Ab1300 Age at Disease Onset Influences the Clinical Phenotype and Outcome of Patients With Takayasu Arteritis: Results From a Monocentric Cohort of 167 Patients

Background: Takayasu's arteritis (TAK) is a large-vessel vasculitis that typically affects people between the ages of 20 and 40. Nevertheless, disease onset can span across different age periods 1. Data on how age at onset affects disease characteristics and outcomes of TAK are limited. Objectives: To evaluate the influence of age at disease onset on baseline clinical features and vascular involvement and on treatment strategies in patients with TAK. Methods: The medical records of patients with a clinical diagnosis of TAK and followed up at our Institution were retrospectively reviewed. Age at disease onset was defined as the age at which the first sign/symptom subsequently associated with TAK appeared. Patients were first divided into 3 groups according to age at TAK onset (A1, ≤ 20; A2, > 20 and ≤ 40; B > 40 and ≤ 60 years), and then into 2 groups (A ≤ 40; B > 40 and ≤ 60 years). Diagnostic delay, fulfillment of the EULAR/ACR 2022 TAK classification criteria, signs/symptoms and vessels involved at baseline were compared. In those with a follow-up ≥ 12 months, treatment strategies (i.e., glucocorticoid GC suspension and introduction of biologic disease-modifying agents bDMARDs) were evaluated at 12 and 60 months after diagnosis. Continuous variables were compared with the Mann-Whitney U or Kruskal-Wallis tests and categorical variables with the Chi-squared test. For the survival analyses, the Kaplan-Meier estimator and the log-rank test were employed. A two-sided pResults: 167 patients (A1, n=41 24%; A2, n=83 50%; A, n=124 75%; B, n=43 26%) were included. Female sex was the most represented in all age groups (A, 91%; A1, 83%; A2, 94%; B, 84%), with no significant differences. In younger patients there was a tendency to a longer diagnostic delay, but with no statistical significance (A1, 19 3-48; A2, 15 2-60; A, 17 2-53; B, 6 1-40 months). The mean EULAR/ACR 2022 TAK classification criteria score was significantly higher in younger patients (A1, 9.8 ± 2.6; A2, 9.7 ± 3; B, 8.2 ± 2.9; p=0.0165; A, 9.7 ± 2.8; B, 8.2 ± 2.9; p=0.0050); however, the % of patients meeting the criteria was comparable between the groups (A, 96%; A1, 98%; A2, 95%; B, 90%). Syncope and upper limbs claudication were more frequent in group A compared to group B (13% vs 0%, p=0.0132 and 40% vs 19%, p=0.0100, respectively). This difference was significant also when comparing the 3 groups (A1 22%, A2 8%, B 0%, p=0.0026 and A1 37%, A2 42%, B 19%, p=0.0299, respectively). Patients in group A also had more frequently pulse loss/reduction in upper extremities and vascular bruits when compared to patients in group B (64% vs 37%, p=0.0025 and 73% vs 51%, p=0.0100, respectively). These differences were significant also when comparing the 3 groups (A1 68%, A2 61%, B2 37%, p=0.0080 and A1 73%, A2 73%, B 51%, p=0.0361, respectively). Patients in group B had more frequently involvement of coronary arteries (A 6%, B 24%, p=0.0018; A1 2%, A2 8%, B 24%, p=0.0045), abdominal aorta (A 44%, B 62%, p=0.0492), and iliac arteries (A 14%, B 30%, p=0.0150). Outcome data were available for 124 patients (A1, n=25 20%; A2, n=67 54%; A, n=92 74%; B, n=32 26%). Age > 40 years was associated with GC suspension at 60 months (Figure 1A and B); only 1 patient stopped GC during the first 12 months, so the analysis was not performed. No age group was associated with bDMARD introduction at 12 and 60 months; however, a tendency to a higher bDMARD introduction at 12 months was found (Figures 2A–D). Conclusion: In our cohort, in a quarter of patients with TAK, disease onset was > 40 years, thus highlighting the importance of maintaining a high level of suspicion for TAK even in this age group. Patients with TAK onset > 40 years seem to have a peculiar presentation both clinical, with reduced frequency of classic symptoms (bruits, pulse loss, limbs claudication), and in terms of vascular involvement (high frequency of coronary vasculitis). Moreover, older patients with TAK seem to have a less aggressive disease course and a reduced need for long-term GC therapy. REFERENCES: 1 Watanabe Y., et al. Circulation. 2015;132:1701–1709. Acknowledgements: NIL. Disclosure of Interests: Alessandro Tomelleri Novartis, Corrado Campochiaro: None declared, Elena Baldissera: None declared, Naomi Viapiana: None declared, Marco Matucci-Cerinic Boehringer Ingelheim, Biogen, Lilly, MSD, Lorenzo Dagna: None declared.