Purpose
To describe the features of Behçet-associated uveitis over a 25-year period.
Design
Retrospective observational case series.
Methods
A chart review of patients with Behçet-associated uveitis who were evaluated from January 1986 to December 2011 at King Khaled Eye Specialist Hospital, Saudi Arabia. Demographic data, symptoms, type of uveitis, treatment, and complications were evaluated. The main outcome measures were presenting symptoms, types of uveitis, treatment, and complications.
Results
There were 132 patients (232 eyes; 102 male [77.3%]) evaluated with age of onset of 36.9 ± 11.4 years. Panuveitis was the most common presentation, affecting 118 patients (89.4%). Episodes were bilateral in 100 patients (75.8%). Baseline best-corrected visual acuity (BCVA) was 20/125 in both eyes. Retinal vasculitis at presentation occurred in 61 eyes (26.3%), occlusive vasculitis in 59 eyes (25.4%), and macular edema in 42 eyes (18.1%). Common therapeutic management included oral corticosteroids in 123 patients (93.2%), intravenous steroid therapy in 35 patients (26.5%), cyclosporine in 98 patients (74.2%), and azathioprine in 65 patients (49.2%). Common anterior segment complications included glaucoma (44 eyes, 19%) and cataracts (34 eyes, 14.7%). The most common posterior segment complication was optic nerve atrophy. Cataract surgery was the most common surgery. At last visit, BCVA was better than 20/50 in 131 eyes (56.5%).
Conclusions
Behçet-associated uveitis predominantly affects young men in Saudi Arabia. Bilateral panuveitis associated with retinal vasculitis was the most common manifestation. More than 50% of patients maintained 20/50 or better BCVA at final follow-up and were primarily managed with oral corticosteroids and other immunosuppressive agents.
Behçet disease is a multisystem inflammatory disease of unknown etiology, characterized by recurrent episodes of retinal vasculitis, uveitis, genital ulcers, oral ulcerations, and skin lesions. The disease is named for Hulusi Behçet, a Turkish dermatologist, who in 1936 described the symptomatic triad of uveitis with recurrent oral and genital ulcers. It should be noted that in the 20th century at least 2 other clinicians reported cases with similar findings: Shigeta from Japan in 1924 and Adamantiadis in 1931. Therefore, this disorder also is known as Adamantiadis-Behçet syndrome. However, the disease was known in ancient times, and Hippocrates was likely the first to describe an association between ocular inflammation and oral and genital lesions.
Behçet disease occurs worldwide. However, its prevalence varies considerably based on geographic location and race. Prevalence rates vary from 1 per 10 000 inhabitants to 42 per 10 000 inhabitants from Japan and Turkey, respectively. In Saudi Arabia, Behçet disease is one of the most common causes of uveitis. In our most recent survey it is second only to Vogt-Koyanagi-Harada syndrome (Arevalo et al. American Academy of Ophthalmology Annual Meeting, Chicago, IL, 2012, unpublished data) as a cause of panuveitis (26% of patients). Although Behçet can occur at any age, the usual age of onset is 20–30 years. Ocular involvement has been reported in up to 70% of patients with Behçet.
Behçet is diagnosed based on specific clinical symptoms and signs, as there is no specific laboratory test. The Behçet Disease Research Committee of Japan devised a set of criteria for the diagnosis of Behçet disease in 1974, and the International Study Group (ISG) for Behçet disease published diagnostic criteria in 1990. According to ISG criteria, the diagnosis of Behçet disease requires the presence of recurrent oral ulceration in addition to at least 2 other features: recurrent genital ulceration, typical eye lesions, typical skin lesions, or positive pathergy test. Intraocular inflammation may involve the anterior or posterior segment or, more commonly, both segments. Intraocular inflammation clinically presents as episodic and recurrent iridocyclitis along with vitritis, retinitis, occlusive vasculitis, and cystoid macular edema, alternating with periods of inactive phases. Immunosuppresive therapy is the mainstay of treatment in these patients. Complications such as band keratopathy, glaucoma, vitreoretinal hemorrhage, retinal detachment, macular degeneration, epiretinal membrane, vein occlusion, and finally phthisis bulbi may be encountered.
