Meri Urgancioglu

Prevalence of Behçet's disease in Istanbul, Turkey

Background  The prevalence of Behçets disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Three previous epidemiologic surveys have been carried out in different regions of Turkey.

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

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.

Use of laser flare-cell photometry to quantify intraocular inflammation in patients with Behçet Uveitis

PurposeTo assess the usefulness of laser flare-cell photometry to quantify intraocular inflammation in patients with Behçet disease.MethodsThe study comprised 47 healthy individuals, 78 Behçet patients without ocular involvement, 54 Behçet patients with a uveitis attack and 53 Behçet patients with uveitis in clinical remission. A single observer assigned clinical scores to anterior chamber cells, vitreous haze, and fundus lesions in the attack group. Laser flare-cell photometry measurements were performed by another observer who was masked to the clinical findings. Fundus fluorescein angiography was performed only in the remission group, and fluorescein leakage was scored by a masked retina specialist. The risk of recurrent uveitis attack was analyzed in eyes with high versus low flare values in the remission group. Main outcome measures were anterior chamber flare in Behçet patients compared to the control group, and correlations of flare with clinical scores of intraocular inflammation and with fluorescein leakage. Mann-Whitney U-test, Spearman’s bivariate correlation test, linear regression method, and Kaplan-Meier method were used for statistical analyses.ResultsMean flare was not increased in Behçet patients without ocular involvement. It was significantly higher in patients with Behçet uveitis both during attacks and in remission (P < 0.001 for each). A significant correlation was found between anterior chamber flare and anterior chamber cell score (rho = 0.705), vitreous haze score (rho = 0.588), and fundus score (rho = 0.464) in the attack group. In the remission group, there was a significant correlation between flare and fluorescein angiography leakage score, and the risk of recurrent uveitis attack was significantly higher in eyes with flare values >6 photons/msec than in eyes with flare values ≤6 photons/msec (right eyes, P < 0.001; left eyes, P = 0.0184).ConclusionsLaser flare-cell photometry is a useful objective method in the quantitative assessment of intraocular inflammation in patients with Behçet uveitis. The use of this quantitative technique in clinical trials of Behçet uveitis may provide comparable data, as it gives an objective measure of intraocular inflammation. In clinical practice, it may reduce the need for fluorescein angiography because it seems to be especially useful in monitoring persistent retinal vascular leakage in patients who are clinically in remission.

Neovascularization of the Optic Disc in Behçet's Disease

PurposeTo investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behçets disease and to evaluate the effects of medical and laser treatments.MethodsThis is a retrospective study of 26 consecutive Behçets patients (38 eyes) with NVD who presented between 1990 and 2004 at our university hospital. Information on age at presentation, sex, disease duration, laterality of NVD, ocular findings, fluorescein angiographic findings, visual acuity, medical treatment, laser photocoagulation, surgical procedures, and the follow-up period was collected. Eyes with diffuse capillary leakage on fluorescein angiography were defined as having inflammation-induced NVD, and eyes with extensive retinal capillary nonperfusion were defined as having ischemia-induced NVD.ResultsEighteen patients were male, and eight were female. Mean age at presentation was 25.4 ± 4.9 years. Median disease duration was 5.5 months. Median follow-up was 24 months. Twelve patients had bilateral NVD; 14 had unilateral NVD. Inflammation-induced NVD was seen in 87% of the eyes, and ischemia-induced NVD in 13%. Initial treatment with high-dose corticosteroids combined with conventional immunosuppressive agents was effective in 45% of the eyes with inflammation-induced NVD. Retinal laser photocoagulation was effective in three of five eyes with ischemia-induced NVD. Treatment with interferon α-2a resulted in resolution of NVD in all seven patients who received this agent for inflammation- or ischemia-induced persistent NVD. Final visual acuity was less than 0.1 in 24% of the eyes.ConclusionsInflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behçets disease. The results of this study suggest that the response to intensive anti-inflammatory and conventional immunosuppressive treatment is not satisfactory; retinal laser photocoagulation may be ineffective even in eyes with retinal ischemia, but interferon α-2a seems to be effective for the treatment of Behçets patients with NVD. Jpn J Ophthalmol 2006;50:256–265

