Uveitis in juvenile Behçet's disease: clinical course and visual outcome compared with adult patients

Kramer, Michal; Amer, Radgonde; Mukamel, Masza; Snir, Moshe; Jaouni, Tareq; Friling, Ronit

doi:10.1038/eye.2008.397

Cited by 17 publications

(6 citation statements)

References 14 publications

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“…Despite not being frequently tested in pediatric cohorts, this result is in agreement with the few studies reporting a frequency which ranges from 44 to 56.8% [ 18 , 30 , 39 , 41 ]. A lower rate has been reported in German patients and a higher rate in Turkish patients [ 24 ] as well as in studies specifically investigating uveitis in juvenile cohorts, where HLA-B51 positivity has reached 75% [ 42 , 43 ]. Interestingly, our group has recently reported long-term outcomes of BS-related uveitis, suggesting HLA-B51 as a predictor of long-term eye-related complications [ 44 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

et al. 2021

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Behçet’s syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263–0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.

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Section: Discussionmentioning

confidence: 99%

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

et al. 2021

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“…These reasons included that patients were not naive to anti-TNF␣, that outcome measures were not extractable (32)(33)(34), and/or that the articles did not refer specifically to children, even if the study population included children (e.g., studies of Behçet's disease [35,36] or other secondary ocular inflammation [37]). Díaz-Llopis et al reported one of the largest prospective studies on ADA efficacy in refractory chronic uveitis, including 39 JIA children among 131 enrolled patients (38).…”

Section: Discussionmentioning

confidence: 99%

Current Evidence of Anti–Tumor Necrosis Factor α Treatment Efficacy in Childhood Chronic Uveitis: A Systematic Review and Meta‐Analysis Approach of Individual Drugs

Simonini

Druce

Cimaz

et al. 2014

Arthritis Care & Research

100

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Objective. To summarize evidence regarding the effectiveness of anti-tumor necrosis factor ␣ (anti-TNF␣) treatments in childhood autoimmune chronic uveitis (ACU), refractory to previous disease-modifying antirheumatic drugs (DMARDs). Methods. A systematic search between January 2000 and October 2012 was conducted using EMBase, Ovid Medline, Evidence-Based Medicine (EBM) Reviews: American College of Physicians Journal Club, Cochrane libraries, and EBM Reviews. Studies investigating the efficacy of anti-TNF␣ therapy, in children ages <16 years, as the first treatment with a biologic agent for ACU, refractory to topical and/or systemic steroid therapy and at least 1 DMARD, were eligible for inclusion. The primary outcome measure was the improvement of intraocular inflammation, as defined by the Standardization of Uveitis Nomenclature Working Group criteria. We determined a combined estimate of the proportion of children responding to anti-TNF␣ treatment, including etanercept (ETA), infliximab (INF), or adalimumab (ADA). Results. We initially identified 989 articles, of which 148 were potentially eligible. In total, 22 retrospective chart reviews and 1 randomized clinical trial were deemed eligible, thus including 229 children (ADA: n ‫؍‬ 31, ETA: n ‫؍‬ 54, and INF: n ‫؍‬ 144). On pooled analysis of observational studies, the proportion of responding children was 87% (95% confidence interval [95% CI] 75-98%) for ADA, 72% (95% CI 64 -79%) for INF, and 33% (95% CI 19 -47%) for ETA. There was no difference in the proportion of responders between ADA and INF ( 2 ‫؍‬ 3.06, P ‫؍‬ 0.08), although both showed superior efficacy compared with ETA (ADA versus ETA: 2 ‫؍‬ 20.9, P < 0.001 and INF versus ETA: 2 ‫؍‬ 20.9, P < 0.001). Conclusion. Although randomized controlled trials are needed, the available evidence suggests that INF and ADA provide proven similar benefits in the treatment of childhood ACU, and they are both superior to ETA.

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“…Boys experience a more severe eye involvement [15,30,41]. Anterior uveitis was reported more often in juvenile than in adult patients [35]. Hypopyon was reported in 9% of JABD patients [15].…”

Section: Clinical Features Of Jabdmentioning

confidence: 99%

“…The frequency of recurrent eye involvement in JABD ranges from 7.5 to 80% [9,12,13,15,16,17,21,25,26,27,28,29,30,33,35]. In a recent cohort of 3,382 ABD patients, only 3.3% were younger than 15 years, whereas 31% of the children had been diagnosed with ocular involvement [15].…”

Section: Clinical Features Of Jabdmentioning

confidence: 99%

Juvenile Adamantiades-Behçet Disease

Vaiopoulos

Kanakis²,

Kapsimali

et al. 2016

Dermatology

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Adamantiades-Behçet disease (ABD) is a chronic, multisystemic, recurrent, inflammatory vascular disorder of unknown etiology. Patients with symptoms initially appearing at the age of 16 or less are considered as cases of juvenile-onset ABD (JABD). JABD is relatively rare compared to ABD of adults, and only case reports and case studies have been published regarding this subtype of the disease. Epidemiology, clinical features, diagnosis and treatment of JABD are discussed in this review.

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Uveitis in juvenile Behçet's disease: clinical course and visual outcome compared with adult patients

Cited by 17 publications

References 14 publications

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

Current Evidence of Anti–Tumor Necrosis Factor α Treatment Efficacy in Childhood Chronic Uveitis: A Systematic Review and Meta‐Analysis Approach of Individual Drugs

Juvenile Adamantiades-Behçet Disease

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