Vasculitis
Abstract 1168: Takayasu Arteritis: Guarded outcomes in an American Cohort
Authors: Kathleen McKinnon, Tiffany M. Clark, Gary S. Hoffman
Background: Takayasu’s arteritis is a large vessel vasculitis that predominantly affects the aorta and the great vessels. Unlike most forms of primary systemic vasculitis, Takayasu’s arteritis is predominantly a disease of young women. Although methotrexate is a standard therapy for this diagnosis, in many cases it is not enough, and glucocorticoids are required to achieve disease remission. Because Takayasu’s arteritis is an uncommon diagnosis, it is not clear if the presentation and outcome of patients in the United States is similar to that of patients in other parts of the world. This study reports the experience of a single center with regards to the clinical manifestations and outcomes associated with this disease.
Study Design: Retrospective analysis of patients with Takayasu’s arteritis seen at the Cleveland Clinic.
Results: The majority (92%) of patients in the Cleveland Clinic cohort were Caucasian; 89% were female. The median age of onset was 26 years, and patients were followed for a median duration of 3 years. Over half of patients had loss of peripheral pulse, asymmetric blood pressures, or bruits, but 11% of patients were asymptomatic at the time of diagnosis. Angiographic studies demonstrated aortic abnormalities in 79% of patients, subclavian abnormalities in 65% of patients, and carotid abnormalities in 43% of patients. Seventy-three percent of patients required immunosuppressive agents in addition to prednisone to achieve and maintain remission, and 7% had active disease despite immunosuppressive therapy. Most patients (93%) achieved remission at some point, but only 28% achieved a sustained remission (defined as no disease activity for 6 months or longer on less than 10 mg of prednisone daily), and 96% of patients had a disease relapse following remission. Disease activity correlated with serologic acute phase response in 70% of patients. Among patients who underwent revascularization, restenosis occurred in 78% of patients who underwent angioplasty, and in 36% of patients who underwent surgical bypass procedures.
Editorial Comments: Many assume that Takayasu’s arteritis is predominantly a disease of Asian women. This is clearly not the case, and it is important to recognize that in the United States, it is predominantly a disease of Caucasian women. Revascularization is a particular problem for this patient population, since rates of restenosis are high. The so-called drug-eluting stents might provide an effective, minimally invasive therapy, but are not widely available, and recent cardiovascular literature indicates that these stents are not without potential complications of their own. This study unfortunately raises more questions than answers, and is further complicated by the fact that these patients are young, so interventions must take place with an eye towards their outcomes over decades rather than years.