In the current study, we retrospectively analyzed the demographic and clinical features, ocular manifestations, complications, visual outcomes, and management of uveitis in a large population of patients with Behçet disease that were seen over the last 25 years in a single tertiary center in Saudi Arabia.
Patients and Methods
This was an observational case series. Data were collected by retrospective chart review. All patients diagnosed with ocular Behçet disease from January 1986 to December 2011 at the King Khaled Eye Specialist Hospital (KKESH), Saudi Arabia, were recruited for a uveitis survey according to a predetermined protocol. Out of 888 consecutive uveitis patients (1455 eyes), 132 patients (14.9%) (232 eyes) with Behçet disease were evaluated. Patients were referred to our institution from other centers within Saudi Arabia and other countries from the Arabian Gulf, and some patients were self-referred. All patients with ocular Behçet disease at initial diagnosis and treated at KKESH were included in this study. The clinical records were reviewed of 132 patients (232 eyes) diagnosed with ocular Behçet. Only patients who met the 1990 ISG classification criteria were included in this study. This study followed the tenets of the 1964 Declaration of Helsinki and was approved as a retrospective study by the KKESH Human Ethics Committee/Institutional Review Board. Demographic information was collected based on self-reported data obtained at the initial visit via a uveitis questionnaire, verbal history, and physical examination. Data were collected on race, ethnicity, and country of origin.
Best-corrected visual acuity (BCVA) was measured with Snellen charts and the values were converted to logarithm of minimal angle of resolution equivalent units (logMAR) for statistical calculations. Ophthalmic examination was performed including slit-lamp biomicroscopy, dilated fundus examination, and ancillary methods as required.
Classification of uveitis, as well as grading of anterior and posterior segment inflammation, was determined using the Standardization of Uveitis Nomenclature (SUN) criteria. The intraocular inflammation was considered inactive or controlled if the inflammatory activity was graded ≤0.5+ cells in the anterior chamber. Vitreous haze was graded according to the National Eye Institute system with binocular indirect ophthalmoscopy.
Disease duration was defined as the time interval between the onset of ocular symptoms and diagnosis of uveitis and time of presentation. For the purposes of this study, eyes that experienced only iridocyclitis were classified as anterior uveitis; posterior uveitis was defined as eyes that experienced retinitis, retinal vasculitis, diffuse vitritis, and/or papillitis; and panuveitis was classified as eyes that experienced retinitis, retinal vasculitis, diffuse vitritis, and/or papillitis in addition to iridocyclitis. The type and duration of therapy used for controlling the intraocular inflammation and the visual outcome were recorded. Complications, such as cataracts, glaucoma, and choroidal neovascularization, were noted.
All data were collected in a Microsoft Office Excel 2007 spreadsheet (Microsoft Corp, Redmond, Washington, USA) and statistically analyzed by MedCalc Software for Windows 8.2.0.3 (MedCalc, Mariakerke, Belgium).
Results
Panuveitis was the most common form of ocular presentation, affecting 118 of 132 patients (89.4%), followed by anterior uveitis in 10 patients (7.6%) and intermediate uveitis in 4 patients (3%). The mean follow-up was 97.5 ± 80.9 months (range: 4–298 months). One hundred and two patients (77.3%) were male. Mean age at onset of disease was 36.9 ± 11.4 years. Episodes were bilateral in 100 patients (75.8%). Baseline BCVA was 20/125 (logMAR = 0.8 ± 0.8) in both eyes. Presenting visual acuity was equal to or better than 20/50 (logMAR = 0.4) in 87 eyes (37.5%) and 20/200 (logMAR = 1) or worse in 89 affected eyes (38.4%). Mean intraocular pressure (IOP) was normal in all eyes. Retinal vasculitis at first presentation was found in 120 eyes and of those, about half (59/120, 49.1%) were occlusive cases ( Figures 1–3 ). The most common location of retinal occlusive vasculitis was peripheral that was present in 37 of 59 eyes (62.7%) with Behçet uveitis. These cases were treated and controlled with immunosuppressive therapy. All the causes and modalities of treatment for occlusive vasculitis are presented in Table 1 . Laser photocoagulation (either focal retinal photocoagulation or panretinal/scatter photocoagulation) was performed for treatment of occlusive vasculitis based on etiology.
