Childhood-onset Takayasu Arteritis — Experience from a Tertiary Care Center in South India

Abstract

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.