Clinical Features and Outcomes of HLA-B27-Positive and HLA-B27-Negative Acute Anterior Uveitis in a Turkish Patient Population

Purpose: To compare the clinical features and outcomes of HLA-B27-positive and HLA-B27-negative acute anterior uveitis (AAU) in a large Turkish patient population. Methods: Forty-four patients with HLA-B27-positive AAU and 65 with HLA-B27-negative AAU were reviewed retrospectively. HLA-B27 typing, gender, laterality, clinical features (fibrinous reaction, hypopyon formation), ocular complications, medical treatment, and final visual acuity were recorded. Statistical analyses included Students t-test, Fishers exact test, Yates corrected χ2 test, and Mann-Whitney U test. A p-value of < 0.05 was considered statistically significant. Results: Forty percent of all patients with AAU were found to be positive for HLA-B27 antigen. HLA-B27-positive AAU was significantly more common in males and HLA-B27-negative AAU in females. Unilateral or bilateral alternating AAU as well as fibrinous reaction and hypopyon formation were more common in HLA-B27-positive patients. Posterior iris synechiae and cataract formation were more common and systemic immunosuppressive agents were exclusively required in patients with HLA-B27-negative AAU. Visual outcome did not differ significantly between the HLA-B27-positive and HLA-B27-negative groups. Conclusions: HLA-B27 association is less common in Turkish patients with AAU than in other populations reported in the literature. Although distinct features of HLA-B27 uveitis were confirmed in our study, visual outcome did not differ from idiopathic AAU. Therefore, we believe that HLA-B27 typing in patients with AAU helps the clinician with the diagnosis. However, it has a limited value in determining the prognosis.

Bilateral acute depigmentation of the iris

PurposeTo report on five cases of unusual bilateral stromal depigmentation of the iris and pigment dispersion masquerading as uveitis.MethodsWe describe the clinical features of five consecutive patients who presented with acute depigmentation of the iris stroma between June and October 2003.ResultsFour patients were female, one was male. Age at presentation ranged from 15 to 25 years. Presenting symptoms were sudden-onset ocular discomfort and red eye in four patients and change of eye color in one patient. All patients had bilateral involvement, with a symmetrical diffuse depigmentation of the iris stroma in three cases and patchy areas of iris depigmentation in two. Other common features were mild ciliary injection (seven eyes), Krukenberg spindle (seven eyes), circulating pigment in the anterior chamber (eight eyes), and heavy pigment deposition in the angle (ten eyes). No eyes had iris transillumination defects, inflammatory keratic precipitates or inflammatory cells in the anterior chamber. Systemic laboratory work-up was unrewarding in all cases, and PCR analysis of the aqueous humor for HSV1 and 2 was negative in one patient. Four patients were treated with a short course of topical corticosteroids and three with oral acyclovir. One patient was lost to follow-up. The remaining four patients were followed up for 6–19 months with a stable clinical picture.ConclusionIn contrast to pigment dispersion syndrome, pigment seemed to be released from iris stroma in the five cases described here. Although patchy depigmentation of the iris resembled the lesions seen in herpetic iridocyclitis in two of the patients, symmetrical bilateral involvement and lack of intraocular inflammation were the differentiating features. The patients described here could represent a new entity or an unusual presentation of herpetic eye disease.

