Childhood-onset Takayasu arteritis -- experience from a tertiary care center in South India.

OBJECTIVE: To study the clinical profile and outcome of Asian Indian children with childhood-onset Takayasu arteritis (c-TA).

METHODS: Records were studied of patients with c-TA onset prior to age 16. Disease Extent Index-Takayasu (DEI.TAK), Indian Takayasu Arteritis Score 2010, and Takayasu Arteritis Damage Score (TADS) were calculated retrospectively from electronic records. Cumulative incidence of sustained remission was estimated using the Kaplan-Meier curve.

RESULTS: There were 40 patients with c-TA, with median age of onset of 12.5 years (range 1-16) and median diagnostic delay of 11.3 months (range 1-60). The most common presenting features were hypertension, headache, malaise, and fever. Pulseless disease was observed in 25 cases (62.5%). The majority (n = 28) had active disease with raised inflammatory markers, high baseline median DEI.TAK score of 10 (range 3-24), and high median TADS of 7 (range 1-14). Of the 34 patients followed for 21.5 months (range 3-192), remission was attained in 30. However, cumulative sustained remission was achieved in only 29% of them at 5 years. Median period of sustained remission was 22.5 months (95% CI 17.1-26.8). New areas of vessel involvement were observed in 13 patients (38%). Disease progression was arrested in the majority (n = 22, 66%) through aggressive medical management and endovascular intervention. All 11 patients with an increment in TADS of ≥ 4 during followup had persistently active or relapsing disease. There was a single fatality.

CONCLUSION: Despite aggressive immunosuppression, damage progressed in one-third of patients with c-TA in association with persistent inflammation, warranting surveillance with clinical instruments and followup imaging.