SAT0489 CLINICAL COURSE AND THERAPY RESPONSE IN TAKAYASU ARTERITIS: COMPARISION BETWEEN CHILDHOOD AND ADULT ONSET

Background: Takayasu arteritis (TA) is a granulomatous vasculitis of the large vessels of unknown origin, which mainly affects the aorta and its main branches. TA is a disease of the young age with onset usually before 40 years and in more than 20% of patients the diagnosis is made before 19 years. Very few clinical series have compared the clinical manifestations, treatment and outcomes between pediatric and adult patients. Objectives: Objective of our study was to compare childhood and adult onset TA evaluating clinical manifestations, treatment (including biotechnological agents) and outcomes. Methods: All consecutive patients with childhood-onset TA (onset All patients met TA classification criteria (ACR 1990 and EULAR/Printo/PrES 2010 for children). Clinical, demographic, laboratory, radiological, therapeutic data were collected retrospectively at baseline, at 6-month follow-up and at the last follow-up. Disease activity at each follow-up visit was evaluated according to NIH criteria. Results: 58 patients were consecutively enrolled: 18 children (C) and 40 adults (A). In both groups there was a higher prevalence of females (83.3% C vs 77.5% A; p NS). The diagnostic delay was lower in the pediatric group (median of 5 months VS 10 months in A; p NS). Fever and headache as presenting manifestations affected more frequently children with statistically significant differences (55,6% C vs 17,5% A, p 0,003; 27,8% C vs 5% A, p 0,025) Adults had a higher frequency of claudication of the upper limbs and carotid/subclavian bruits (30% and 55% A vs 5,6% and 38,9% C respectively; p NS), while the discrepancy in blood pressure of the four limbs was higher in children (22,2% C vs 12,5% A; p NS). Hypertension was not significantly different between the two groups at baseline and during the follow-up. We found a significantly more frequent inflammatory involvement of the aorta (arch, thoracic, or abdominal) in children (72,2% C vs 30% A, p 0.003). The subclavian arteries were most affected in the adults (65% A vs 38,9% C; p NS), although the difference was not statistically significant. Treatment regimen were different between the groups: glucocorticoid monotherapy was more frequently used in adults (45% A vs 27,8% C; p NS), glucocorticoids in combination with Cyclophosphamide or anti-TNF were more significantly used to induce remission at the beginning in the pediatric group (22% C vs 0 A, p 0,007 and 22% C vs 2,5% A, p 0,029). At the last follow-up the disease was significantly more active in children according to NIH criteria (55.6% C vs 27.5% A, p 0,04). Conclusion: We reported some differences between children and adults. TA in children was characterized by a shorter diagnostic delay, a more frequent inflammatory involvement of the aorta and a more refractory disease. Patients with pediatric TA were treated more aggressively at the beginning. Disclosure of Interests: None declared