Caspase-9 expression is increased in endothelial cells of active Behçet's disease patients

Background Behçet's disease is a multisystem disease of unknown etiology. Caspase‐9 is responsible for initiating the caspase activation cascade during apoptosis. The aim of this study was to examine caspase‐9 expression in both endothelial and perivascular infiltrates of patients with active Behçet's disease. Methods Fifteen patients with active Behçet's disease, attending the First Dermatology Department, Ankara Numune Hospital, Ankara, Turkey between June 2003 and December 2005, were included in the study. Oral biopsy specimens from nine healthy volunteers were taken as the healthy control group, and skin biopsies from 18 psoriasis patients were used as the inflammatory control group. The specimens were examined with caspase‐9 primary antibody. Statistical analyses were performed using SPSS 11.5. Results The mean caspase‐9‐positive endothelial cell counts were 7.17 ± 2.45 in active Behçet's disease, 4.81 ± 0.76 in healthy controls, and 4.35 ± 1.34 in inflammatory controls. The difference between Behçet's disease and healthy controls was statistically significant, with increased endothelial staining in active Behçet's disease (P = 0.049). The difference between Behçet's disease and inflammatory controls was also statistically significant; the rate of staining was higher in Behçet's disease (P = 0.006). The mean caspase‐9‐positive dermal perivascular cell counts were 5.15 ± 2.32 in Behçet's disease, 3.32 ± 0.82 in healthy controls, and 5.54 ± 4.95 in inflammatory controls. These values did not show any statistically significant difference (P = 0.407). Conclusion Endothelial cells are one of the key cells in Behçet's disease, and our findings support the role of endothelial cells in the etiopathogenesis of Behçet's disease.     

Evaluation of pro-atherogenic lipid profile and high atherohenic indexes in patients with Behçet's disease: A case–control study

Background

Behçet's disease is a systemic auto-immune and auto-inflammatory chronic disease in which genetic and environmental factors play a role. Patients with Behçet's are at significant risk for developing many comorbidities, including cardiovascular diseases.

Aims

It was aimed to investigate the relationship between serum lipid parameters and atherogenic indexes to evaluate the cardiovascular risk status in patients with Behçet's disease.

Patients/Methods

This study was designed as a single-center, retrospective case–control study. The study was conducted with 212 patients over 18 years of age, 106 in the case group and 106 in the control group.

Results

There was a significant difference in lipid values between the patients with Behçet's disease and the control group. While the serum triglyceride, total cholesterol, low-density lipoprotein cholesterol (LDL-C), and non-high-density lipoprotein cholesterol (NHC) levels were significantly higher, the high-density lipoprotein cholesterol (HDL-C) level was low in patients with Behçet's disease. From atherogenic indexes, Atherogenic Index of Plasma (AIP) (0.03 ± 0.27 vs. −0.07 ± 0.23, p = 0.003), Castelli Risk Index I (CRI-I) (4.24 ± 1.07 vs. 3.02 ± 0.96, p < 0.001), Castelli Risk Index II (CRI-II) (2.65 ± 0.81 vs. 1.84 ± 0.59, p < 0.001) and Atherogenic Coefficient (AC) (3.24 ± 1.07 vs. 2.02 ± 0.96, p < 0.001) levels were significantly higher in patients with Behçet's disease.

Conclusion

Our study shows that patients with Behçet's have a higher pro-atherogenic lipid profile and atherogenic indexes at high risk. Patients with Behçet's have an increased risk of cardiovascular diseases associated with atherosclerosis.     

