F. Nilüfer Yalçındağ

Behçet Disease in Children

Purpose: To investigate the incidence and clinical characteristics of Behçet disease in children. Methods: The authors retrospectively reviewed the charts of 3382 patients with Behçet disease from October 1986 to December 2005 at Ankara University Medical School Behçet Unit and/or Atmaca private clinic. Results: 110 children were diagnosed with Behçet disease. The 110 children represented 3.3% of the total number of Behçet patients that were reviewed. 62.7% were girls and 37.3% were boys. The mean age at initial manifestation was 11.63 ± 3.46 years and at diagnosis was 14.15 ± 2.13 years. Ocular involvement was found in 30.9% of the children. Of these, 61.8% were bilateral and 38.2% were unilateral. Anterior uveitis was found in 18 eyes (32.8%), posterior uveitis in 24 eyes (43.6%), and panuveitis in 13 eyes (23.6%). Conclusion: Behçet disease should be considered in the differential diagnosis of childhood uveitis, particularly in endemic areas. The collaboration of pediatricians, rheumatologists, dermatologists, ophthalmologists, and other specialists when necessary is required in the diagnosis and management of children with Behçet disease.

Results of Interferon Alpha-2a Therapy in Patients with Behcet's Disease

PURPOSE Corticosteroids, the main treatment option in acute attacks of ocular Behcets disease (BD), are ineffective in preventing recurrent inflammation. Even combined therapy with systemic corticosteroids and azathioprine, cyclosporine, chlorambucil, or cyclophosphamide may be ineffective in controlling the disease in severe cases. We report our clinical experience with the efficacy and tolerability of interferon alpha-2a (IFN-α-2a) monotherapy in patients with Behcet uveitis refractory to conventional immunosuppressive agents. METHODS We retrospectively analyzed the medical records of 12 Turkish Behcet patients who had been treated with IFN-α-2a between February 2009 and October 2011 because of severe uveitis refractory to traditional immunosuppressants. IFN-α-2a was initially administered at 4.5×10(6) IU/day subcutaneously; then, the dosage was tapered gradually depending on the clinical response. Previous immunosuppressive drugs such as systemic corticosteroids, azathioprine, and cyclosporine were stopped 1 day before the initiation of IFN-α-2a treatment. RESULTS The mean duration of IFN-α-2a use was 13.3 months (range: 3-28 months). Eighty-three percent of patients with ocular BD achieved partial or complete response. Best-corrected visual acuity improved or remained stable in all of patients. All patients experienced flu like symptoms responding to oral acetaminophen, hair loss was also developed in 7 of them (58%), and psoriatic skin rash was seen in 3 patients (25%) during treatment with IFN-α-2a. No other side effects were observed. CONCLUSIONS IFN-α-2a treatment is effective in Behcet uveitis refractory to traditional treatment regimens and provides significant improvement in visual prognosis.

Soluble endothelial protein C receptor levels in Behçet patients with and without ocular involvement

BackgroundThe protein C system is an important natural anticoagulant mechanism. Endothelial cell-activated protein C receptor (EPCR), which was discovered at the surface of endothelial cells, binds protein C and enhances its activation. The soluble form of EPCR (sEPCR) has been detected in plasma. Behçet’s disease is a chronic inflammatory disorder affecting multiple organs. Arterial and venous thrombosis is a common clinical manifestation of Behçet’s disease and the pathogenic mechanism of thrombotic tendency in the disease is not well known. The aim of this study is to determine sEPCR concentrations in Behçet patients with and without ocular involvement as well as to investigate the association between sEPCR levels and clinical manifestations of Behçet’s disease.MethodsSixty patients with Behçet’s disease and 67 healthy control subjects were included in this study. A complete ophthalmic examination was performed by ophthalmologists with an interest in Behçet’s disease. Sixty patients with Behçet’s disease were divided into two groups. Group 1 consisted of 30 patients with ocular involvement and Group 2 consisted of 30 patients without ocular involvement. Soluble EPCR levels were determined in plasma by using sEPCR Asserachrom enzyme-linked immunosorbent assay (ELISA) kits according to the manufacturer’s instructions. Differences of the mean sEPCR levels between groups were evaluated using Mann-Whitney U-test. Pearson’s correlation analysis was used for evaluating the correlation between sEPCR levels and age, gender, duration of the disease as well as different clinical manifestations of Behçet’s disease.ResultsAge and gender ratio were not different between patients and controls. Plasma sEPCR concentrations were significantly higher in patients with Behçet’s disease than those in controls (p<0.05). There was no statistically significant difference in serum sEPCR levels between the patients with versus the ones without ocular involvement. There were no statistically significant correlations between sEPCR levels and age, gender, duration of the disease or clinical manifestations.ConclusionsOur data suggests a possible role of soluble EPCR in the pathogenesis of Behçet’s disease. Further studies by possible mutations and polymorphisms in EPCR gene in patients with Behçet’s disease would be useful to bring to light the pathogenic mechanism of ocular and systemic vascular complications of the disease.

