SAT0489 CLINICAL COURSE AND THERAPY RESPONSE IN TAKAYASU ARTERITIS: COMPARISION BETWEEN CHILDHOOD AND ADULT ONSET
Authors: E. Cocchiara, E. Galli, A. De Fanti, C. Malattia, A. Ravelli, C. Salvarani
Journal: Annals of the Rheumatic Diseases (2020)

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 &lt;18 years) and adult-onset (onset ≥ 18 years) TA followed from 2002 to 2019 in two Italian centers (Genoa Gaslini Hospital and Santa Maria Nuova Hospital of Reggio Emilia) were retrospectively evaluated and compared. 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