Serum galectin‐3 levels in patients with Behçet’s disease: association with disease activity over a long‐term follow‐up

Background There is a need for a laboratory marker that correlates with the clinical activity of Behçet’s disease (BD). Objective We aimed to investigate whether serum galectin‐3 (Gal‐3) levels were affected during the course of the disease with regard to disease activity. Methods A total of 131 subjects were involved in the study as follows: Group 1: BD active (n = 39); Group 2: BD inactive (n = 31); Group 3: Disease controls with leucocytoclastic vasculitis confirmed with a skin biopsy (n = 22); and Group 4: Healthy control subjects (n = 39). The BD patients were followed regularly and samples were taken in their active and inactive periods of the disease over a 2‐year period. Results Serum Gal‐3 levels were significantly higher in active BD patients (mean 2.38) than inactive BD patients (mean 0.63; P < 0.0001) and the healthy control subjects (mean 0.75; P < 0.0001). There was no significant difference between the leucocytoclastic vasculitis and active BD patients (P = 0.093). Serum Gal‐3 levels were positively correlated with clinical activity scores of active BD patients (r = 0.66, P < 0.0001). In addition, the Gal‐3 levels were significantly higher in the active disease period when compared with the inactive period during the follow‐up. There were no significant differences between the two inactive periods of the disease among the same patients. Further analyses revealed that patients with vascular involvement had significantly higher Gal‐3 levels than the other active BD patients (mean 7.57; P = 0.007). Limitations The limitation of the study is the small number of patients with vascular involvement in the active BD patient group. Conclusion Gal‐3 levels are correlated with the activity of Behçet’s disease especially with the vascular involvement.     

Adiponectin levels,insulin resistance and their relationship with serum levels of inflammatory cytokines in patients with Behçet’s disease

Aim Increased frequency of cardiovascular disease and its possible relations with insulin resistance have been reported in patients with inflammatory diseases. The aim of our study was to investigate insulin resistance and serum adiponectin levels as cardiovascular risk markers in patients with Behçet’s disease. Method Study population consisted of 40 patients with Behçet’s disease (BD) and a control group composed of age, gender, body mass index‐matched 46 healthy individuals. All patients were examined for signs of Behçet’s disease. Body mass index, waist and hip circumference were measured. Insulin resistance was evaluated using the homeostasis model assessment‐insulin resistance method. Erythrocyte sedimentation rate (ESR), lipid profile, high sensitive CRP (hsCRP), adiponectin, TNF‐α, IL‐6 and IL‐8 levels were measured. Results Erythrocyte sedimentation rate, serum hsCRP and IL‐6 levels were significantly higher in patients with BD than those in the controls (P = 0.001, P = 0.001, P = 0.001, respectively). Fasting plasma glucose, insulin levels and lipid profile were not different between the two groups. Insulin resistance and decreased levels of the serum adiponectin were not detected in the patients. There was no relationship between insulin resistance, adiponectin levels and inflammatory markers. Active and inactive patients did not differ in respect of any parameters. Conclusion Being a systemic vasculitis, BD may cause cardiovascular involvement. In this study, dyslipidemia, insulin resistance and low adiponectin levels were not detected among our patients with Behçet’s disease. Our results suggest that there exists no increased risk for atherosclerotic cardiovascular disease associated with adiponectin levels and insulin resistance in patients with Behçet’s disease.     

Vascular endothelial growth factor levels are increased and associated with disease activity in patients with Behçet's syndrome

