25. Takayasu's Arteritis
- Gary S. Hoffman MD, MS2,
- Cornelia M. Weyand MD, PhD3,
- Carol A. Langford MD, MHS2 and
- Jörg J. Goronzy MD, PhD3
Published Online: 3 MAY 2012
Copyright © 2012 Blackwell Publishing Ltd
Inflammatory Diseases of Blood Vessels, Second Edition
How to Cite
Maksimowicz-Mckinnon, K. and Hoffman, G. S. (2012) Takayasu's Arteritis, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch25
Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA
Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
- Published Online: 3 MAY 2012
- Published Print: 8 JUN 2012
Print ISBN: 9781444338225
Online ISBN: 9781118355244
- Takayasu arteritis;
- Takayasu's disease;
- pulseless disease;
- large vessel vasculitis;
Takayasu arteritis (TAK) is an idiopathic chronic inflammatory disease affecting the aorta and/or its primary branches. The caprice of medical history and historians has lead to crediting the seminal report of this disease to the ophthalmologist, Mikito Takayasu. Indeed, Takayasu's case in 1905 did not recognize large vessel disease at all. It recounted the story of a 21-year-old woman who had acute blindness associated with a peculiar anastomotic wreath of vessels surrounding the optic disc. Earlier clinical and post-mortem reports by Morgagni in 1761 and Savory in 1856 described an inflammatory disease of large vessels. Savory's patient also had monocular blindness. The rarity of this illness has lead to other misconceptions that have compromised both the diagnostic process and treatment of TAK. The following critical review will attempt to distinguish observations that are generally reliable from those that are dubious and those for which answers are not available.
TAK has a definite female gender bias. However, it has been recognized in both men and women worldwide. Patients may initially present with acute constitutional symptoms or after months or years of undetected disease when only features of arterial insufficiency are apparent. In the great majority of treated patients, TAK is a remitting/relapsing disease. Permanent treatment-induced or spontaneous remissions are infrequent. High dose glucocorticoid therapy usually leads to disease remission. However, in most patients, dose reduction leading to glucocorticoid-free remissions is difficult to achieve. Preliminary data with certain biologic agents suggests this goal may be achieved in the future.
Surgical techniques to restore arterial patency have a high rate of immediate success, but long-term patency is not assured, especially for angioplasty and vascular stents. Chronic morbidity and disability frequently occur in a population who are often affected in the prime of their lives.