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      Characteristics of a cohort of children with Juvenile Idiopathic Arthritis and JIA-associated Uveitis

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          Abstract

          Background

          Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to poor visual outcomes and impact a child’s quality of life (QOL) and function. Our aim is to identify risk markers of JIA-U and examine differences in the QOL of children with JIA and JIA-U.

          Methods

          Rheumatology and ophthalmology record reviews and questionnaires were completed every 4–6 months on 287 children with JIA. We collected arthritis, uveitis, and QOL data. We examined data through last study visit.

          Results

          There were 52/287 (18 %) children with JIA-U who were younger at arthritis diagnosis, had oligoarticular persistent JIA, and ANA positive. Confirmed uveitis predictors were age at JIA diagnosis (OR = 0.86) and oligoarticular subtype (OR = 5.92). They had worse vision specific QOL and function, but similar general QOL. Blindness occurred in 17.5 % of children but was more common in African American children compared to non-Hispanic Caucasian children ((5/7 (71 %) vs. 2/29 (7 %), p <0.001) despite a similar uveitis prevalence (22 % vs. 16 %). Both races had similar complications, although band keratopathy was more frequent in African Americans (75 % vs. 15.6 %, p = 0.003).

          Conclusions

          We confirm young age at JIA diagnosis and the oligoarticular JIA subtype as predictors of uveitis development. Although we were unable to identify predictors of ocular complications or blindness, AA children appeared to have a more severe disease course manifested by increased ocular complications, vision loss and blindness. Potential causes that warrant additional study include underlying disease severity, access to medical care and referral bias. Further investigation of the risk factors for vision-compromising uveitis and its’ long-term effects should be conducted in a large racially diverse population.

          Electronic supplementary material

          The online version of this article (doi:10.1186/s12969-015-0018-8) contains supplementary material, which is available to authorized users.

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          Most cited references34

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          Juvenile idiopathic arthritis.

          Juvenile idiopathic arthritis is a broad term that describes a clinically heterogeneous group of arthritides of unknown cause, which begin before 16 years of age. This term encompasses several disease categories, each of which has distinct methods of presentation, clinical signs, and symptoms, and, in some cases, genetic background. The cause of disease is still poorly understood but seems to be related to both genetic and environmental factors, which result in the heterogeneity of the illness. Although none of the available drugs has a curative potential, prognosis has greatly improved as a result of substantial progresses in disease management. The most important new development has been the introduction of drugs such as anticytokine agents, which constitute a valuable treatment option for patients who are resistant to conventional antirheumatic agents. Further insights into the disease pathogenesis and treatment will be provided by the continuous advances in understanding of the mechanisms connected to the immune response and inflammatory process, and by the development of new drugs that are able to inhibit selectively single molecules or pathways.
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            Measurement of health status in children with juvenile rheumatoid arthritis.

            To develop and validate a self- or parent-administered instrument for measuring functional status in children with juvenile rheumatoid arthritis (JRA). We adapted the Stanford Health Assessment Questionnaire (HAQ) for use in children ages 1-19 years, by adding several new questions, such that for each functional area, there was at least 1 question relevant to children of all ages. The face validity of the instrument was evaluated by a group of 20 health professionals and parents of 22 healthy children. The questionnaire was then administered to parents of 72 JRA patients (mean age 9.1 years, onset type systemic in 16, polyarticular in 21, pauciarticular in 35). The instrument showed excellent internal reliability (Cronbach's alpha = 0.94), with a mean inter-item correlation of 0.6. The convergent validity was demonstrated by strong correlations of the Disability Index (average of scores on all functional areas) with Steinbrocker functional class (Kendall's tau b = 0.77, P 8 years) was 0.84 (n = 29; P 0.9 by paired t-test; Spearman's correlation coefficient = 0.8, P < 0.002). The Childhood HAQ, which takes less than 10 minutes to complete, is a valid, reliable, and sensitive instrument for measuring functional status in children with JRA.
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              Prevalence and complications of uveitis in juvenile idiopathic arthritis in a population-based nation-wide study in Germany: suggested modification of the current screening guidelines.

              To analyse the prevalence and complications of uveitis and their predictors in a large cohort of patients with juvenile idiopathic arthritis (JIA). Data of 3271 JIA patients as classified by International League of Associations for Rheumatology (ILAR) criteria included in a national database during 1 yr were analysed. Uveitis prevalence was 12% of all JIA patients. The most frequent were oligoarthritis extended (25%) and persistent (16%). JIA patients with uveitis were significantly younger at onset of arthritis (3.8 vs 7.0 yrs) or ANA-positive (86% vs 42%) than the patients without uveitis. Predictors of uveitis included age at onset (P= 0.03) and ANA-positivity (P< 0.01) besides the presence of a certain JIA subgroup (P= 0.04). Uveitis was clinically silent in 75% of the oligoarthritis but in none of the enthesitis-related arthritis patients. The median onset of uveitis was 5.5 months after arthritis manifestation. In 73%, 77% and 90%, uveitis developed within 1, 2 and 4 yrs after arthritis, respectively. Anterior uveitis was the most common anatomic type of uveitis (83%). Uveitis complications at mean follow-up of 5.6 yrs were common (56%), and predictors for complications included presence of complications at first visit (P< 0.001) and uveitis manifestation before arthritis (P= 0.001), but not ANA positivity. The JIA subgroups markedly differ with respect to the prevalence and course of associated uveitis. Ophthalmological screening should be initiated early after arthritis onset and the intervals be related to the JIA subgroup. A modification of the current screening guidelines is suggested.
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                Author and article information

                Contributors
                sangele@emory.edu
                courtney.mccracken@emory.edu
                steven.yeh@emory.edu
                Kirsten.Jenkins@choa.org
                daneka.stryker@emory.edu
                kelly.a.rouster-stevens@emory.edu
                lvogler@emory.edu
                scott.lambert@emory.edu
                cdrews@emory.edu
                sprahal@emory.edu
                Journal
                Pediatr Rheumatol Online J
                Pediatr Rheumatol Online J
                Pediatric Rheumatology Online Journal
                BioMed Central (London )
                1546-0096
                2 June 2015
                2 June 2015
                2015
                : 13
                : 19
                Affiliations
                [ ]Department of Pediatrics, Emory University School of Medicine, 2015 Uppergate Drive, Atlanta, GA 30322 USA
                [ ]Children’s Healthcare of Atlanta, 1405 Clifton Rd, Atlanta, GA 30329 USA
                [ ]Department of Ophthalmology, Emory University School of Medicine, 1365-B Clifton Rd, Atlanta, GA 30322 USA
                [ ]Rollins School of Public Health, Emory University, 1518 Clifton Rd, Atlanta, GA 30322 USA
                [ ]Department of Genetics, Emory University School of Medicine, Atlanta, GA 30322 USA
                Article
                18
                10.1186/s12969-015-0018-8
                4450472
                26031738
                356a5187-ac0c-4ef6-9cc0-383114c496a1
                © Angeles-Han et al.; licensee BioMed Central. 2015

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 17 February 2015
                : 22 May 2015
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2015

                Pediatrics
                juvenile idiopathic arthritis,uveitis,risk factors,quality of life
                Pediatrics
                juvenile idiopathic arthritis, uveitis, risk factors, quality of life

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