Results of intravitreal triamcinolone acetonide (IVTA) injection for the treatment of panuveitis attacks in patients with Behçet disease.

AIM: The aim of this study was to report our experience on an intravitreal triamcinolone acetonide (IVTA) injection for the treatment of unilateral severe panuveitis attacks in patients with Behçet disease (BD). PATIENTS AND METHODS: We reviewed the medical records of 18 eyes of 15 consecutive patients with BD who received an IVTA injection. RESULTS: The indication for an IVTA injection was severe panuveitis attacks in 15 patients who were unresponsive or intolerant to systemic medications. Intraocular inflammation resolved after a mean period of 25 days following the IVTA injection. During a mean followup period of 28 months (range, 9-50), all eyes showed improvement in visual acuity (mean, 0.71 logMAR units). Twelve (12) eyes (66.6%) had an elevation of intraocular pressure (IOP). Cataract progression or new cataract formation was detected in 10 eyes (55.5%). Four (4) eyes (22%) had relapses 10-28 months (mean, 19) after the IVTA injection. CONCLUSIONS: The IVTA injection may be used as an adjunct therapy for the treatment of panuveitis attacks in patients with BD who are unresponsive or intolerant to systemic medications. Although it hastens the resolution of attacks and seems to have a long-term, anti-inflammatory effect, relapses can occur in the long term. It is also associated with a high rate of IOP elevation and cataract progression in this group of patients.

Neovascularization of the optic disc in Behçet's disease.

PURPOSE: To investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behçet's disease and to evaluate the effects of medical and laser treatments. METHODS: This is a retrospective study of 26 consecutive Behçet's 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. RESULTS: Eighteen 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 alpha-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. CONCLUSIONS: Inflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behçet's 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 alpha-2a seems to be effective for the treatment of Behçet's patients with NVD.

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 Student's t-test, Fisher's exact test, Yate's corrected Chi2 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.

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.

Uveitis in Behçet disease: an analysis of 880 patients.

PURPOSE: We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Behçet uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990. DESIGN: Observational case series. METHODS: A retrospective study of 880 consecutive patients (1,567 eyes) with Behçet uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Behçet's Disease. Information on the patient's sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected. RESULTS: Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s. CONCLUSION: Behçet uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s.

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.

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

PURPOSE: To assess the usefulness of laser flare-cell photometry to quantify intraocular inflammation in patients with Behçet disease. METHODS: The 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. RESULTS: Mean 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 < or =6 photons/msec (right eyes, P < 0.001; left eyes, P = 0.0184). CONCLUSIONS: Laser 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.

Bilateral acute depigmentation of the iris.

PURPOSE: To report on five cases of unusual bilateral stromal depigmentation of the iris and pigment dispersion masquerading as uveitis. METHODS: We describe the clinical features of five consecutive patients who presented with acute depigmentation of the iris stroma between June and October 2003. RESULTS: Four 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. CONCLUSION: In 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.

Results of interferon-alfa therapy in patients with Behçet uveitis.

PURPOSE: To report on the results of interferon-alpha 2a (IFNalpha) treatment in patients with Behçet uveitis unresponsive to conventional immunosuppressive therapy. METHODS: We retrospectively analyzed the medical records of 44 patients who had been treated with IFNalpha between September 2001 and May 2005. The initial dose of IFNalpha was 6 MU/day in 37 patients (84.1%) and 3 MU/day in 7 patients (15.9%), and was gradually tapered after ocular inflammation was suppressed. Immunosuppressive agents were discontinued. Oral corticosteroids were discontinued or maintained at a dosage of less than 10 mg prednisone equivalent per day. Main outcome measures were recurrence of posterior or panuveitis attacks and changes in visual acuity. RESULTS: Sixteen patients (36.4%) remained relapse free during treatment, whereas 28 (63.6%) patients had recurrent uveitis attacks. Four of these were considered treatment failures and were switched to other treatments. In the remaining 40 patients, the mean duration of treatment was 12.4+/-10.8 months (range 3-45 months). In 9 of 40 patients (22.5%) treatment could be discontinued 22.2+/-13.4 months after therapy, and 8 (20%) of these patients had sustained remission for up to 24 months. Three patients (7.5%) were switched to other therapies because of side effects. The frequency of uveitis attacks per 6 months was reduced from 1.6+/-1.2 to 0.8+/-0.9 in 26 patients who had a minimum follow-up of 6 months before and after IFNalpha therapy (p<0.05). There was a significant improvement in visual acuity and this was preserved throughout follow-up in 38 (95%) of 40 patients. CONCLUSIONS: A partial or complete response was obtained with IFNalpha therapy in 91% of Turkish patients with Behçet uveitis refractory to conventional immunosuppressive treatment. Our results suggest that there may be differences in therapeutic efficacy and side-effect profile of IFNalpha in different patient populations. Comparative studies are needed to investigate this hypothesis.

Active ocular toxoplasmosis in Turkish patients: a report on 109 cases.

