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Takayasu Arteritis

Takayasu's Syndrome

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.

Anemia
  • Correction of anemia improves oxygen delivery to the hypoxic retina and relieves recurrent amaurosis.[ncbi.nlm.nih.gov]
  • Because of the occurrence of anemia and exacerbation of rectocolitis, she had started infliximab associated with low doses of cortisone and mesalazine.[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]
  • 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]
Hodgkin's Disease
  • Hodgkin's disease . Polyarteritis nodosa . Rheumatic fever . Giant cell arteritis . Buerger's disease . Systemic lupus erythematosus . Fibromuscular dysplasia. Systemic-onset juvenile idiopathic arthritis . Migraine . Infection. Malignancy.[patient.info]
  • Hodgkin's disease. Polyarteritis nodosa. Rheumatic fever. Giant cell arteritis. Buerger's disease. Systemic lupus erythematosus. Fibromuscular dysplasia. Systemic-onset juvenile idiopathic arthritis. Migraine. Infection. Malignancy.[patient.info]
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]
Epilepsy
  • At the end of follow-up, 59% of patients had a neurological impairment, 35% had a recurrence of stroke, and 24% suffered from epilepsy. CONCLUSIONS: Stroke is a major cause of disability in TA patients.[ncbi.nlm.nih.gov]
Chest Pain
  • 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]
  • With coronary artery involvement, manifestations range from chest pain and shortness of breath to sudden death. We report a case of a 15-year-old white girl who presented with syncope immediately before passing.[ncbi.nlm.nih.gov]
  • We report a case of a 21-year-old female who presented with acute chest pain with an isolated descending thoracic AMT on imaging.[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]
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]
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.[saintlukeskc.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.

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

  • 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]
  • In this study, the primary and secondary treatment outcomes were found to be steadily attenuated in patients who received curcumin treatment, while showed no significant change in patients in placebo group.[ncbi.nlm.nih.gov]
  • We aimed to explore the anti-TNF function of resveratrol, a natural compound, in the treatment of TA.[ncbi.nlm.nih.gov]
  • Treatment regimens included corticosteroids alone (15%), corticosteroids plus methotrexate (37%), cyclophosphamide (19%), or a biologic agent (11%).[ncbi.nlm.nih.gov]
  • When sarcoidosis preceded TA, a recovery of sarcoidosis was achieved mostly without treatment. TA is a prognostic and therapeutic factor.[ncbi.nlm.nih.gov]

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]
  • For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides.[ncbi.nlm.nih.gov]
  • The impact of stroke on TA prognosis has not been well evaluated.[ncbi.nlm.nih.gov]
  • The degree of arterial damage closely aligns with prognosis and therefore its prevention is the predominant aim of therapy.[ncbi.nlm.nih.gov]
  • Immunosuppressive treatment, including steroids, led to a good prognosis for TA as well as for sarcoidosis. 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.[ncbi.nlm.nih.gov]

Etiology

  • Takayasu arteritis is a panarteritis of unknown etiology involving the aorta and its main branches, with higher prevalence in women and peak of incidence in the second and third decades of life.[ncbi.nlm.nih.gov]
  • 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]
  • The etiology remains unclear and its presentation depends on the affected arteries. With coronary artery involvement, manifestations range from chest pain and shortness of breath to sudden death.[ncbi.nlm.nih.gov]
  • Takayasu's arteritis is known to cover the youngest age group in the etiology of secondary hypertension. The type 3 pattern is rare and involves the thoraco-abdominal aorta and its main abdominal branches.[ncbi.nlm.nih.gov]
  • Diagnosis was elusive after an exhaustive, multidisciplinary evaluation including structural, hypercoagulable, and rheumatologic etiologies. After hypertension control and anticoagulation, she was asymptomatic without embolic sequelae.[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • BACKGROUND: Studies investigating the epidemiology and mortality of Takayasu Arteritis (TA) are scarce.[ncbi.nlm.nih.gov]
  • With Australian data lacking, we describe the epidemiology, clinical features and outcomes of TAK in Western Australia (WA).[ncbi.nlm.nih.gov]
  • KEYWORDS: Epidemiology; Mortality; Takayasu arteritis; Vascular surgery; Vasculitis[ncbi.nlm.nih.gov]
  • Epidemiology In children, the mean age of onset is 12 years. The female:male ratio is about 2:1. There are geographic variations in the presentation of TA.[link.springer.com]
Sex distribution
Age distribution

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]
  • Although the ante-mortem diagnosis was angio-Behçet disease, its pathophysiology along with her clinical history, morphology of the lead pipe-like aorta, predominant destruction of the outer arteries, and a human leukocyte antigen (HLA) haplotype of B39[ncbi.nlm.nih.gov]
  • These enhanced cytokines might play roles in the pathophysiology of TAK.[ncbi.nlm.nih.gov]
  • Our findings indicate novel genetic susceptibility loci for Takayasu arteritis and uncover potentially important aspects of the pathophysiology of this form of vasculitis.[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]
  • Author information 1 Unit of Advanced Preventive Medical Sciences, Departments of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. 2 Unit of Advanced Preventive Medical[ncbi.nlm.nih.gov]
  • The quality of these studies was considered low, preventing recommendations on effectiveness and safety of the studied interventions for clinical practice.[ncbi.nlm.nih.gov]
  • In this article, we present the case of a patient with heart failure with biological aortic valve prosthesis and multiple vascular changes consistent with Takayasu arteritis (TA) who was seen in our department receiving corticosteroids and secondary prevention[ncbi.nlm.nih.gov]
  • To prevent intraoperative complications, we conducted intubation with a rigid video laryngoscope with careful consideration of the concentrations of analgesics and sedatives used.[ncbi.nlm.nih.gov]

References

Article

  1. American College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology. Systemic Vasculitis. 2009; 65–67.
  2. Shikino Kiyoshi, Takako Masuyama, Masatomi Ikusaka. FDG-PET of Takayasu Arteritis. JGIM 2014 Jul; 29(7): 1072–1073.
  3. John Barone, M.D. USMLE Step 1 Lecture Notes. Vascular Pathology. 2008; Kaplan Inc.101.
  4. 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.
  5. 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.
  6. Magalhães P, Morais A, Carvalho S, et al. Chest Pain: The Need to Consider Less Frequent Diagnosis. Case Rep Cardiol. 2016; 2016:4294780.
  7. Andrews J, Mason JC. Takayasu's arteritis--recent advances in imaging offer promise. Rheumatology (Oxford). 2007 Jan.;46(1):6-15.
  8. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994 Jun 1;120(11):919-29.
  9. 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.

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Last updated: 2019-07-11 21:27