Background/aims Vascular endothelial growth factor (VEGF) is a cytokine participating in inflammation with potent endothelial cell effects. It is produced by macrophages, neutrophils and vascular endothelial cells and can alter vessel permeability. Behçet's syndrome is a systemic inflammatory disorder with unknown etiology. Vascular endothelial dysfunction is one of the prominent features of the disease. We previously demonstrated the possible involvement of proinflammatory cytokines [tumor necrosis factor (TNF)‐α, soluble interleukin‐2 receptor (sIL‐2R), interleukin (IL)‐6 and IL‐8], nitric oxide (NO) and adrenomedullin in the etiopathogenesis of Behçet's syndrome. Since VEGF expression is induced by these cytokines and VEGF itself is a potent stimulator of NO production with endothelial cell effects, this study aimed to investigate whether VEGF was affected during the course of Behçet's syndrome. We also assessed the possible involvement of VEGF in ocular Behçet's syndrome or in disease activity. Methods This multicenter case–control study included a total of 39 patients with active (n = 22) or inactive (n = 17) Behçet's syndrome (mean age, 38.1 ± 10.4 years; 21 men and 18 women) satisfying International Study Group criteria, and 15 healthy hospital‐based control volunteers (mean age, 39.2 ± 9.3 years; eight men and seven women) matched for age and gender from a similar ethnic background. Patients were examined by a dermatologist and an ophthalmologist with an interest in Behçet's syndrome. Plasma VEGF concentrations were measured using a newly established enzyme‐linked immunosorbent assay. Clinical findings and acute‐phase reactant parameters such as erythrocyte sedimentation rate, α₁‐antitrypsin, α₂‐macroglobulin, and neutrophil count were used to classify the disease in Behçet's patients as active or inactive. The Wilcoxon test or the Mann–Whitney U‐test was used for statistical analysis as indicated and the results were expressed as mean ± SD, with range. Results The mean plasma VEGF level in patients with Behçet's syndrome (291.9 ± 97.1 pg/mL; range 121–532 pg/mL) was higher than that in control subjects (103.0 ± 43.6 pg/mL; range 25–187 pg/mL) and the difference was significant (P < 0.001). Patients with active disease had significantly (P < 0.001) higher VEGF levels than patients with inactive disease (347.6 ± 87.1 vs. 219.9 ± 51.6 pg/mL). In addition, ocular Behçet's patients (n = 23) had higher VEGF levels (315.7 ± 92.1 pg/mL) than nonocular patients (n = 16, 257.8 ± 96.6 pg/mL) and the difference was of borderline significance (P = 0.041). The levels of all acute‐phase reactant parameters were significantly higher in the active stage than in the inactive stage (for each, P < 0.01) or in control subjects (for each, P < 0.001). Conclusions VEGF may participate in the course of Behçet's syndrome, especially in the active stage, and elevated levels of VEGF may be an additional risk factor for the development of ocular disease, contributing to poor visual outcome.     

Serum leptin concentration is increased in patients with Behçet's syndrome and is correlated with disease activity

Summary Background Behçet's syndrome is a systemic, relapsing immuno‐inflammatory disease with a generalized vasculitis of the microvasculature endothelial dysfunction. Leptin, a recently discovered neuroendocrine hormone, is a metabolic peptide that appears to be involved. Serum proinflammatory cytokines upregulate leptin levels and leptin itself directly induces nitric oxide production from endothelial cells with its specific receptors. Objectives To detect changes of serum leptin concentrations in patients with Behçet's syndrome compared with age‐ and sex‐matched healthy volunteers by using enzyme‐linked immunosorbent assay. We also investigated whether disease activity or the duration of Behçet's syndrome correlates with leptin concentration. Methods Thirty‐five consecutive patients with Behçet's syndrome (41·2 ± 8·4 years, 16 male, 19 female) and 20 age‐ and sex‐matched healthy control subjects (40·4 ± 10·91 years, nine male, 11 female) were included in this study. The body mass index (BMI) [weight (kg) height^?1 (m²)] was calculated for subjects at study enrolment. We measured serum leptin with a leptin enzyme immunoassay kit, and acute‐phase reactants, including erythrocyte sedimentation rate, α₁‐antitrypsin, α₂‐macroglobulin and neutrophil count. The Mann–Whitney U‐test was used for statistical analysis and P < 0·05 was considered significant. Values were expressed as mean ± SD. Results The gender ratio, age and BMI were not substantially different among Behçet's patients and controls. The mean serum leptin concentrations in patients with Behçet's syndrome (16·8 ± 7·49 ng mL^?1) were significantly (P < 0·001) higher than in healthy control volunteers (7·5 ± 2·77 ng mL^?1). Active Behçet's patients had significantly (P = 0·001) higher leptin concentrations (20·5 ± 7·99 ng mL^?1) when compared with patients in inactive periods (12·8 ± 4·43 ng mL^?1). In addition, patients with longer disease duration (mean, 20·1 ± 5·15 years) had also significantly (P = 0·013) higher leptin concentrations (20·2 ± 8·52 ng mL^?1) than those with shorter disease duration (13·4 ± 4·52 ng mL^?1) (mean, 7·4 ± 3·29 years). All acute‐phase reaction parameters were found to be significantly (for each, P < 0·01) increased in active disease. Conclusions Leptinmay have a role in modulating endothelial function and may be involved in mechanisms for vessel endothelium repair, during an exacerbation as well as in chronic disease.     

