THU0552 Articular and extra-articular damage in patients with juvenile idiopathic arthritis – results from the inception cohort of newly diagnosed patients with juvenile idiopathic arthritis (ICON)
Authors: J. Klotsche, C. Sengler, M. Niewerth, J. Kuemmerle-Deschner, F. Weller, T. Hospach, G. Ganser, I. Foeldvari, K. Minden
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Juvenile idiopathic arthritis (JIA) may lead to disability and damage, preventing both is an important therapeutic goal. The frequency of damage in children and adolescents with JIA and the question of whether the damage increases with the duration of the disease also in the biologic treatment era have hardly been investigated. <h3>Objectives</h3> To assess the prevalence and accrual of damage in patients with JIA over six years and to analyse damage association with disease activity, quality of life and functional limitations. <h3>Methods</h3> We analysed data of patients with JIA who were enrolled in ICON. Clinical characteristics such as disease activity (e.g. JIA core set criteria) and details on current treatment as well as patient's quality of life (PedsQL) and functional limitations (CHAQ) were assessed quarterly during the first 12 months in ICON and half-yearly thereafter. The Juvenile Arthritis Damage Index (JADI, range 0–89, best=0) was reported by the physician at the 3 year, 4 year and 6-year-FU, respectively. The JADI is composed of two sub-scores, the JADI-A scores joint damage (in 36 joints or joint groups) and the JADI-E extraarticular damage (in 5 different organs/systems: ocular, musculoskeletal excluding joints, cutaneous, endocrine, any organ/system). <h3>Results</h3> A total of 953 patients (67.2% female, mean age 7.9 years (SD 4.8)) with JIA were included in ICON after a median disease duration of 6 months (IQR: 3.0–11.1). About half of the patients (46%) had oligoarthritis, followed by rheumatoid factor-negative polyarthritis (RF-PA) (26%) and enthesitis-related arthritis (11%). The mean disease activity score cJADAS10 was 9.8 (6.2) and the mean CHAQ was 0.57 (0.69) at enrolment. Any damage was reported for 58 patients (8.6%) at the 3-year-Follow-up (FU) (mean JADI-A 0.17, mean JADI-E 0.06, JADI-A &gt;0: 6.1%, JADI-E &gt;0: 3.1%). At the 4 year (mean JADI-A 0.17, mean JADI-E 0.07) and 6-year-FU (mean JADI-A 0.13, mean JADI-E 0.12), 8.6% and 10.7% of patients had any damage. The number of patients with articular damage did not change during FU (6-year-FU: 6.5%), whereas the proportion of patients with extra-articular damage slightly increased (6-year-FU: 5.0%). At the 6-year-FU, the most frequently scored joints were the knee joints, followed by the wrist. JADI-E was dominated by eye damage. Among the JIA categories, patients with RF+PA showed most frequently damage (16.7%), followed by patients with enthesitis-related arthritis (15.4%) and extended oligoarthritis (14.3%) at the 6 year FU. The JADI-A score significantly correlated (r=0.27, p&lt;0.001) with the number of active joints and JADI-E with the cJADAS10 (r=0.14, p=0.041) However, there was no significant association between the JADI scores and quality of life (PedsQL), and functional limitations (CHAQ, all r&lt;0.05). <h3>Conclusions</h3> About one in ten patients with JIA has developed any damage three, four and six years after disease onset. Thus, a relevant increase in damage over time does not occur under current therapeutic conditions. Articular and extra-articular damage is similarly frequent. <h3>Acknowledgements</h3> ICON is funded by the Federal Ministry of Research (FKZ: 01ER0812) <h3>Disclosure of Interest</h3> None declared