BACKGROUND: To describe the clinical characteristics of active ocular toxoplasmosis in a large population of Turkish patients. METHODS: A retrospective study of 109 consecutive patients with active ocular toxoplasmosis seen at the Department of Ophthalmology, Istanbul Faculty of Medicine, from 1995 to 2005. RESULTS: Fifty-seven patients were female and 52 were male. The mean age at presentation was 25.7 +/- 6.8 years. All patients had positive serum anti-toxoplasma IgG antibodies, but negative IgM antibodies. Preexisting retinochoroidal scars were found in 90 (83%) patients. Central active lesions were significantly more common in eyes without previous involvement than in eyes with preexisting scars (97% vs. 59%). Active lesions were adjacent to a scar in 60 (78.9%) of 76 eyes with preexisting scars. The most common accompanying signs were vitritis (100%), anterior uveitis (49.5%), and periphlebitis (33%). All patients received antiparasitic treatment. Systemic corticosteroids were used in 86% of the patients. Kaplan-Meier survival analysis estimated the cumulative risk of recurrence as 74% at 42 months of follow-up. In 29 (80.5%) of 36 recurrent attacks, active lesions were associated with the scars of the most recent attack. After the resolution of the presenting attack, visual acuity was better than 0.5 in 90%, between 0.1 and 0.5 in 5%, and less than 0.1 in 5% of eyes. Further decrease in visual acuity occurred in only two eyes during our follow-up. CONCLUSIONS: Most of the patients with active ocular toxoplasmosis have asymptomatic retinochoroidal scars. Proximity of active lesions to the scars, and especially to those of the most recent episode, may have implications for treatment. Although the recurrence risk is high, the visual prognosis is good in most patients with typical ocular toxoplasmosis.

Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behçet's disease: an open-label trial.

OBJECTIVE: To investigate the effect of infliximab on the frequency of uveitis attacks and the visual prognosis in male patients with Behçet's disease in whom uveitis was resistant to combination therapy with corticosteroids, azathioprine, and cyclosporine. METHODS: The study group comprised patients who were receiving combination therapy but experienced at least 2 attacks of posterior uveitis/panuveitis or retinal vasculitis during the 6-month period prior to enrollment. Infliximab infusions (5 mg/kg) were administered at weeks 0, 2, 6, and 14. Weeks 0-22 were defined as the infusion period, and weeks 23-54 were defined as the observation period. Patients continued to receive azathioprine and corticosteroids, but cyclosporine was discontinued after the screening visit. The primary outcome measures were the absence of uveitis attacks during the infusion period (remission), and the absence of uveitis attacks throughout the study period (sustained remission). RESULTS: Thirteen patients were enrolled in the study. Thirty-two uveitis attacks involving the posterior segment occurred during the previous-treatment period. During the infusion period, 4 patients (30.8%) remained attack-free, and 9 patients had a total of 13 uveitis attacks. Ten of these attacks (76.9%) occurred at either week 14 or week 22. One of 13 patients fulfilled the definition of sustained remission, and the remaining 12 patients had a total of 36 uveitis attacks during the observation period. The mean number of uveitis attacks and daily corticosteroid doses were significantly lower during the infusion period than during the previous-treatment period or the observation period. Although potential visual acuity was regained following infliximab infusion, this beneficial effect was not preserved until week 54. None of the patients experienced a serious adverse event. CONCLUSION: The results of this trial suggest that infliximab is effective in suppressing the occurrence of uveitis attacks, has a corticosteroid-sparing effect, and has favorable implications for the visual prognosis of patients with resistant Behçet's uveitis.

Prevalence of Behçet's disease in Istanbul, Turkey.

BACKGROUND: The prevalence of Behçet's 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. PATIENTS AND METHODS: This study investigated the cross-sectional prevalence of BD in individuals aged > 12 years in Istanbul, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) by visiting them in their homes, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The sample size was determined to be 24,000 with an expected BD prevalence rate of 1/1000 and a sampling error of 4/10,000, with a 95% confidence interval (CI) of 6-14/10,000. The number of individuals to be screened in each district was determined in proportion to the population of all districts in Istanbul. RESULTS: The standard questionnaire was applied to a total of 23,986 individuals at their homes. A history of ROU was recorded in 2289 individuals (9.5%), and a previous diagnosis of BD was recorded in 47. The diagnosis of ROU was confirmed in 700, and the diagnosis of BD was established in 101 according to the International Study Group criteria. The prevalence rate of BD was estimated as 42/10,000 (95% CI, 34-51/10,000) in Istanbul, Turkey. CONCLUSIONS: This survey conducted in Istanbul, the largest cosmopolitan city in Turkey with immigrants from all over the country, has a larger sample size than other previous studies, and therefore the reported prevalence rate of BD has a more acceptable confidence interval. This study aids in the estimation of the prevalence of BD in Turkey, and supports previous findings that Turkey has the highest prevalence rate of the disease in the world.