Immunopathologic Study of the Conjunctiva in Patients with Behçet Disease

PURPOSE Even though conjunctiva is not primarily involved in patients with uveitis due to Behçet disease, it may reflect the immunopathologic process when inflammation is induced by biopsy of conjunctiva, a phenomenon similar to the induced inflammation at skin pathergy sites. METHODS Conjunctival biopsy specimens obtained 48 hours after a 2-mm biopsy of the epibulbar conjunctiva in 26 Turkish patients with inactive ocular Behçet disease and 9 Turkish patients with inactive idiopathic uveitis were studied by immunoperoxidase using a panel of monoclonal antibodies: anti-CD1, -CD3, -CD4, -CD5, -CD14, -CD22, -CD25, and -CD67, HLA-DR, E-selectin, intercellular adhesion molecule-1 (ICAM-1), and vascular cell adhesion molecule-1 (VCAM-1). RESULTS Immunopathology of the conjunctival specimens obtained at the time of first biopsy was not significantly different between the Behçet disease and the idiopathic uveitis groups. The second-biopsy specimens of the patients with Behçet disease showed significantly greater numbers of T cells (CD3+, CD4+) and granulocytes (CD67+) as well as HLA-DR+ and ICAM-1+ cells in the substantia propria. Vascular endothelium of the conjunctiva in a patient with Behçet disease had significantly more pronounced expression of the adhesion molecules, E-selectin, and ICAM-1. None of the conjunctival specimens in either group showed VCAM-1 positivity. CONCLUSIONS These results show that a more intense antigen-independent inflammation develops with recruitment of both neutrophils and T lymphocytes of helper/ inducer phenotype in the conjunctiva of patients with Behçet disease in response to surgical trauma. Increased expression of E-selectin and ICAM-1 in the conjunctiva of patients with Behçet disease may suggest a critical role for these adhesion molecules in the initial events of inflammation.

Comparison of Presenting Features and Three-year Disease Course in Turkish Patients with Behçet Uveitis Who Presented in the Early 1990s and the Early 2000s

Purpose: To compare Behçet uveitis patients who presented in the 1990s and 2000s. Methods: Records of 170 patients seen in 1990–1994 and 258 patients seen in 2000–2004 were reviewed. Presenting features were analyzed in the whole cohort. Uveitis attacks, vision, complications, and immunomodulatory treatment (IMT) were compared in patients with 3-year follow-up. Results: Posterior segment involvement was less common, visual acuity was better, and more patients received IMT before referral in the 2000s. Follow-up revealed no significant difference in number of uveitis attacks in the first 3 years. Less eyes lost useful vision, no patient became legally blind, and fewer severe ocular complications occurred in the 2000s. IMT was started within 3 months in more patients and interferon alfa-2a was used only in the latter study period. Conclusions: The findings suggest a milder disease at referral, lower rate of severe complications, and a better 3-year visual outcome in patients who presented in the early 2000s.

Corneal sensitivity after penetrating keratoplasty

In order to determine corneal sensitivity after penetrating keratoplasty, 71 corneal transplants and their recipient beds were tested using the Cochet-Bonnet esthesiometer. The periods from surgery to examination were two weeks to 15 years. When the test results were divided into six groups according to the postoperative period, correlation analysis revealed a progressive improvement of sensitivity in the central and peripheral graft as well as in the recipient cornea. Only one graft had normal central sensitivity at 24 months; 36 grafts were completely anesthetic while 35 grafts had some level of sensitivity at the time of last clinical testing. Age, preoperative diagnosis or graft size were not correlated with the recovery of sensitivity. The extent and time of reinnervation in corneal grafts varied in individual patients.

Cytomegalovirus retinitis in a patient with Wegener's granulomatosis.

Cytomegalovirus retinitis (CMVR) is a rare complication of systemic immunosuppressive therapy in patients with rheumatic disorders. We describe a 44-year-old man with Wegener’s granulomatosis who was treated with cyclophosphamide and methylprednisolone and who subsequently developed bilateral CMVR. In spite of discontinuation of immunosuppressive therapy and administration of systemic ganciclovir, retinitis recurred and maintenance therapy was needed. In conclusion, increased awareness of this potentially serious complication of immunosuppressive therapy is critical. If immune recovery is slow after discontinuation of immunosuppressive agents, prolonged antiviral therapy is required in order to prevent recurrences of CMVR.