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.
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  .
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  . 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 . 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 . 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
Correction of anemia improves oxygen delivery to the hypoxic retina and relieves recurrent amaurosis. [ncbi.nlm.nih.gov]
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]
Patients with TAK may also have anemia due to chronic (long-term) inflammation. Anemia is also tested for with a blood test. [rheumatology.org]
In view of the chronic process and good collateral development, Raynaud's phenomenon or digital gangrene are very rare in Takayasu arteritis. A rare complication of this condition are coronary artery aneurysms. [en.wikipedia.org]
Keeping warm If your fingers or toes turn blue in response to the cold, this may be due to Raynaud's phenomenon. [arthritisresearchuk.org]
Specific skin manifestations in TA include Raynaud phenomenon, pyoderma gangrenosum, erythema nodosum-like lesions, necrotic or ulcerated nodules, livedo reticularis, and purpura 2, 3, 12. [jrheum.org]
Intermittent attacks of decreased blood flow to the hands and feet (Raynaud’s phenomenon) typically occur in people with Takayasu arteritis. [rarediseases.org]
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]
atherosclerosis.8 Hypertension is a common comorbidity, found in 33–83% of cases,4 linked to renal artery stenosis (observed in 20–38% of patients).6 Although there is disagreement concerning the treatment of these lesions, percutaneous angioplasty has [revportcardiol.org]
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. [symptoma.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]
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]
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 [medlineplus.gov]
Examination demonstrated subclavian bruits, palpable epigastrium aortic pulsation, faint radial pulses, right retinal pallor, arteriolar narrowing, and bilateral boxcarring. [ncbi.nlm.nih.gov]
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]
This can cause painful, cool, or blanched extremities, dizziness, headaches, chest pain, and abdominal pain. Other symptoms can include fatigue, weight loss, and low-grade fever. [medicinenet.com]
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]
[…] claudication of the jaw or extremities, back pain (due to involvement of the aorta), syncope (rare), hypertension (the most common presentation in children). [ 4, 5 ] Neurological - dizziness, headaches, transient ischaemic attacks (TIAs), visual disturbance, seizures [patient.info]
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. [saintlukeskc.org]
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 . 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  . 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.
Elevated Sedimentation Rate
In fact, elevated blood pressure with an elevated sedimentation rate is distinctly uncommon in children and very helpful in suggesting Takayasu disease as a possible cause. Anemia ( low red blood cell count ) is frequent. [medicinenet.com]
Blood work revealed an elevated sedimentation rate (66 mm/hour), normal coagulation profile, and normal complete blood count. Despite supportive medical treatment, the patient's clinical status deteriorated, necessitating intubation. [ajnr.org]
The commonest causes of death are heart failure, myocardial infarction and stroke. 11 In general, steroid therapy is recommended in cases where inflammatory signs such as an elevated sedimentation rate, elevated C-reactive protein and a high white blood [ispub.com]
Treatment is defied by the relapsing nature of the disease and frequent adverse effects of corticosteroids and immunosuppressors, rendering failure of treatment in a significant portion of patients. [ncbi.nlm.nih.gov]
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]
Recent epidemiologic studies suggest that TA is being increasingly recognized in Europe with reported incidence estimates varying from 0.4 to 1.5 per million. [ncbi.nlm.nih.gov]
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]
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]
- 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.