Evaluation of serum resistin levels in patients with ocular and non-ocular Behçet’s disease

BACKGROUND Resistin, a recently identified adipocytokine, has been found to play an important role in inflammation and the processes of inflammation-related diseases. Serum resistin levels in patients with Behçets disease (BD) have not yet been investigated. We aimed to evaluate the relation between resistin and interleukin-6 (IL-6) in Behçet patients with or without ocular involvement and in normal controls. METHODS Twenty-two patients with BD and 19 healthy control subjects were included in this study. While 14 patients had posterior segment involvement of the eye, the other 8 did not have ocular disease. Serum resistin and interleukin-6 (IL-6), levels were measured in all samples. Data from all groups were tested for statistical significance. RESULTS The mean resistin and IL-6 concentrations were significantly higher in patients with BD than the control subjects (p = 0.011 and p = 0.0001, respectively). There was a significant difference in resistin and IL-6 levels between the patients with non-ocular BD and controls (p = 0.013 and p = 0.0001, respectively), as well as resistin and IL-6 levels between the ocular BD group and the control group (p = 0.05 and p = 0.0001, respectively). However, there was no significant difference between patients with ocular versus non-ocular BD. INTERPRETATION Resistin levels were found to be raised in Behçet patients with or without ocular involvement compared with the control subjects.

Serum Leptin Levels in Patients with Ocular and Nonocular Behçet's Disease

Aims. To investigate serum leptin levels in Behçets patients with or without ocular involvement compared with healthy subjects and the relationship between serum leptin and uveitis activity in patients with ocular involvement. Methods. Fifty-seven patients with Behçets disease and 20 healthy control subjects were included in this study. While 27 patients had ocular involvement (18 had acute uveitis, 9 had inactive ocular involvement), 30 did not have ocular disease. C-reactive protein, alpha 1-antitrypsin, and serum leptin levels were measured in all samples. Results. There was a significant difference between the patients with Behçets disease and control group for both logarithm of leptin (P = .000) and logarithm of CRP (P = .031). Logarithm of leptin in non-ocular Behçets patients was significantly higher compared to its level in ocular Behçets disease and controls (P = .009). There was a significant difference between the patients with active ocular disease and control group (P = .03). Conclusions. Leptin might have a possible role in the pathogenesis of Behçets disease.

Intron F G79A Polymorphism of the Protein Z Gene in Turkish Behçet Patients

Purpose: Vasculitis is one of the major findings of Behçet’s disease (BD). Protein Z (PZ) is a glycoprotein that acts as a cofactor of PZ-dependent protease inhibitor and suppresses trombus formation by inhibiting activated factor Xa. Polymorphism of the PZ gene was mentioned as a genetic risk factor for various thrombotic events. The aim of this study is to investigate the intron F G79A polymorphism of the PZ gene in Behçet patients with and without ocular involvement. Methods: Seventy-six patients and 70 controls were included in the study. Intron F G79A polymorphism of PZ gene was determined by polymerase chain reaction based DNA analysis. The frequency of A allele and the distribution of genotypes were assessed by χ2 test and the genotype distribution and Hardy–Weinberg equilibrium were tested with the χ2 test for quality of fit. Results: The frequency of the A allele was significantly higher in overall Behçet patients than in controls (odds ratio [OR] = 6.8; 95% CI, 2.6 to 17.9; p = 0.0001). It was also significantly higher in patients with (OR = 5.3; 95% CI, 1.83 to 15.6; p = 0.0024) or without (OR = 8.2; 95% CI, 2.95 to 22.5; p = 0.0001) ocular involvement compared to controls. However, A allele frequency was not significantly different between patients with eye involvement versus patients without eye involvement (OR = 0.65; 95% CI, 0.3 to 1.4; p = 0.28). Conclusions: Although thrombosis in BD is multifactorial, intron F G79A polymorphism of PZ gene in BD may be one of the factors that contribute to this pathological process.

