Original article
Childhood-onset uveitis in Behçet disease:a descriptive study of 36 cases

This study was presented in part at the 12th Congress of the European Society of Ophthalmology, Stockholm, Sweden, June 27–July 1, 1999, and at the International Symposium on Intraocular Inflammation, Bangalore, India, March 1–2, 2003.
https://doi.org/10.1016/S0002-9394(03)00791-8Get rights and content

Abstract

Purpose

To describe the demographic and clinical features, complications, treatment, and visual results in patients with childhood-onset Behçet uveitis.

Design

Observational case series.

Methods

A retrospective study was made of 36 consecutive patients with Behçet disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Inclusion criteria were fulfillment of the classification criteria of the International Study Group for Behçet Disease and onset of uveitis at 16 years of age or younger. The medical records of 36 patients with childhood-onset Behçet uveitis were reviewed. The main outcome measures were sex, age at onset of uveitis, the initial symptom of Behçet disease, clinical ocular features, ocular complications, systemic treatment, complications of treatment, and final visual acuity.

Results

Twenty-five patients were male, 11 were female. Mean age at onset of uveitis was 13.6 years. The initial symptom was oral ulcer in 63.8% of the patients. The majority of patients (83.3%) had bilateral involvement. Panuveitis was the most common form (86.2%). Retinal vasculitis and retinitis were the most common ocular findings seen in 83.3% and 68.2% of the involved eyes, respectively. Cataract, maculopathy, and optic atrophy were the most common complications seen in 46.9%, 45.4%, and 39.4% of the involved eyes, respectively. Immunosuppressive therapy was administered to 75% of the patients. Response to treatment was variable. The most common complications of systemic treatment were associated with corticosteroid therapy. Final visual acuity was worse than 0.1 in 22.7% of the involved eyes.

Conclusions

Childhood-onset Behçet uveitis was more common among males. Bilateral panuveitis with retinal vasculitis and retinitis was the most common form of ocular involvement, similar to the adult patient. The treatment is challenging, as the use oral corticosteroids is associated with significant complications and the response to conventional immunosuppressive therapy is variable.

Section snippets

Methods

We performed a retrospective analysis of the medical records of 36 consecutive patients with childhood-onset ocular involvement of Behçet disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Only patients who met the 1990 classification criteria of the International Study Group (ISG) for Behçet disease24 and who had uveitis at 16 years of age or younger were included in the study. Data

Results

The demographic features of the patients are shown in Table 1. Twenty-five patients were male, 11 were female. Mean age at presentation was 15.4 ± 3.3 years (range, 9–27). Mean age at onset of uveitis was 13.6 ± 2.1 years (range, 9–16). The initial symptom of Behçet disease was oral ulcers in 23 patients, genital ulcers in 2, both oral and genital ulcers in 3, skin lesions in 4, uveitis in 3, and arthritis in 1 patient. Twenty-one patients (58.3%) sought medical attention for the first time

Discussion

In the present study, only patients with childhood-onset Behçet uveitis selected through a uveitis service were analyzed, which may lead to a bias in drawing conclusions on the epidemiologic and systemic features of Behçet disease in this age group. Our male patients outnumbered female patients by a ratio of 2.3 to 1. Onset of uveitis was in late childhood. Bilateral panuveitis with retinal vasculitis and retinal infiltrates was the typical presentation. Although uveitis was the initial

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    This study was supported by the Istanbul University Research Foundation (Project Number B-108/150499).

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