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      Severe aortic regurgitation complicating Takayasu’s arteritis

      case-report
      , MD, , FACC, FESC, , MD, , MD
      Saudi Medical Journal
      Saudi Medical Journal

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          Abstract

          We present an uncommon case of a 48-year-old female patient with symptomatic presentation of a severe aortic regurgitation with aneurysm of the ascending aorta and progressive dyspnea. Detailed investigation of laboratory tests and imaging identified Takayasu’s arteritis (TA) as the underlying etiology. Computed tomography scan revealed complete occlusion of the right carotid artery as well as stenosis at the origins of left subclavian and vertebral arteries. In addition, cardiac magnetic resonance angiogram showed aneurysm at the proximal segment of right subclavian artery. Intervention with corticosteroids effectively diminished the need for immediate surgical intervention. Treating physicians should always consider differential diagnosis of TA in the presence of atypical clinical findings in all patients with cardiac problems especially when there is valve involvement.

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          Diagnostic and classification criteria of Takayasu arteritis.

          Takayasu arteritis (TA) is a chronic large vessel vasculitis that affects aorta, its main branches and pulmonary arteries. The inflammatory process results in stenosis, occlusion, dilation or aneurysm formation in the arterial wall. TA has been described in different parts of the world and affects predominantly young individuals (<50 years of age). Patients with TA may present constitutional symptoms, vascular pain (e.g. carotidynia) and typical features such as limb claudication, decreased or absent peripheral pulses, vascular bruits, hypertension, and reduction or discrepancies in blood pressure between arms. A proper diagnosis of TA is an important issue since delays may result in significant morbidity. The definition of TA was included in the 1994 and 2012 Chapel Hill Consensus Conference and TA was categorized as a large vessel vasculitis. The first diagnostic criteria for TA were developed by Ishikawa in 1988 and modified by Sharma et al., in 1995. Two sets of classification criteria were developed for TA to include patients in epidemiologic studies and clinical trials: the 1990 ACR Classification Criteria for TA and the Classification Criteria for childhood TA proposed by the European League Against Rheumatism (EULAR), the Pediatric Rheumatology European Society (PRES) and by the Pediatric Rheumatology International Trials Organization (PRINTO) to be used for patients younger than 18 years. The Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) is an international effort that is under way to develop a single classification system and a validated set of diagnostic criteria for systemic vasculitides using data-driven methods.
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            Management of Takayasu arteritis: a systematic review.

            Assessment of the pattern and extent of arterial involvement and measurement of current disease activity are essential for the management of Takayasu arteritis (TA). Since there is no completed, placebo-controlled, randomized clinical trial, the level of evidence for management of TA is low, generally reflecting the results of open studies, case series and expert opinion. The most commonly used agents include corticosteroids and conventional immunosuppressive agents such as MTX, AZA, MMF and LEF. In patients who remain resistant and/or intolerant to these agents, biologic drugs including TNF inhibitors, rituximab and tocilizumab seem to be promising. Antiplatelet treatment may also lower the frequency of ischaemic events in TA. In the presence of short-segment, critical arterial stenosis, balloon angioplasty or stent graft replacement may be useful. On the other hand, long-segment stenosis with extensive periarterial fibrosis or occlusion requires surgical bypass of the affected segment, which is clearly associated with superior results compared with endovascular intervention. As a general rule, both endovascular intervention and surgical procedures should be avoided during the active phase of the disease. Earlier diagnosis, better assessment of disease activity and future clinical trials will obviously improve the management of TA.
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              Takayasu's arteritis.

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                Author and article information

                Journal
                Saudi Med J
                Saudi Med J
                Saudi Medical Journal
                Saudi Medical Journal (Saudi Arabia )
                0379-5284
                August 2017
                : 38
                : 8
                : 863-867
                Affiliations
                [1] From the Department of Internal Medicine (Alali), the Department of Rheumatology (Alrashidi), Prince Sultan Military Medical City, and the Department of Cardiology (Alahmari, Alhebaishi), Prince Sultan Cardiac Center, Riyadh, Kingdom of Saudi Arabia
                Author notes
                Address correspondence and reprint request to: Dr. Wala M. Alali, Department of Internal Medicine, Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia. E-mail: wala.alali@ 123456hotmail.com
                Article
                SaudiMedJ-38-863
                10.15537/smj.2017.8.12473
                5556305
                28762441
                5cc20734-50ff-4c02-8f4c-80b51201b39a
                Copyright: © Saudi Medical Journal

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 March 2017
                : 22 June 2017
                Categories
                Case Report

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