PP17. TAKAYASU'S ARTERITIS (TA): DISEASE CHARACTERISTICS AND OUTCOMES IN A COHORT OF 75 PATIENTS

Abstract

Background: Large series of patients with TA are uncommon in the United States, where the incidence has been reported as 2.6 cases/million yearly. Our purpose was to provide clinical data from our cohort in regard to disease presentation, clinical characteristics, and outcomes. Methods: Retrospective review of 75 patients meeting ACR criteria for TA. Signs, symptoms, laboratory, imaging data and co-morbidities were recorded for each patient at disease onset. The data were tabulated using a spreadsheet program and were descriptively analyzed. All cases were evaluated by a single physician (GSH). Additional data was collected on a subset of patients that received continued care at our center for at least 1 year. Response to treatment was considered successful if signs or symptoms of disease activity were absent, laboratory studies were normal, and new vascular abnormalities were not identified on sequential imaging studies over an interval of at least 6 months. Remission was judged to be present if these guidelines were met and the patient required <10 mg prednisone/day. Results: 91% of TA patients were female, 88% were Caucasian, and mean age was 26 years. The most common signs and symptoms of disease included absent pulse (57%), BP asymmetry (55%), headache (53%), limb claudication (52%), fever (35%), hypertension (28%), arthralgias with myalgias (13%). The most common vessels involved were subclavian (65%), carotid (44%), mesenteric (32%), renal (25%), and femoral (28%) arteries. 16% of patients had a monophasic course, requiring no sustained therapy. In the 30 patients who received follow up care at our center, relapses were frequent, occurring on average 2.9 times in each patient over a median period of 2 years. 19/30 patients required vascular interventions. 8/30 became disabled. There were 2 deaths in this group. Conclusions: Our experience represents the largest cohort of TA patients in the US. There clinical characteristics are similar to those published from the NIH cohort in regards to signs, symptoms, and lesion distribution. Although most patients achieved remission, 70% required agents other than prednisone for maintenance therapy. Of all patients who received therapy, 30% required more than 2 immunosuppressive agents in addition to corticosteroids. The number of patients that achieved long term remission is low. Of the group followed at our center for at least 2 years (median 3.75 years), treatment resulted in disease control in 16/18 patients, but only 2 achieved steroid-free remission. Data from a recently published open label study suggests that anti-TNF agents may improve outcomes in TA. Despite continued advances in medical and surgical interventions, significant morbidity from TA and its therapy remains high.