Author: Michael G Rossman, MD, Rheumatology Fellow, Walter Reed Army Medical Center, Washington, DC Coauthor(s): Jefferson R Roberts, MD, Rheumatology Fellow, Walter Reed Army Medical Center, Washington, DC; Mohammed Mubashir Ahmed, MD, Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine; Robert EWolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport Contributor Information and Disclosures
Updated: Feb 8, 2010
Takayasu arteritis is a rare systemic inflammatory large-vessel vasculitis of unknown etiology that most commonly affects women of child-bearing age. It is defined as "granulomatous inflammation of the aorta and its major branches" by the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis.1 Takayasu arteritis commonly occurs in woman younger than 50years and can manifest as isolated, atypical, and/or catastrophic disease.
Dr. Takayasu described the retinal changes of the disease in 1905 at the annual Ophthalmology Society meeting in Japan, and his abstract was subsequently published in 1908. The condition is now called Takayasu arteritis in his honor.2
Keywords: Takayasu arteritis, pulseless disease, Takayasu's disease, Takayasu's arteritis,Takayasu disease, aortic arch syndrome, angioplasty, renovascular stenosis, coronary artery stenosis, extremity claudication, cerebral ischemia, critical stenosis, aortic regurgitation, thoracic aneurysm, abdominal aneurysm.
The pathophysiology of Takayasu arteritis is poorly understood. Cell-mediated autoimmunity appears to play an important role in the mechanism of vascularinjury. Takayasu arteritis is characterized by cellular immune responses involving T cells, antigen-presenting cells, and macrophages, with no evidence of an autoantibody or B-cell component.3
Takayasu arteritis is estimated to affect 2.6 persons per million annually. Any discrepancy in terms of pinpointing the prevalence is attributed to genetic factors and difficultyin diagnosis. Between 1971 and 1983 in Olmstead County, Minnesota, 3 cases were recorded, thus establishing an annual incidence of 2.6 cases per million population.4
Although Takayasu arteritis has a worldwide distribution, it is observed more frequently in Asian countries such as Japan, Korea, China, India, Thailand, Singapore, Israel, and Turkey, as well as Central and SouthAmerica. About 100-200 new cases of Takayasu arteritis are registered each year in Japan.5
Takayasu arteritis is a chronic relapsing and remitting disorder. The overall 10-year survival rate is approximately 90%; however, this rate is reduced in the presence of major complications.6
Complications of Takayasu arteritis include valvular heart disease, stroke, heart failure,retinopathy, and renovascular hypertension. The 5- and 10-year survival rates are approximately 69% and 36%, respectively, in patients with two or more complications. The 5- and 10-year survival rates associated with one or fewer complications are 100% and 96%, respectively.7
A 2008 study assessing quality of life with Takayasu arteritis shows worse scores for physical and mental health compared withmany other chronic diseases associated with peripheral vascular disease. Disease remission is the only factor that positively influences both physical and mental quality of life.8 Patients with rheumatoid arthritis or ankylosing spondylitis rate their quality of life as similar to those with Takayasu arteritis.9
Takayasu arteritis is observed more frequently in patients of Asian or Indian...