Aysel Gürler

Evaluation of clinical findings according to sex in 2313 Turkish patients with Behcet's disease

Background Behçets disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients.

The prevalence of Behçet's disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey.

PURPOSE The aim of this study was to determine the prevalence of Behçet’s disease above the age of 10 years by means of a population-based study. METHODS The epidemiological investigation (cross-sectional study) was made between May 1997 and May 1998 at the Park Primary Health Care Center, which is one of the education and research divisions of the Department of Public Health, Faculty of Medicine, Ankara University. The research aimed to cover all 17,256 (49.2% male, 50.8% female) inhabitants over 10 years of age living in this area. The screening team first surveyed and selected patients with recurrent aphthous stomatitis. These patients were further examined, free of charge, in the Preventive Ophthalmology Unit of the Public Health Center, at Ibni Sina Hospital’s Behçet Center or in other clinics if necessary. In this study the International Study Group For Behçet’s disease Criteria were used. RESULTS As the final result of the screening, 11 female and 5 male patients with Behçet’s disease were found (female/male = 2.2). These patients represented 9 already known and 7 newly diagnosed cases of Behçet’s disease. The prevalence of Behçet’s disease over 10 years of age is 0.11%. CONCLUSION The existing regional prevalance studies conducted in Turkey have indicated that the real number of Behçet’s patients in our country is markedly higher than the number of registered patients. Therefore the National Behçet’s Disease Commity and Surveillance System was founded by our research group in December 1999.

Plasma von Willebrand factor, tissue plasminogen activator, plasminogen activator inhibitor, and antithrombin III levels in Behçet's disease.

Sixty-three patients with Behçets disease (BD), 30 patients with recurrent oral ulcer and 30 healthy individuals as control group were included in the study. ISG criteria was used for the diagnosis of BD and patients were classified as active and inactive and evaluated accordingly. In the patient and control groups, von Willebrand factor (vWF), tissue plasminogen activator (tPA), and plasminogen activator inhibitor (PAI) levels were determined using ELISA method and antithrombin III (AT-III) by nephelometric methods. High levels of endothelial product, vWF in the active Behçet patient group (p < 0.005) supports endothelial destruction due to vasculitis related with BD. In the active patient group tPA levels were significantly lower (p < 0.05) than the inactive and control groups with higher levels of PAI (p < 0.05 and p < 0.01) respectively. In Behçet disease, besides the decrease in tPA synthesis, high PAI levels also can affect tPA decrease and lead to inhibition of fibrinolytic activity. In active Behçet group, levels of AT-III were low and no significant difference was observed in recurrent oral ulcer and control groups. This situation may arise from the excessive use of AT-III in active disease. In conclusion, high levels of vWF in Behçet patients is thought to arise from vasculitis and high levels of PAI from the accumulation of thrombocytes on the damaged surface of endothelium leading to a decrease in tPA levels and inhibition of fibrinolytic activity.

Elevated levels of soluble intercellular adhesion molecule-1 correlate with disease activity in Behçet's disease

The objective of this study was to measure soluble intercellular adhesion molecule-1 (sICAM-1) in patients with Behçets disease (BD) and to analyse the relationship of sICAM-1 levels with clinical and some laboratory measures of disease activity. Forty patients with BD fulfilling the International Study Group Criteria for the diagnosis of BD and 20 healthy controls were studied. Twenty patients had active, and 20 patients had inactive disease. Serum sICAM-1 was determined by a sandwich ELISA. The mean (±SD) sICAM-1 level was significantly higher in the whole BD group (297.3±86.6 ng/ml) than in the healthy controls (213±83.5 ng/ml; P< 0.05). The mean sICAM-1 levels in active and inactive BD patients were 315.7±76.3 ng/ml and 258.3±73.3 ng/ml, respectively. The mean sICAM-1 level in active patients was significantly higher than in inactive patients and healthy controls (P< 0.02 and P< 0.001, respectively). No statistically significant difference in mean sICAM-1 levels was found between inactive BD patients and healthy controls (P>0.05). There was no statistically significant difference between the mean sICAM-1 levels of active patients with (351.3±77.2 ng/ml) or without vascular lesions (292±68.8; P>0.05). In spite of a positive correlation between disease activity and both erythrocyte sedimentation rate and C-reactive protein (CRP; P< 0.01), we found no correlation between sICAM-1 and either of them (P>0.05). The elevated levels of sICAM-1 may be due to endothelial cell activation and/or damage or may be the result of inflammation. In either case it did not seem to be superior to more conventional measures of disease activity.

