; 29(7): 1072–1073.
- John Barone, M.D. USMLE Step 1 Lecture Notes. Vascular Pathology. 2008; Kaplan Inc.101.
- Milan B, Josip. Ocular manifestations of the aortic arch syndrome (pulseless disease; Takayasu's disease) (Translated from French). 1967. Annales d'oculistique. 1967; 200 (11): 1168–79.
- Zeng QX, Liu ZH, He JG, et al. Clinical features of Takayasu arteritis patients with pulmonary hypertension due to pulmonary artery involvement. 2016. Apr 26;96(16):1252-5.
- Magalhães P, Morais A, Carvalho S, et al. Chest Pain: The Need to Consider Less Frequent Diagnosis. Case Rep Cardiol. 2016; 2016:4294780.
- Andrews J, Mason JC. Takayasu's arteritis--recent advances in imaging offer promise. Rheumatology (Oxford). 2007 Jan.;46(1):6-15.
- Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994 Jun 1;120(11):919-29.
- Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar; 56(3):1000-9.