A Case of Typical Cogan's Syndrome with Aortitis

Hye Won Kim; Yun Jong Lee; Sung Hae Chang; Han Hee Ryu; Chan Young Yun; Eun Ha Kang; Eun Bong Lee; Yeong Wook Song

doi:10.4078/jkra.2010.17.3.301

Journal List > J Korean Rheum Assoc > v.17(3) > 1003742

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Kim, Lee, Chang, Ryu, Yun, Kang, Lee, and Song: A Case of Typical Cogan's Syndrome with Aortitis

Original Article

J Korean Rheum Assoc 2010;17(3):301-305.

Published online: 23 September 2010

DOI: https://doi.org/10.4078/jkra.2010.17.3.301

A Case of Typical Cogan's Syndrome with Aortitis

Hye Won Kim¹, Yun Jong Lee², Sung Hae Chang³, Han Hee Ryu³, Chan Young Yun², Eun Ha Kang², Eun Bong Lee¹, Yeong Wook Song¹

¹Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Seoul National University College of Medicine, Seoul, Korea

²Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Seoul National University College of Medicine, Seoul, Korea

³Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea

Received 5 April 201018 May 2010 Accepted 19 May 2010

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Cogan's syndrome can accompany a variety of systemic vasculitides including aortitis. A 45-year-old woman with a history of typical Cogan's syndrome presented with orthopnea and exertional dyspnea. Echocardiography demonstrated severe aortic valve insufficiency. Computed tomographic angiography demonstrated active vasculitis affecting the ascending and descending aortas and also showed stenosis of the left subclavian artery, both renal arteries, the celiac axis, the superior mesenteric artery, and the right common iliac artery. She received high dose corticosteroid and then underwent an aortic valve replacement. This is the first case of Cogan's syndrome with aortitis in Korea.

Keywords: Cogan's syndrome, Aortitis, Arthritis

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Fig. 1.

Initial axial views of computed tomographic angiography revealed a thickened vascular wall with delayed enhancement in the right carotid artery, aortic arch, and descending aorta (A). The 3-D reconstruction images showed focal or diffuse stenotic lesions of the major branches of the aortic arch and abdominal aorta (B). Black thick arrow, the left common carotid artery; black thin arrow, the right innominate artery and common carotid artery; white thick arrow, the left subclavian artery; white thin arrow, the right common iliac artery.

Fig. 2.

The time-course of CRP and ESR levels during the immunosuppressive treatment of the patient. The dosage of azathioprine was increased to 150 mg/day, that of cyclosporine to 100 mg/day, and that of methotrexate to 17.5 mg/week. black circles (), CRP; white diamonds (), ESR.

Fig. 3.

Computed tomographic angiography taken after 21 months still revealed active aortitis.

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