@article {Goeljrheum.131117, author = {Ruchika Goel and T. Sathish Kumar and Debashish Danda and George Joseph and Visali Jeyaseelan and Ajith Kumar Surin and Paul Bacon}, title = {Childhood-onset Takayasu Arteritis {\textemdash} Experience from a Tertiary Care Center in South India}, elocation-id = {jrheum.131117}, year = {2014}, doi = {10.3899/jrheum.131117}, publisher = {The Journal of Rheumatology}, 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{\textendash}16) and median diagnostic delay of 11.3 months (range 1{\textendash}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{\textendash}24), and high median TADS of 7 (range 1{\textendash}14). Of the 34 patients followed for 21.5 months (range 3{\textendash}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{\textendash}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.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/early/2014/04/28/jrheum.131117}, eprint = {https://www.jrheum.org/content/early/2014/04/28/jrheum.131117.full.pdf}, journal = {The Journal of Rheumatology} }