Severe supraaortal atherosclerotic disease resembling Takayasu’s Arteritis

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Abstract

We report a case of a 64 year-old man whose clinical presentation and neuroimaging findings strikingly resembled those found in Takayasu’s Arteritis which is characterized by the triad of absent radial pulses, ischemic retinopathy, and carotid sinus hyperreflexia causing syncopes. Angiographically, the patient exhibited severe atherosclerotic changes of the supraaortic large vessels. Stent-assisted angioplasty resulted in both clinical improvement and increased cerebral blood flow as measured by angiography and ultrasound.

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Most cited references 20

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Takayasu arteritis.

J. Giordano, M Rottem, G. Kerr … (1994)

To evaluate prospectively the clinical features, angiographic findings, and response to treatment of patients with Takayasu arteritis. 60 patients with Takayasu arteritis were studied at the National Institute of Allergy and Infectious Diseases between 1970 and 1990 and were followed for 6 months to 20 years (median follow-up, 5.3 years). Data on clinical features, angiographic and laboratory findings, disease course, and response to therapy were all recorded and stored in a computer-based retrieval system. The Warren Magnuson Clinical Center of the National Institutes of Health. In our series of patients, Takayasu arteritis was more common in Asian persons compared with persons from other racial groups. Females (97%) were most frequently affected. The median age at disease onset was 25 years. Juveniles had a delay in diagnosis that was about four times that of adults. The clinical presentation ranged from asymptomatic to catastrophic with stroke. The most common clinical finding was a bruit. Hypertension was most often associated with renal artery stenosis. Only 33% of all patients had systemic symptoms on presentation. Sixty-eight percent of patients had extensive vascular disease; stenotic lesions were 3.6-fold more common than were aneurysms (98% compared with 27%). The erythrocyte sedimentation rate was not a consistently reliable surrogate marker of disease activity. Surgical bypass biopsy specimens from clinically inactive patients showed histologically active disease in 44% of patients. Although clinically significant palliation usually occurred after angioplasty or bypass of severely stenotic vessels, restenosis was common. Medical therapy was required for 80% of patients, whereas 20% had monophasic self-limiting disease. Immunosuppressive treatment with glucocorticoids alone or in combination with a cytotoxic agent failed to induce remission in one fourth of patients; about half of those who achieved remission later relapsed. In North America, Takayasu arteritis is a rare disease. It is heterogeneous in presentation, progression, and response to therapy. Current laboratory markers of disease activity are insufficiently reliable to guide management. Most patients require repeated and, at times, prolonged courses of therapy. Although mortality was low, substantial morbidity occurred in most patients.

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Takayasu arteritis: a review.

M Gompels, D R Lock, Emma L. Johnston (2002)

Takayasu arteritis is a well known yet rare form of large vessel vasculitis. This review details the history, clinical features, differential diagnoses, classification, and immunology of the disorder. Suppression of inflammation and preservation of vascular competence are the aims of treatment. As with any rare disease, randomised controlled treatment trials are either lacking or based on small patient numbers, making management decisions difficult. Current evidence based treatments are presented and discussed.

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Inflammation: a pivotal link between autoimmune diseases and atherosclerosis.

Anna Abou-Raya, Suzan Abou-Raya (2006)

Premature coronary heart disease has emerged as a major cause of morbidity and mortality in systemic autoimmune diseases. Recent epidemiologic and pathogenesis studies have suggested a great deal in common between the pathogenesis of prototypic autoimmune disease such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) and that of atherosclerosis. Some of the most remarkable data in support of a link between autoimmunity and atherosclerosis comes from epidemiological studies of patients with autoimmune disorders (RA and SLE). Many epidemiologic observations have linked systemic inflammation with the cardiovascular events in autoimmune disease such as RA and SLE. Inflammation is increasingly being considered central to the pathogenesis of atherosclerosis and an important risk factor for vascular disease. Systemic inflammation may be regarded as accelerating the atherosclerotic process. Systemic levels of soluble inflammatory mediators such as C-reactive protein (CRP) have been associated with cardiovascular risk in the general population. CRP, or more specifically high sensitivity-hsCRP, is a marker of systemic inflammation that has been identified as a valid biomarker of cardiovascular risk. Furthermore, the immunomodulatory and anti-inflammatory actions of statins may affect their utility in the context of chronic inflammatory autoimmune disease. Thus, effective control or dampening of inflammation, with such agents, should be included in the therapeutic armamentarium of autoimmune diseases with the aim of protecting against cardiovascular disease.

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Author and article information

Journal

Journal ID (nlm-ta): Vasc Health Risk Manag

Journal ID (publisher-id): Vascular Health and Risk Management

Title: Vascular Health and Risk Management

Publisher: Dove Medical Press

ISSN (Print): 1176-6344

ISSN (Electronic): 1178-2048

Publication date (Print): June 2007

Publication date (Electronic): June 2007

Volume: 3

Issue: 3

Pages: 351-354

Affiliations

[1 ]simpleDepartment of Neurology, Alfried Krupp Hospital Essen, Germany

[2 ]simpleDepartment of Psychiatry, University of Duisburg-Essen Essen, Germany

[3 ]simpleDepartment of Neuroradiology, Alfried Krupp Hospital Essen, Germany

[4 ]simpleDepartment of Neuroradiology, Technical University of Munich Munich, Germany

Author notes

Correspondence: Bernhard Kis Department of Psychiatry and Psychotherapy, University of Duisburg-Essen, Virchow Street 174, D-45147 Essen, Germany Tel +49 201 7227 120 Fax +49 201 7227 310 Email bernhard.kis@ 123456uni-duisburg-essen.de

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PMC ID: 2293962

PubMed ID: 17703643

SO-VID: c21d7060-b99a-4c87-a369-022bff030b68

Severe supraaortal atherosclerotic disease resembling Takayasu’s Arteritis

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Karger: Cardiovascular System

Most cited references 20

Takayasu arteritis.

Takayasu arteritis: a review.

Inflammation: a pivotal link between autoimmune diseases and atherosclerosis.

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