P079 Early vascular changes in a paediatric connective tissue disease cohort - results from the Lupus Extended Autoimmune Phenotype Study

Abstract Background/Aims The Lupus Extended Autoimmune Phenotype (LEAP) cohort is a multi-centre, prospective study in the UK focusing on patients with various connective tissue diseases (CTDs), including Systemic Lupus Erythematosus (SLE). Adult patients with lupus have a 7-10-fold increased risk of developing cardiovascular disease (CVD). This is not yet quantified in paediatric patients. Pulse wave velocity (PWV) and aortic stiffness index are simple, non-invasive tools for the diagnosis and risk stratification of CVD. Methods Participants from a tertiary paediatric centre were enrolled, including patients with CTD, and age-sex-matched controls from physiotherapy clinics. Patients had aortic femoral PWV and aortic stiffness index measured. Sociodemographic data, symptomatology, antibody profiles, treatment data were described (table 1). Results 24 paediatric LEAP patients (20 with SLE, 2 with DM, 2 with uCTD) and 9 healthy controls were studied. Median age of the disease group was 14 years old with female predominance (79%) and 46% (n = 11) were White. The median disease duration was 2.66 years. 87% (n = 22) of patients were ANA positive at time of data collection, 62% (n = 15) were dsDNA positive. ENAs (anti-RO, anti-La, chromatin, anti-Scl 70, RNP) were positive in more than a third of the patients’ group. 33% had a positive lupus anticoagulant. Constitutional symptoms were the commonest clinical features. Nearly half of the CTD patients have haematological abnormalities. A third of the cohort has organ specific involvement. At the time of recruitment, 91% (n = 22) were on DMARDs and 25% (n = 6) were on biologics. 45% (n = 11) had steroids, with high steroid burden. PWV was 6.7, compared to 6.5 in controls. Their aortic stiffness index was 7.9, and 3.85 in the control group respectively. Conclusion We describe a cohort ofpaediatric CTDs including which have moderate to severe disease with highantibody profiles and organ manifestations. There was no difference between PWVin the patient versus control groups. The aortic stiffness index was significantlyhigher in the patient group (p-value= 0.031) than the control group, which may beindicative of early atherosclerosis change in the early paediatric CTDs.Additional multi-centre studies are required to confirm and expand upon thesefindings regarding early cardiovascular risk in paediatric CTDs. Disclosure C. Ciuculete: None. S. Dyball: None. S. Govindarajan: None. B. Parker: None. I.N. Bruce: None. A. Chieng: None.