Salivary levels of HNP 1–3 are related to oral ulcer activity in Behçet’s disease

Background Saliva contains antimicrobial peptides derived from oral epithelium as well as neutrophils in the innate immune response. The aim of this study was to examine the association between salivary human neutrophil peptide (HNP) 1–3 levels originating from neutrophils and oral ulcers in patients with Behçet’s disease (BD). Methods Ninety‐five patients with BD (F/M: 39/56; mean age: 38.7 ± 11.9 years) and 53 healthy controls (HC; F/M: 23/30; mean age: 35.2 ± 10.1 years) were included in the study. The disease control group (F/M: 20/33; mean age: 33.7 ± 10.7 years) was comprised of patients with oral infection regarding endodontic infection (n = 32) and pericoronitis (n = 21). Salivary HNP 1–3 levels of groups were measured in unstimulated samples by ELISA (Hycult, the Netherlands). Results A statistically significant increase was found in salivary HNP 1–3 levels of patients with BD (2268.28 ± 1216.38 μg/ml) compared with HC (1836.49 ± 857.76 μg/ml), patients with endodontic infection (849.9 ± 376.1 μg/ml), and patients with pericoronitis (824.3 ± 284.02 μg/ml; P = 0.024, 0.000 and 0.000, respectively). The ratio of active oral ulcer (100%, n = 14) was higher in low HNP 1–3 levels (≤1000 μg/ml) than the others (66.7%, n = 54) in active patients with BD (P = 0.008). Moreover, salivary HNP 1–3 levels were significantly lower in patients with endodontic infection and patients with pericoronitis compared with those in the HC group and patients with BD (P = 0.000). Conclusion A decrease in salivary HNP 1–3 levels might be a biological factor for predisposition to oral ulcers in patients with BD and oral infection in healthy patients.     

Evaluation of sexual function in patients presenting with Behçet’s disease with or without depression

Aim Sexual dysfunction has been found in many disorders that are chronic or disabling. The aim of this study was to evaluate the sexual satisfaction levels, sexual function and their relationship with the mental state in a group of patients being followed‐up with a diagnosis of Behçet’s disease (BD). Method A total of 50 BD patients and 50 control‐group subjects were administered the Hamilton Depression Rating Scale (HDRS), Hamilton Anxiety Rating Scale (HARS), Golombok Rust Sexual Satisfaction Scale (GRISS) and Arizona Sexual Experiences Scale (ASEX). Results The ASEX, GRISS total, HDRS and HARS scores were significantly higher in the patient group than the control subjects (P = 0.0001, P = 0.007, P = 0.0001, P = 0.0001 respectively). Sexual dissatisfaction was seen in 40 (80%) of the patient‐group and 16 (32%) of the control‐group subjects according to the GRISS (P = 0.0001). Female study participants had higher mean scores than the control subjects for the ASEX, GRISS total scores and the GRISS satisfaction, avoidance, vaginismus and orgasm subscale scores (P = 0.0001, P = 0.002, P = 0.02, P = 0.001, P = 0.006, P = 0.03 respectively). Male study participants had different mean scores for the controls regarding the ASEX scores and the GRISS impotence, premature ejaculation, satisfaction and frequency subscale scores (P = 0.01, P = 0.01, P = 0.0001, P = 0.03, P = 0.007 respectively). Discussion The negative effect of the disorder on the biological and functional status and daily living activities in BD patients also influences the patients’ sexual experiences and satisfaction. The negative effects of chronic diseases such as BD should therefore be defined and the disorder evaluated from a wide perspective during the treatment process.     

