Enthesitis-related Arthritis: Prevalence and Complications of Associated Uveitis in Children and Adolescents From a Population-based Nationwide Study in Germany

Abstract

Objective Enthesitis-related arthritis (ERA) represents a subgroup of juvenile idiopathic arthritis (JIA) which is regularly accompanied by anterior uveitis. This study describes the prevalence and characteristics of ERA-related uveitis. Methods Cross-sectional data from the National Pediatric Rheumatological Database (NPRD) were used to characterize ERA-related uveitis (ERA-U). In addition to sociodemographic parameters, we documented the occurrence of uveitis and course of disease - including symptoms, visual acuity, and complications - as well as JIA characteristics such as disease activity (cJADAS10), functional ability (CHAQ score), laboratory parameters, and treatment. Results In the years from 2002 to 2014, 3,778 (15.2%) of a total of 24,841 JIA patients recorded in the NPRD had ERA, and 280 (7.4%) of them had developed uveitis. Detailed ophthalmological documentation by an uveitis add-on module was available for 22.9% of these patients. Uveitis onset was acutely symptomatic in 63% of patients. Patients with uveitis were more frequently male, HLA-B27 positive, and younger at ERA onset, and they had higher ESR values at first uveitis documentation than those without uveitis. Uveitis was diagnosed at a mean age of 11.5 (± 3.9) years (50% within two years after ERA onset). Systemic treatment with corticosteroids and synthetic and biologic disease-modifying antirheumatic drugs was associated with a (not significantly) lower risk of developing uveitis. Conclusion The course of disease in ERA-U patients is frequently similar to HLAB27-associated uveitis in adults; however, a subgroup of patients presents with asymptomatic uveitis.

Uveitis bei juveniler idiopathischer Arthritis – leitliniengerechte Diagnostik und Therapie anhand von Kasuistiken

“…Guideline of the German Society of Ophthalmology (DOG) 1 and the Society for Paediatric and Adolescent Rheumatology (GKJR) 2 with the cooperation of the following specialist societies: Professional Association of Ophthalmologists (BVA) 3 ; German Society of Rheumatology (DGRh) 4 ; patient advocacy group of children suffering from uveitis and their families 5 . Methodologically reviewed by the AWMF according to AWMF rules for the guideline register; methodological support 6 .…”

“…Leitlinie der Deutschen Ophthalmologischen Gesellschaft (DOG) 1 und der Gesellschaft für Kinder-und Jugendrheumatologie (GKJR) 2 unter Mitwirkung folgender Fachgesellschaften: Berufsverband der Augenärzte (BVA) 3 ; Deutsche Gesellschaft für Rheumatologie (DGRh) 4 ; Patientengruppe an Uveitis erkrankter Kinder und deren Familien 5 . Methodisch nach AWMF-Regeln für das Leitlinienregister geprüft durch die AWMF; methodische Begleitung 6 .…”

Juvenile Idiopathic Arthritis Associated Uveitis

“…A recent study from Japan reported a uveitis prevalence of 6.1% among JIA patients [16]. [5,6,9,10,14,[16][17][18][19][20]. RF, rheumatoid factor.…”

“…ERA JIA is defined as arthritis and enthesitis, or arthritis or enthesitis with at least two of the following: the presence of or a history of sacroiliac joint tenderness and/or inflammatory lumbosacral pain, HLA-B27 positivity, the onset of arthritis in a male over 6 years of age, acute (symptomatic) anterior uveitis, or history of HLA-B27-associated disease [65]. HLA-B27 positive ERA JIA patients are at increased risk compared with HLA-B27 negative [20,66]. Uveitis is also a common feature of adultonset SpA where the vast majority of patients are HLA-B27 positive [67], underscoring the aforementioned potential role for HLA-B27 in uveitis development.…”

Recent advances in the treatment of juvenile idiopathic arthritis–associated uveitis

“… 13 , 14 ERA is slightly less prevalent, accounting for 10–20% of JIA with incidence ranging from 7% to 30%. 4 , 15 , 16 The peak age of onset for JIA occurs at around 2 years, and JIA-associated uveitis presents at a median age of 3.5 years in females and 5.7 years in males. 5 , 10 In the majority of these cases, uveitis presents within 3 years of the diagnosis of JIA, with about 50% of cases occurring during or immediately after symptomatic arthritis.…”