Pathergy-Like Reaction Following Intravitreal Triamcinolone Acetonide Injection in a Patient with Behçet Disease

Purpose: To describe a patient with Behçet disease who developed a pathergy-like reaction following intravitreal triamcinolone acetonide injection. Methods: Case report. Results: A 30-year-old man with a history of Behçet disease received an intravitreal injection of triamcinolone acetonide for the treatment of cystoid macular edema. The following day, he was noted to have erythematous swelling around the injection site. The lesion disappeared spontaneously within 2 weeks. Conclusion: Pathergy-like reaction to minor trauma may develop not only on the skin but also in the mucous membranes such as conjunctiva in patients with Behçet disease.

Demographic and Clinical Characteristics of Uveitis in Turkey: The First National Registry Report.

ABSTRACT Purpose: To describe the demographic and clinical profiles of uveitis patients seen at secondary and tertiary care centers in Turkey. Methods: A nationwide web-based registry of patients with uveitis was initiated in November 2008. We analyzed data from a single baseline registry-enrollment visit. Results: In 33 centers, 6967 eyes of 4863 consecutive patients were registered. The mean age at presentation was 36.6 ± 15.7 (1–92) years; 51.3% were male. Behçet disease was the leading diagnosis (24.9%), followed by ankylosing spondylitis and/or HLA-B27-associated anterior uveitis (9.7%), toxoplasmosis (7.1%), Fuchs uveitis (6.3%), and presumed herpetic anterior uveitis (6.0%). Visual acuity was 0.1 or worse in 22% of eyes. The most common ocular complications were posterior synechiae (16.2%) and cataract formation (16.2%). Conclusions: Behçet disease is still the most common non-infectious etiology in Turkish uveitis patients, while ocular toxoplasmosis and herpetic anterior uveitis are the most common infectious uveitic entities.

Urokinase plasminogen activator receptor levels in Behcet's disease.

PURPOSE To determine urokinase plasminogen activator receptor (uPAR) concentrations in Behcet patients with and without ocular involvement; and to investigate the associations between uPAR levels and clinical manifestations of Behcets disease. METHODS Sixty-four patients with Behcets disease (31 patients with and 33 patients without ocular involvement) and 23 healthy control subjects were included in this study. A complete ophthalmologic examination was performed. Venous blood was collected from all patients and control subjects. Serum uPAR levels were determined by using human uPAR immunoassay (Quantikine) kits. RESULTS There was no statistically significant difference in serum uPAR levels between the patients and the control subjects (p>0.05). There were no statistically significant correlations between uPAR levels and age, gender, duration of the disease, clinical manifestations (genital ulcer, arthritis, skin lesions, ocular and vascular involvements) and activity of the disease. CONCLUSION This finding is important since this is the first study regarding uPAR levels in Behcets disease.

Optical Coherence Tomography Findings of Retinal Folds in Nanophthalmos

Aim. To report the optical coherence tomography (OCT) findings in three members of the same family with nanophthalmos associated with elevated papillomacular retinal fold. Methods. Complete ophthalmic examination as well as ultrasonography and OCT was performed in all patients. Results. Axial lengths ranged from 16.75 mm to 17.48 mm and refractive errors ranged from +17.50 D to +20.50 D. Main fundus findings were the hyperopic crowded, cupless optic disc, and retinal fold through papillomacular region. Macular OCT scans revealed retinal fold with normal retinal pigment epithelium and choriocapillaris. Interpretation. It is presumed that the retinal folds in nanophthalmos result from a redundancy of the retinal layer caused by retarded growth of the scleral, choroidal, and retinal pigment epithelial layers. The anatomic information provided by the current study is consistent with this thesis.