Polymerase chain reaction reveals herpes simplex virus DNA in saliva of patients with Behçet’s disease

The etiology of Behçet’s disease is unclear, but viral infection is thought to be one etiologic factor. The aims of this study were to detect herpes simplex virus (HSV) DNA in the saliva of patients with Behçet’s disease and of healthy persons, to determine whether the presence of HSV in saliva is associated with the presence of intraoral ulcer, and to investigate the relationship between HSV and Behçet’s disease. The polymerase chain reaction (PCR) was used to detect HSV DNA sequences in DNA extracted from the saliva of patients with Behçet’s disease and of healthy control subjects. Of 66 patients with Behçet’s disease diagnosed clinically, 19 were diagnosed as complete type, 29 as incomplete type and 18 as suspected type. Of 66 DNA preparations from the saliva of the patients, 26 (39.4%) showed the 289-bp band. This contrasts with 12 of 87 preparations (13.8%) from healthy controls (P<0.01). There were no, significant differences among the three patient groups. All the 289-bp bands analyzed by restriction endonuclease digestion yielded the expected 158-bp and 131-bp fragments when digested withPst I. HSV DNA was detected in 12 of 33 Behçet’s disease patients (36.4%) with oral ulceration and 14 of 33 patients (42.4%) without oral ulceration at the time of testing. There was no statistically significant correlation in the PCR results between the two groups.     

Comparison of histopathologic and clinical evaluations of pathergy test in Behçet's disease

Background The pathergy test, an important test in the diagnosis of Behçet’s disease, is currently applied with disposable/sharp needles and evaluated only clinically (no histopathologic evaluation). In this study, the usefulness of the pathergy test conducted intradermally and intravenously with disposable/sharp needles in the diagnosis and determination of the activation of the disease is studied in comparison with the test conducted with nondisposable/blunt needles. In addition, histopathologic evaluation of the pathergy test is compared with the clinical evaluation. Methods The study group consists of 43 Behçet’s disease patients together with 15 patients with dermatosis as the control group. The pathergy test was applied to the Behçet’s disease patients and the control group intradermally and intravenously with disposable/sharp and nondisposable/blunt needles. Results The results of the pathergy test on the patients and the control group were evaluated clinically and histopathologically. Conclusions Clinical evaluation of the pathergy test conducted intradermally with nondisposable/blunt needles is sufficient for both the diagnosis and determination of the activation of Behçet’s disease. Histopathologic evaluation of the test is not found to be more sensitive than the clinical evaluation.     

Investigation of pregnancy associated plasma protein‐A and neopterin levels in Behçet's patients

Behçet's disease (BD) is an autoimmune disease that affects many organs. We aimed to investigate the relationship between BD and these pregnancy‐associated plasma protein A (PAPP‐A), neopterin, and high sensitive C‐reactive protein (hsCRP) parameters. The study included 57 BD patients and 54 healthy controls. After evaluating the active and inactive disease status of the patients, analyzes were performed. When comparing the patient and control groups, neopterin (111.27 ± 37.49; 76.77 ± 38.27 [nmol/L]; P < .001) and hsCRP (11.81 ± 16.8; 3.62 ± 5.06 [mg/L]; P = .001) parameters were significantly higher in patients. Neopterin (117.68 ± 41.67; 94.85 ± 14.75 [nmol/L]; P = .038) and hsCRP (14.68 ± 18.7; 4.47 ± 7.27 [mg/L]; P = .002) found different in active and inactive patients. The sensitivities of neopterin and hsCRP were also found to be high in BD (respectively 93%, 67%). PAPP‐A was especially elevated in skin pathologies (P = .02) and neopterin in joint involvement (P = .03). We think that the use of neopterin and hsCRP can help in diagnosis and follow‐up of BD.     