| Diagnosis | N (%) | LP | PRP | IMT | Observation |
|---|---|---|---|---|---|
| BRVO | 12 (20.3%) | 4 | 8 | ||
| CRVO | 2 (3.4%) | 2 | |||
| NVE | 4 (6.8%) | 4 | |||
| NVD | 4 (6.8%) | 4 | |||
| PV | 37 (62.7%) | 37 | |||
| Total | 59 (100%) | 4 | 10 | 37 | 8 |
Retinal detachment (RD) was present in 22 of 232 eyes (9.4%); of those, an exudative retinal detachment (ERD) was diagnosed in 11 of 22 eyes (50%), tractional retinal detachment (TRD) in 9 of 22 eyes (40.9%) ( Figure 4 ), and a rhegmatogenous retinal detachment (RRD) in 2 of 22 eyes (9.1%).
Therapeutic management included oral systemic corticosteroids in 123 patients (93.2%), intravenous steroid therapy in 35 patients (26.5%), immunosuppressive agents such as cyclosporine in 98 patients (74.2%), azathioprine in 65 patients (49.2%), infliximab in 12 patients (9.1%), and methotrexate in 6 patients (4.5%). Local steroid therapy (sub-Tenon triamcinolone) and intravitreal triamcinolone were administered in 14 (10.6%) and 5 patients (3.8%), respectively.
We observed that the combination of drugs used for the treatment of Behçet disease varied considerably over the years. Therefore, we decided to divide our patients into different treatment groups according to the therapy administered. Visual outcomes and the treatment strategies are summarized in Table 2 . Only the combination of prednisone, cyclosporine, and infliximab demonstrated a significant improvement in BCVA ( P = .032).
| Patients, n (%) | Eyes, n (%) | Basal BCVA | Final BCVA | P Value | |||
|---|---|---|---|---|---|---|---|
| ETDRS | logMAR | ETDRS | logMAR | ||||
| PD+AZA+CSP | 44 (33.3%) | 72 (31.1%) | 20/150 | 0.9 ± 0.6 | 20/80 | 0.6 ± 0.8 | .074 |
| PD+CSP | 35 (34.1%) | 64 (27.6%) | 20/150 | 0.9 ± 0.6 | 20/125 | 0.8 ± 1.1 | .436 |
| Prednisone | 16 (12.1%) | 30 (12.9%) | 20/125 | 0.8 ± 0.9 | 20/100 | 0.7 ± 1.2 | .551 |
| PD+AZA | 16 (12.1%) | 28 (12.1%) | 20/80 | 0.6 ± 0.5 | 20/100 | 0.7 ± 1 | .726 |
| PD+CSP+IFX | 10 (7.5%) | 16 (6.9%) | 20/200 | 1 ± 0.3 | 20/50 | 0.6 ± 0.4 | .032 |
| CSP+AZA | 5 (3.8%) | 10 (4.3%) | 20/40 | 0.3 ± 0.3 | 20/25 | 0.1 ± 0.1 | .433 |
| MTX+CSP | 4 (3.0%) | 8 (3.4%) | 20/100 | 0.7 ± 0.8 | 20/30 | 0.2 ± 0.7 | .091 |
| PD+MTX+IFX | 2 (1.5%) | 4 (1.7%) | 20/50 | 0.4 ± 0.3 | 20/50 | 0.4 ± 0.5 | .677 |
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