Abstract
BackgroundEnthesitis-related arthritis (ERA) is a specific subtype of juvenile idiopathic arthritis (JIA) defined according to the International League of Associations for Rheumatology (ILAR) criteria. We aimed to characterize the clinical features and treatment regimens in an inception cohort of children with ERA.MethodsWe performed a retrospective, cross-sectional, multicenter cohort study including subjects diagnosed with ERA between 1989 and 2012. Patients all fulfilled the ILAR criteria for ERA within 3 months of initial presentation to the rheumatology clinic. Differences in the prevalence of clinical criteria across study sites and by human leukocyte antigen (HLA)-B27 status were assessed using the Wilcoxon rank-sum or chi-square test, as appropriate.ResultsTwo hundred thirty-four children met the inclusion criteria. Their median age at diagnosis was 11.6 years, and 59% were HLA-B27-positive. Sixty-nine percent had enthesitis and arthritis at the time of diagnosis. Seventy-eight percent had a pauciarticular onset. The prevalence of all ILAR criteria at diagnosis, except arthritis and acute anterior uveitis, differed significantly across sites (all p < 0.01). Medication use varied significantly across sites for children with peripheral arthritis (p < 0.001), but not for sacroiliitis or enthesitis only. Nonsteroidal anti-inflammatory drugs and disease-modifying antirheumatic drugs were the most commonly prescribed treatments, with anti-TNF agents primarily being initiation for sacroiliitis. HLA-B27 positivity was associated with male sex, higher active joint count, sacroiliitis, and higher disease activity at disease onset.ConclusionsThe majority of children had a pauciarticular onset, and several statistically significant clinical differences based on HLA-B27 status were identified. The observed heterogeneity in clinical presentation across sites reflects either true differences in patient populations or differences in how the ILAR criteria are being applied.
Highlights
Enthesitis-related arthritis (ERA) is a specific subtype of juvenile idiopathic arthritis (JIA) defined according to the International League of Associations for Rheumatology (ILAR) criteria
In terms of the initial treatment of ERA, we found that Nonsteroidal anti-inflammatory drug (NSAID) and Disease-modifying antirheumatic drug (DMARD) were most often prescribed
Despite the intersite variation in treatments, our findings suggest that pediatric rheumatologists are generally adhering to American College of Rheumatology (ACR)-recommended treatment guidelines [13, 33, 34] to initiate a Tumor necrosis factor-α (TNF-α) blocker for axial disease, especially early in the disease course [35]; barriers imposed by insurance companies may account for the fact that only 15% of patients received biologic agents in the first 3 months of disease
Summary
Enthesitis-related arthritis (ERA) is a specific subtype of juvenile idiopathic arthritis (JIA) defined according to the International League of Associations for Rheumatology (ILAR) criteria. Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease [1] and includes a number of heterogeneous arthritides. The majority of research conducted in children with ERA has been in the context of juvenile SpA, which includes other conditions such as inflammatory bowel disease and psoriatic arthritis. Treatment recommendations have been published by the American College of Rheumatology (ACR) and are based primarily on the number of active joints and the presence of sacroiliitis [13]. It is unknown whether routine practice reflects these recommendations
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