A comparison between the effects of low (1 µg) and standard dose (250 µg) ACTH stimulation tests on adrenal cortex functions with Behçet's disease

Introduction Behçet's disease is a rare, chronic disorder. The cause of Behçet's disease is unknown. It is believed to be caused by an autoimmune reaction. As in other chronic autoimmune diseases, Behçet's disease may show a subclinical adrenal failure and some changes in cortisol levels. We aimed to evaluate adrenal gland function in Behçet's disease patients. Material and method This study included 18 Behçet's disease patients and 15 healthy controls. Patient and control groups were administered i.v. 1 µg low dose test (LDT) and 250 µg standard dose test (SDT) adrenocorticotropic hormone (ACTH) stimulation test after 12 h of night fasting with an interval of 3‐days and cortisol responses in the 0th, 30th and 60th minutes were evaluated. Results There was no statistically significant difference between basal cortisol values of Behçet's disease and control groups. Cortisol values in the 60th minute in LDT were significantly lower in Behçet's disease group than in the control group. In the peak cortisol responses to LDT, a significant decrease was found in Behçet's disease group. Conclusion These findings suggest that hypothalamo‐pituitary adrenal axis is partially suppressed in Behçet's disease.     

Increased arterial stiffness assessed by pulse wave velocity in Behçet’s disease and its association with the lipid profile

Objective To evaluate the structural and functional properties of vessels in Behçet’s Disease (BD) using carotid‐femoral pulse wave velocity (PWV) and an echo‐tracking system. Methods BD patients without traditional cardiovascular risk factors were selected. All BD patients performed PWV and carotid ultrasound. BD patients were divided into groups based on the presence of systemic (vascular and/or ocular and/or central nervous system involvement) and vascular involvement. Healthy controls age‐ and sex‐matched with the same exclusion criteria were selected. Results A total of 23 BD patients (mean age 35.0 ± 7.6 years) had significantly higher PWV levels compared with controls (8.48 ± 1.14 vs. 7.53 ± 1.40 m/s, P = 0.017). Intima‐media thickness (594.87 ± 138.61 vs. 561.08 ± 134.26 μm, P = 0.371), diastolic diameter (6383.78 ± 960.49 vs. 6447.65 ± 1159.73 μm, P = 0.840), distension (401.95 ± 117.72 vs. 337.91 ± 175.36 μm, P = 0.225) and relative distension (6.26 ± 2.83 vs. 5.42 ± 2.46 μm, P = 0.293) were similar in both groups. The systemic disease group had significantly higher levels of PWV (8.79 ± 1.21 vs. 7.88 ± 0.72 m/s, P = 0.036) compared to those with exclusive mucocutaneous manifestations. BD patients with vascular involvement had similar PWV and echo‐tracking parameters compared to those without vascular involvement (P > 0.05), but had higher total and LDL cholesterol levels (P = 0.019 and P = 0.012, respectively). The multivariate linear regression analysis identified triglycerides as the most important factor in increasing PWV levels (P = 0.001) in BD. Conclusions PWV is more useful than carotid ultrasound in detecting structural and functional vascular damage in BD and emphasizes the role of the disease itself in promoting these alterations. Our findings also reinforce the need for rigorous control of all risk factors in BD, particularly lipoproteins.     

Serum GDF‐15 level in Behçet's disease: relationships between disease activity and clinical parameters

Growth differentiation factor‐15 (GDF‐15), a member of the transforming growth factor‐β superfamily of cytokines, plays an important role in cell growth, signal transduction, and apoptosis regulation. The aim of this study was to evaluate serum GDF‐15 levels and their relationships with disease‐related variables in patients with Behçet's disease (BD). Forty‐six patients diagnosed with BD and 30 demographically matched healthy control subjects participated in the study. GDF‐15 levels were measured in blood samples from patients and controls. The Behçet's Disease Current Activity Form (BDCAF) was used to evaluate the disease activity of BD. There were no significant differences between the two groups in C‐reactive protein (CRP) level, mean erythrocyte sedimentation rate (ESR), age, body mass index, and mean GDF‐15 levels (P > 0.05). Serum GDF‐15 levels were positively correlated with findings for peripheral arthritis and CRP, and with BDCAF erythema nodosum, BDCAF arthralgia, and BDCAF arthritis scores. Patients with BD were divided into two groups according to the presence of peripheral arthritis; nine subjects (20%) were positive for peripheral arthritis. Serum ESR, CRP, white blood cell counts, and GDF‐15 levels were significantly higher in the group that was positive for peripheral arthritis (P < 0.05). GDF‐15 may play a role in the progression and pathway of Behçet's joint involvement and erythema nodosum that is independent of classic inflammatory response measures.     

