Ab1310 Poor Prognostic Factors of Takayasu Arteritis, Cerebral Infarction, Coronary Artery Lesions, or Aortic Regurgitation Are Associated With Mortality

Background: Takayasu's arteritis (TA) is a chronic granulomatous vasculitis that predominantly affects the aorta and its major branches, resulting in narrowing, occlusion, dilation, and aneurysm formation in the arterial lumen 1. Objectives: TA causes symptoms of impaired blood flow in the extremities and organs, but the long-term prognosis and prognostic factors are unknown. Methods: We retrospectively analyzed the clinical data of 70 patients with TA treated at our hospital. All cases met the ACR diagnostic criteria for TA 2. Relapse was defined as a case that met Kerr's criteria and changed treatment for TA 3. Results: The male to female ratio was 1:4.4, the average age of onset was 31.2 ± 11.6 (mean ± SD) years, and 2.6 ± 5.1 years passed from the onset of TA to diagnosis. The observation period was 18.3 ± 12.5 years. Symptoms at diagnosis included systemic symptoms such as fever and malaise (51 %), local symptoms such as vascular pain (43.1 %), as well as ischemic symptoms (29.4 %), aortic regurgetation (AR) (27.1 %), and signs of heart failure (13.7 %). Most cases had some subjective symptoms. (84.3 %), but there were also cases with no subjective symptoms (15.7 %). Asymptomatic cases were diagnosed based on pulse differences, pulse defects, vascular murmurs, heart murmurs, and hypertension. At the time of diagnosis, the most common laboratory test abnormalities were increases in CRP and ESR, but both CRP and ESR were negative in 11.1% of cases. The most commonly affected areas were the common carotid artery (76.1 %) and left subclavian artery (56.7 %). As remission induction therapy, the initial dose of prednisolone was 37.5 ± 11.3 mg, for maintenance therapy, the prednisolone dose was 2.48 ± 2.62 mg. The concomitant use rate of immunosuppressants were 56.9%, and the rate of concomitant use of biological agents were 28.3%. TNF inhibitors were used in 10 patients and discontinued in 6 patients. IL-6 inhibitors were used in 7 patients and discontinued in 2 patients. There was a tendency for TNF inhibitors to be discontinued more frequently due to insufficient efficacy than IL-6 inhibitors (p = 0.147). Antiplatelet drugs were used in 72.1% and anticoagulant therapy in 30.9%. During the observation period, 14 patients experienced 27 relapses, of which 39.1% relapsed within 1 year after induction therapy, with some cases having repeated relapses and some cases relapsing early after induction therapy. In addition, new vascular stenosis requiring major reconstruction surgery was observed in 7 cases with negative CRP and ESR. Although the frequency of new vascular stenosis was low after immunosuppressive therapy, the incidence of stenosis was higher after completion of treatment and in patients who were considered to have low disease activity and were being followed up without treatment. Among cases with severe AR, 72.2% required AVR or Bentall, and surgery was often unavoidable. There were 5 cases of AR appearing after treatment, and it is necessary to keep an eye on the appearance of AR in cases with inflammation in the ascending aorta. Analysis of long-term life prognosis revealed the 10-year survival rate was 96% and the 20-year survival rate was 85.2%, but 9 patients died an average of 25.4 years after diagnosis, and most couse of the deaths were due to infections and cardiovascular complications. In multivariate analysis, cerebral infarction (p = 0.0097), severe AR (p = 0.0085), and coronary artery lesions (p = 0.0207) were associated with mortality. Patients with these severe conditions had a worse life prognosis than those without these conditions (p Conclusion: It is difficult to evaluate the disease activity of TA, and even if CRP and ESR are negative, inflammation remains in the blood vessels, leading to vascular stenosis and aneurysm formation in some cases. Patients with cerebral infarction, coronary artery lesions, heart failure, or severe AR had poor prognosis. In cases with poor prognostic factors, prompt control of disease activity and surgery are both important. REFERENCES: 1 Arthritis Rheum. 2013 Jan; 65(1):1-11. 2 Arthritis Rheum. 1990 Aug; 33(8): 1065-7. 3 Ann. Intern. Med. 1994, 120, 919–929. Acknowledgements: NIL. Disclosure of Interests: None declared.