Background: Childhood-onset Takayasu Arteritis (c-TAK) may differ from adult-onset Takayasu Arteritis (a-TAK) in clinical maaifestations and treatment.
Objectives: To compare c-TAK with a-TAK patients for vascular involvement, disease activity, damage, and treatment.
Methods: Patient charts from two tertiary-care centers of a pediatric and adult clinic were reviewed. Adult patients diagnosed before the age of 18 were included in the c-TAK group. The activity was assessed with the physician’s global assessment (PGA) and Indian Takayasu Clinical Activity Score (ITAS). The damage was evaluated with Takayasu Arteritis Damage Score (TADS) and Vasculitis Damage Index (VDI).
Results: Twenty four c-TAK and 121 a-TAK patients were compared. 21 (88%) of the c-TAK group and 104 (89%) of the a-TAK group were female. Age at symptom onset was 14 (IQR: 9-15) for c-TAK and 30 (IQR: 24-43) for a-TAK patients. Diagnostic delay in months was shorter for c-TAK patients [c-TAK: 3 (1-10) vs. a-TAK: 12 (5-58)]. Follow-up duration was similar [53 months (IQR: 16-131) vs. 68 (IQR: 30-102), p=0.763].
ITAS was comparable for c-TAK and a-TAK patients on the first visit [14 (SD: 7) vs. 13 (SD: 5), p=0.362, respectively]. However, the PGA score was higher in the c-TAK group compared to the a-TAK group [9 (IQR 7-10) vs. 7 (IQR 6-8), p<0.001].
14 (64%) of c-TAK patients and 10 (9%) of a-TAK patients received pulse glucocorticoids, p= 0.002. Cumulative glucocorticoid dose was 10 grams (IQR: 6-13) for c-TAK patients and 7 grams (IQR: 4-12) for a-TAK patients (p=0.128).
After diagnosis, children had more vascular interventions than the adults did [9 (38%) vs. 20 (18%), p=0.031, respectively].
Rates of achieving at least one remission were lower for c-TAK patients [c-TAK: 12 (50 %) vs. a-TAK: 94 (82%), p=0.001]. c-TAK patients had a PGA score of 6 (IQR 3-8), the PGA score in a-TAK patients was 1 (IQR 1-3), p<0.001. Still, ITAS was similar for both groups [c-TAK: 1 (IQR 0-3) vs. a-TAK: 0 (IQR 0-2), p= 0.579]. 9 (38%) of c-TAK patients had at least one relapse, and the 43 (38%) of a-TAK patients had at least one relapse (p=0.960).
TADS was similar [c-TAK: 8 (IQR 4-12), a-TAK: 8 (IQR 6-10), p=0.919]. However, VDI of the a-TAK patients was higher than the c-TAK patients [c-TAK: 4 (IQR 2-5), a-TAK: 5 (IQR 3-7), p=0.017]. Glucocorticoid related damage was higher in a-TAK patients (Diabetes: 8% vs. 4%, avascular necrosis: 6% vs. 0, and cataracts: 11% vs. 0)
Conclusion: Aorta involvement, biologic agent use, and vascular interventions were more common in c-TAK patients. However, cumulative damage was not increased for c-TAK patients which may be partly explained by more common corticosteroid related side-effects in adults.