Background Disease activity assessment in childhood Takayasu Arteritis (cTA) is an ongoing issue. Recently a new index of disease activity Pediatric Vasculitis Assessment Score (PVAS) was validated for children.
Objectives Our aim was to compare disease activity scoring utilizing the PVAS, to imaging features indicating ongoing inflammation in our small cohort of pediatric Takayasu arteritis patients, at onset and at follow up.
Methods Case notes of children fulfilling the EULAR vasculitis classification criteria for cTA were assessed from the initial rheumatology consultation and the visit corresponding to the last MRA. PVAS scores were calculated. MRA reports were assessed for imaging features indicating disease activity i.e. wall contrast enhancement and irregularity. Descriptive statistics (median, interquartile range (IQR) & percentiles) were used to describe the data.
Results Patients #1 & 2 presented with acute stroke and Patients #3 & 4 with hypertension. Patient#5 was referred 20 months after diagnosis but had never received immunosuppression. Median age of onset was 11 years (IQR 7.8-11.9) and median time for follow up was 13 months (IQR 8-15). 80% had MRA progression with further vessel involvement and continued wall enhancement. 40% of patients initially only had branch involvement +/- renal artery stenosis but later developed aortic involvement. ESR was elevated on follow up in 40%.
Conclusions In this small cohort of cTA patients, follow up PVAS did not reflect MRA findings of continued disease activity in 4 out of 5 patients. Further studies in larger cohorts are necessary to further characterize this issue and this is ongoing in our region.
References Dolezalova P, Price-Kuehne FE, Özen S et al.: Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). Ann Rheum Dis 2012;00:1–6.
Andrews J, Mason JC et al.: Takayasu’s arteritis—recent advances in imaging offer promise. Rheumatology 2007;46:6–15
Disclosure of Interest None Declared