Juvenile Spondyloarthritis: focus on uveitis
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Juvenile Spondyloarthritis: focus on uveitis Achille Marino1,2, Pamela F. Weiss3,4, Timothy G. Brandon4 and Melissa A. Lerman3,4*
Abstract Background: Juvenile spondyloarthritis (JSpA) represents a group of inflammatory arthritides with several distinctive features (enthesitis, involvement of spine and sacroiliac joint, HLA-B27 association and development of uveitis). There are limited data on the course of uveitis in children with JSpA. This study aims to estimate the prevalence of uveitis and to look at the presence of HLA-B27 in relation to uveitis occurrence and ocular symptoms in a cohort of JSpA patients. Findings: This is a cross sectional/retrospective study involving patients with JSpA followed in a tertiary referral hospital. Two hundred twenty-three patients were enrolled in the study. The prevalent diagnosis was enthesitis-related arthritis (ERA) (62%) followed by juvenile psoriatic arthritis (PsA), undifferentiated arthritis (UA), and the arthropathies associated with inflammatory bowel disease (IBD-A) (18, 14, 6%, respectively). Uveitis was reported in twenty-four patients (11%) of the JSpA cohort (JSpA-U). ERA patients had the highest uveitis prevalence (ERA-U) (13%) with similar prevalences in UA, PsA and in IBD-A (7% each). The prevalence of HLA-B27 positivity was similar amongst the entire JSpA-U cohort (N = 22, 45%) and those with ERA-U (N = 8, 44%). The overall prevalence of symptomatic uveitis was 79%. Neither the likelihood of uveitis, nor of symptomatic uveitis, varied by HLA-B27 status either in the entire cohort nor in those with ERA. Conclusions: About one-tenth of patients developed uveitis, the majority of which was symptomatic. Fewer than half of the patients with uveitis were HLA-B27 positive. HLA-B27 status was not statistically associated with either the development of uveitis or symptomaticity of uveitis. Keywords: Juvenile spondyloarthritis, Juvenile idiopathic arthritis, Enthesitis-related arthritis, Uveitis, HLA-B27
Introduction Spondyloarthritis (SpA) represents a group of inflammatory arthritides affecting both adults and children with several distinctive features such as the presence of enthesitis, the potential involvement of spine and sacroiliac joint, a strong association with HLA-B27 and development of uveitis that is more often acute onset and/or symptomatic [1]. International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA) * Correspondence: [email protected] 3 Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Blvd, Philadelphia, PA 19104, USA 4 Division of Rheumatology, The Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104-4399, USA Full list of author information is available at the end of the article
[2] does not recognize a specific category for Juvenile SpA (JSpA) patients. Yet, spondyloarthritis includes enthesitis-related arthritis (ERA), juvenile psoriatic arthritis (PsA), undifferentiated arth
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