JUVENILE BEHÇET'S DISEASE AMONG 1784 TURKISH BEHÇET'S PATIENTS

Background. Behçet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. Materials and Methods. Patients suffering from Behçet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behçet's disease (JBD), were detected among 1784 Turkish Behçet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 ± 7.1 years. Results. The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. Conclusions. Severe Behçet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.     

Lipoprotein‐associated phospholipase A2 level in patients with Behçet’s disease

Background Behçet’s disease (BD) is a chronic multisystem inflammatory disorder characterized by vasculitis. Vasculitis is thought to underlie many of the clinical manifestations of Behçet’s disease. Lipoprotein‐associated phospholipase A₂ (Lp‐PLA₂) is a highly specific biomarker for vascular inflammation, and has low biological variability. Those features make it more attractive than other inflammatory markers including C‐reactive protein (CRP) and erythrocyte sedimentation rate (ESR), which may reflect systemic inflammation non‐specifically. Objectives It was aimed to investigate circulating Lp‐PLA₂ levels and its relationship with CRP and ESR in patients with BD by considering disease activity. Methods Study group included 72 patients with BD (34 men and 38 women with a mean age of 35.3 years) and 30 sex‐ and age‐matched healthy subjects (15 men and 15 women with a mean age 32.6 years). Patients group included 40 patients with active and 32 patients with inactive BD. Results Lp‐PLA₂, CRP and ESR levels were found to be significantly higher in patient group than controls. In addition, those levels were also significantly higher in patients with active BD than in patients with inactive disease. Lp‐PLA₂ showed positive correlations with CRP and ESR (r = 0.63, P < 0.05 and r = 0.33, P < 0.05 respectively). Lp‐PLA₂ also showed significant important area under curve (AUC) value (0.779), besides CRP (0.941) and ESR (0.888). Optimum cut‐off value was obtained as 218.5 ng/mL. Conclusions It was concluded that Lp‐PLA₂ may be a new useful biomarker to evaluate clinical or subclinical activity of the disease besides CRP and ESR.     

A randomized,controlled and blinded study of papulopustular lesions in Turkish Behçet’s patients

Background Papulopustular lesions (PPL), the commonest presentation of skin lesions in Behçet’s disease (BD) are cutaneous, sterile folliculitis or acne-like lesions on erythematous base. Our purpose was to determine the true frequency and anatomic location of the PPL and compare this with controls. We also sought to determine whether or not there was any relationship between PPL and either disease activity or other manifestations of BD. Methods Fifty patients with BD, diagnosed according to the criteria of the International Study Group for Behçet’s Disease, were enrolled in the study. The control group consisted of 100 patients with other dermatologic diseases (21 acne and 79 non-acne patients), selected randomly. A dermatologist counted the lesions, in a blind protocol, on seven anatomic locations: scalp, face, neck, trunk, upper and lower extremities and genitalia. Results The frequency of PPL in patients with BD was 96% and the most common location was the trunk, whereas in the control group the frequency was 89% and the most common location was the face. In acne and non-acne patients, the frequency was 100% and 86.1% respectively. The total mean number, and mean numbers of PPL on the location of trunk, upper and lower extremities, and genitalia were higher in patients with BD than in controls. When the PPL in BD patients with a positive pathergy test was compared with that in patients with a negative pathergy test, the difference was significantly higher. Conclusions Our results indicate that PPL appear to be non-specific. In the diagnosis of BD the mean number and anatomic location of the lesions are of more importance than the frequency.     

