Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre

Abstract

BackgroundChildhood-onset spondyloarthropathies usually start with enthesitis and peripheral arthritis. However, axial disease may develop afterward. Patients are most often classified, following revised (Edmonton 2011) ILAR criteria, as enthesitis-related arthritis, psoriatic arthritis, or unclassified juvenile idiopathic arthritis, particularly in cases of psoriasis in the patient or a first-degree relative. In adults, peripheral spondyloarthritis is classified by ASAS criteria.MethodsWe retrospectively studied patients with childhood-onset spondyloarthropathies followed for more than one year in our referral centre. We did not exclude patients with a personal or familial history of psoriasis.ResultsWe included 114 patients followed between January 2008 and December 2015 for a median of 2.5 years (IQR = 2.3). Sixty-nine per-cent of patients fulfilled the revised ILAR classification criteria for enthesitis-related arthritis, and 92% the ASAS criteria for peripheral spondyolarthritis (p < 0.001). Axial disease and sacroiliitis were rare at disease onset. However, they appeared during follow-up in 63% and 47% of cases respectively, after a median disease duration of 2.6 (IC 95% [2.2–4.4]) and 5.3 years (IC 95% [4.1–7.7]), respectively. Multivariable analysis showed that familial history of spondyloarthritis was associated with the presence of sacroiliitis and active disease at the latest follow-up (OR = 3.61 [1.5–8.7], p < 0.01 and 2.98 [1.2–7.3], p = 0.02, respectively).ConclusionAxial involvement developed in most patients within five years. Revised Edmonton criteria were less sensitive than ASAS criteria to classify patients as having childhood-onset spondyloarthropathies. The main risk factor for both sacroiliitis and persistent active disease was a familial history of spondyloarthritis.Electronic supplementary materialThe online version of this article (10.1186/s12969-018-0238-9) contains supplementary material, which is available to authorized users.

Juvenile Spondyloarthritis: focus on uveitis.

“…The high rate of PsA patients in our cohort may explain the absence of male prevalence and lower rate of HLA-B27 positivity compared to other studies [6][7][8]. This may also contribute to the higher rate of uveitis in our cohort.…”

“…Indeed, the previous reported JSpA cohorts are small and analysis does not focus on ocular in ammation. Two recently published JSpA cohorts, one from France (114 patients) and one from Germany (118 patients) shared similar features: ERA patients as the prevalent subset (69% and 52%), male predominance (63% and 73%) and high rate of HLA-B27 positivity (43% and 66%) [6,7]. Another study of JIA reported an HLA-B27 prevalence of 71%, and 87% for ERA and UA, respectively [8].…”

“…JSpA uveitis were only provided in the German cohort: the uveitis rate was 7%, without further description of associated characteristics [6] Similar to these cohorts, the ERA subset was the most represented in our population.…”

Juvenile Spondyloarthritis: focus on uveitis.

“…The high rate of PsA patients in our cohort (18%) may explain the absence of male prevalence and the lower rate of HLA-B27 positivity compared to the other cohorts [6,7]. This may also contribute to the higher rate of uveitis in our cohort compared to the German JSpA cohort (11% vs 7%).…”

“…Two recently published JSpA cohorts, one from France (114 patients) and one from Germany (118 patients) shared similar features: ERA patients as the prevalent subset (69% and 52%), male predominance (63% and 73%) and a rate of HLA-B27 positivity ranging from 43% and 66% [6,7]. Data on uveitis were provided just in the German cohort: the uveitis rate was 7%, without further description of associated characteristics [6].…”