Comparison of Coagulation Parameters for Healthy Subjects and Behçet Disease Patients with and without Vascular Involvement

Behçet disease (BD) is an inflammatory illness that may attack several systems. Vascular involvement with thrombosis is one of the basic characteristics of this disease. The etiology of thrombosis tendency in BD has not been understood well. In this study, the role of primary coagulation parameters in patients with BD who have thromboembolic events was evaluated. In addition, the relation between the clinical activity of the disease and these parameters was compared. A total of 96 patients with a diagnosis of BD were studied at Ankara University, Medical School, Department of General Surgery and Behçet Center. BD patients were separated into two groups with respect to vascular involvement. Group A contained 22 patients with vascular attacks, and group B contained 74 patients without vascular attacks. In addition, 25 healthy persons comprised the control group (group C). The coagulation parameters assessed included protein C, protein S, antithrombin III activity, fibrinogen level, prothrombin time (PT), and partial thromboplastin time (PTT). There were no differences among the three groups with respect to protein C, protein S, fibrinogen, PT, and PTT values (p > 0.05). The antithrombin III value was found to be high in BD patients compared to those in the control group (p= 0.011). There was no difference regarding the antithrombin III value between BD patients with or without vascular attacks (p= 0.954). There was no correlation between the activity of disease and coagulation parameters (p > 0.05), but a positive correlation was seen between the duration of illness and vascular involvement. Fifty percent of patients who had had BD more than 8 years had vascular involvement (p= 0.002). We could not determine a specific pathology in relation to coagulation parameters that could explain the etiology of thrombosis in patients with BD. The elevated antithrombin III levels in patients with BD (compared to controls) may be due to a compensatory defense mechanism against increased procoagulant activity. There was a correlation between the duration of illness and vascular involvement.

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.

Adhesion molecule expression in erythema nodosum-like lesions in Behçet's disease A histopathological and immunohistochemical study

Abstract Behçets (BD) is a systemic inflammatory di-sease with histological evidence for vasculitis. Leucocyte-leucocyte and leucocyte-endothelial cell interactions are critical in inflammatory reactions that are influenced by the expression, activation and shedding of adhesion molecules. We investigated the expression of some adhesion molecules (E- and L-selectin, VLA-4, ICAM-1, PECAM-1, VCAM-1 and CD18 and CD11c chains of beta-2 integrins) on endothelial and inflammatory cells by immunohistochemistry on cryostat sections of erythema nodosum lesions taken from 15 patients with BD and 12 patients with erythema nodosum of unknown cause. Hematoxylin-eosin stained sections of all specimens were also assessed. The major histopathological findings were perivascular mononuclear cell infiltration and secondary vasculitic changes with no difference between the two groups (P>0.05). However, the frequency of thrombophlebitis was higher in BD (P<0.001). Endothelial and inflammatory cell adhesion molecule expression did not show any significant diffe-rence between groups (P>0.05). Although VCAM-1 expression and intensity on endothelial cells of BD patients seemed to be lower, this did not reach statistical significance (P=0.056). We concluded that subcutaneous thrombophlebitis is an important feature of erythema nodosum like lesions in BD, which is almost impossible to understand by physical examination alone. Colchicine, which is known to have some influence on adhesion molecules, might have affected our results, as these showed no signi-ficant difference regarding adhesion molecules between the two groups.

Thrombomodulin levels in Behçet's disease with and without the factor V Leiden mutation

The plasma levels of thrombomodulin (TM) in 34 patients with Behçets disease and 79 healthy control subjects were studied. Eight patients had the factor V Leiden (FVL) mutation. The TM level was significantly lower in patients with the FVL mutation than in patients without the mutation and in the healthy controls (p<0.05 andp<0.01). However, there was no difference in overall mean plasma TM concentration between the patients without the mutation and the healthy controls.

Fibronectin and circulating immune complexes in Behçet's disease

Being a high-molecular-weight adhesive glycoprotein, fibronectin (Fn) is suggested to be a component of immune complexes and may participate in the clearance of immune complexes. In Behçets disease (BD), a multisystem disorder of unknown etiology, immune complexes have been shown to be deposited in affected tissue during disease activity, suggesting an immune mechanism. This study investigates the relationship between Fn and circulating immune complexes (CIC) and evaluates the changes in the levels of Fn and CIC along with disease activity. In 63 patients (31 active, 32 inactive) with BD, plasma Fn and serum CIC, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and the third and fourth components of the complement system (C3, C4) were studied. The mean ESR, CRP, C3 and C4 levels of active BD patients were found to be significantly higher than those of the inactive BD patient group. Although the mean Fn and CIC levels of BD patients were not significantly different from those of the healthy control group, Fn and CIC values of active BD patients were significantly lower than in the inactive group. Moreover, no significant correlation was observed among Fn levels and ESR, CRP, C3, C4 and CIC levels in BD patients. The result of this study suggest that the variation in Fn concentration is independent of the acute-phase response. The lack of relationship between the CIC and Fn concentrations indicates that IC deposition in the vessel wall is independent of the CIC levels. In order to determine the exact roles of Fn and IC, further studies in tissue specimens are required.

Isotretinoin treatment in nodulocystic acne with and without polycystic ovary syndrome: efficacy and determinants of relapse

Background  Isotretinoin is the most efficacious long‐lasting treatment for acne; however, some factors, including polycystic ovary syndrome (PCOS), patient age, family history, and type and number of acne lesions, may lead to treatment resistance or relapse following treatment. The aim of this study was to compare the efficacy and permanence of systemic isotretinoin (SI) in nodulocystic acne patients with and without PCOS and to evaluate the factors associated with relapse during the first and second post‐treatment years.