Stressful life events, anxiety, depression and coping mechanisms in patients with Behçet''s disease

Background Behçet's disease is a systemic immunoinflammatory disease of young adults characterized by systemic vasculitis of arteries and veins. Although many studies have been published since its discovery in 1937, the etiopathogenesis of this unique disorder is still unclear. Objective To assess the relationship between stress factors, psychological and somatic symptoms, and coping mechanisms in patients with Behçet's disease. Method Thirty‐four patients with Behçet's disease and 43 control subjects were compared by using sociodemographic data collection forms, a psychosocial and environmental problems list, the Beck Anxiety Inventory (BAI), Hamilton Depression Rating Scale (HAM‐D) and Toronto Alexithymia Scale (TAS). Results Twenty‐four patients (70.6%) defined stress factors in the first stage of the disease. Twenty‐seven (79.4%) out of 34 patients stated that the recurrence period of the disease was related to the stress factors. Fear was expressed by 10 (29.4%) patients, sadness by 11 (32.3%), and fear plus sadness by 13 (38.2%) when they first learnt the diagnosis. While coping with these emotions 14 (41.2%) revealed active‐reliance strategy. A statistically significant difference was present between the Behçet's patients and control subjects regarding TAS (P < 0.05), HAM‐D (P < 0.001) and BAI (P < 0.001) scores. Conclusion It seems that stressful life events have important implications in both relapsing and remission periods of Behçet's disease via secondary problems.     

Lack of association between leptin G2548A gene polymorphism and Behçet''s disease

Background Behçet's disease is a chronic, multisystem, inflammatory disease characterized by the predominance of T‐helper 1 cytokines. The disease is also characterized by infiltration of lymphocytes and neutrophils into the affected tissues. Because cytokines are involved in the regulation of lymphocyte and phagocyte functions, they may play an important role in the pathogenesis of Behçet's disease. Leptin, a member of the gp 130 family of cytokines, induces a strong T‐helper 1 response and is regarded as a proinflammatory inducer. Recent studies have shown that serum leptin concentration was increased in patients with Behçet's disease and correlated with disease activity. Objectives We aimed to investigate the role of G2548A polymorphism of leptin gene in patients with Behçet's disease and compare the results with healthy controls. Patients and methods A total of 93 subjects with Behçet's disease and 125 healthy controls were included in this study. Analyses of G‐2548A polymorphism of the LEP gene were performed using the PCR‐restriction fragment length polymorphism technique. The genotypes (GG, GA, and AA of leptin G2548A) and alleles (G and A of leptin 2548) were scored and the frequency was estimated. The frequencies of the alleles and genotypes in patients and controls were compared. We analysed the correlation between leptin gene polymorphism and the clinical features of BD. Results Both genotype and allele frequencies were not significantly different between controls and Behçet's disease patients [OR = 0.67, 95% CI (0.35–1.29), P = 0.197 and OR = 0.77, 95% CI (0.52–1.15), P = 0.184]. We did not find any significant relationship between leptin gene polymorphism and the clinical features of BD (P > 0.05). Conclusion In the present case‐control study, we found no evidence of an association between the G‐2548A variant of the leptin gene and BD among Turks. Further studies are needed to investigate serum leptin level to explain the mechanisms behind the lack of association between leptin G2548A gene polymorphism and BD.     

Serum interleukin-8 as a serologic marker of activity in Behçet's disease

Background Immune dysregulation has been shown to be one of the major aspects of the yet unknown pathogenesis of Behçet's disease. Interleukin-8 (IL-8), a major chemokine with pivotal effects concerning leukocytes and endothelial cells, has been found to be elevated in patients with Behçet's disease. Aim To evaluate the significance of elevated levels of IL-8 with respect to the activity of Behçet's disease. Methods Sixty-seven consecutive patients with Behçet's disease (37 males, 30 females; 32.5 ± 9.3 years) were enrolled in our study. The number of active clinical manifestations at the time of serum sampling was recorded. The degree of association between disease activity and IL-8, C-reactive protein, and erythrocyte sedimentation rate was assessed. Results Serum levels of IL-8 increased as the number of clinically involved organs increased (P < 0.05). C-reactive protein and the erythrocyte sedimentation rate showed no correlation with disease activity. Conclusions Our study confirms that the IL-8 level is a more sensitive marker of disease activity than the erythrocyte sedimentation rate and C-reactive protein. It may be assumed that IL-8 plays an important role in the pathophysiology of Behçet's disease.