Clinical Features of Chinese Patients with Behçet’s Disease

Purpose

To characterize the clinical features of Behçet's disease in Chinese patients.

Design

Retrospective noncomparative case series.

Participants

Seven hundred seventy-five eyes of 437 patients with Behçet's disease initially examined from August, 1995, through June, 2006.

Methods

The history, demographic parameters, and clinical findings of all consecutive Behçet's patients referred to the uveitis study center of Sun Yat-sen University were reviewed. Laser flare-cell photometry (LFCM), fundus fluorescein angiography (FFA), optical coherence tomography (OCT), ultrasound biomicroscopy (UBM), and B-scan ultrasonography were performed in certain cases. Most patients were treated by immunosuppressive agents combined with oral corticosteroids.

Main Outcome Measures

Patient demographics, clinical presentation, and auxiliary examination findings.

Results

Four hundred thirty-seven patients were diagnosed with Behçet's disease. There were 319 male and 118 female patients. Panuveitis was the most common type of uveitis in both genders, although anterior uveitis was seen more frequently in females. Retinal vasculitis, vitreitis, and retinitis were the most common ocular manifestations in these patients. Cataract and macular edema were the most common complications. Oral aphthae were the most frequent extraocular manifestation, followed by dermatologic lesions, and genital ulcers. The results of FFA, OCT, B-scan ultrasonography, LFCM, and UBM generally were in accordance with or comparable with clinical observations. At the final visit, uveitis was well controlled in 86.2% of patients. However, 20.4% of eyes became legally blind despite aggressive treatment. Kaplan-Meier analysis estimated the risk of losing useful vision (0.05) at 5 and 10 years for males and females as 29% versus 6% and 65% versus 33%, respectively.

Conclusions

Behçet's disease in Chinese patients mainly manifests as nongranulomatous uveitis frequently associated with oral aphthae and dermatologic lesions. Fundus fluorescein angiography, B-scan ultrasonography, LFCM, UBM, and OCT may provide much information about pathophysiologic hallmarks of Behçet's disease. A combination of corticosteroids with other immunosuppressive agents is effective for most patients with Behçet's disease. Males had a more severe course and were at higher risk for losing vision than females.