Behcet's Patients Research Articles

Advancing Thrombosis Research: A Novel Device for Measuring Clot Permeability.

Thromboembolism, a global leading cause of mortality, needs accurate risk assessment for effective prophylaxis and treatment. Current stratification methods fall short in predicting thrombotic events, emphasizing the need for a deeper understanding of clot properties. Fibrin clot permeability, a crucial parameter in hypercoagulable states, impacts clot structure and resistance to lysis. Current clot permeability measurement limitations propel the need for standardized methods. Prior findings underscore the importance of clot permeability in various thrombotic conditions but call for improvements and more precise, repeatable, and standardized methods. Addressing these challenges, our study presents an upgraded, portable, and cost-effective system for measuring blood clot permeability, which utilizes a pressure-based approach that adheres to Darcy's law. By enhancing precision and sensitivity in discerning clot characteristics, this innovation provides a valuable tool for assessing thrombotic risk and associated pathological conditions. In this paper, the authors present a device that is able to automatically perform the permeability measurements on plasma or fibrinogen in vitro-induced clots on specific holders (filters). The proposed device has been tailored to distinguish clot permeability, with high precision and sensitivity, between healthy subjects and high cardiovascular-risk patients. The precise measure of clot permeability represents an excellent indicator of thrombotic risk, thus allowing the clinician, also on the basis of other anamnestic and laboratory data, to attribute a risk score to the subject. The proposed instrument was characterized by performing permeability measurements in plasma and purified fibrinogen clots derived from 17 Behcet patients and 15 sex- and age-matched controls. As expected, our results clearly indicate a significant difference in plasma clot permeability in Behcet patients with respect to controls (0.0533 ± 0.0199 d vs. 0.0976 ± 0.0160 d, p < 0.001). This difference was confirmed in the patient's vs. control fibrin clots (0.0487 ± 0.0170 d vs. 0.1167 ± 0.0487 d, p < 0.001). In conclusion, our study demonstrates the feasibility, efficacy, portability, and cost-effectiveness of a novel device for measuring clot permeability, allowing healthcare providers to better stratify thrombotic risk and tailor interventions, thereby improving patient outcomes and reducing healthcare costs, which could significantly improve the management of thromboembolic diseases.

A Comparison of Adult and Juvenile Behcet Patients and a Look at Clinical Trends: Retrospective Data from a Turkish Follow-Up Cohort Study

Background: Previous reports indicate that juvenile Behçet’s disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. Objectives: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. Methods: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet’s Disease at or before 16 years of age. Results: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976–1997 and 1998–2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. Conclusions: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.

Evaluation of the Psychoeducation Program Given to Behcet's Patients in the Context of the Roy Adaptation Model.

The physical, social, and psychological effects of Behcet's disease necessitate the person's adaptation in many areas. This study was conducted to examine the effect of psychoeducation provided to people living with Behcet's disease in the context of the Roy adaptation model on illness adjustment, dyadic adjustment, self-esteem, and psychiatric symptoms. The study was quasi-experimental. The study was with 70 patients with Behcet's disease: 35 patients were in the intervention group and the same number in the control group. The data of the study were collected using the Adaptation to Chronic Illness Scale, Dyadic Adjustment Scale, Rosenberg Self-Esteem Scale, and Brief Symptom Inventory. A seven-session psychoeducation program was provided to the intervention group. After the psychoeducation program, adaptation to illness increased significantly and psychiatric symptoms decreased significantly in the intervention group compared with the control group (P < .05).

Protective effect of early immunomodulatory treatment on ocular involvement in Behcet's disease: Historical cohort of 1166 patients

ObjectivesEye involvement is a main presentation of Behcet's disease. This study was performed to evaluate possible determinants affecting the occurrence of eye involvement, especially the role of early systemic treatment with immunomodulatory drugs on the incidence of ocular involvement. MethodsThis is a retrospective cohort study performed on 1166 Behcet's patients in the Behcet's Clinic of Rheumatology Research Center. All patients were followed up for at least 10 years and a maximum of 15 years. Data analysis was performed using survival analysis models including Kaplan-Meier Survival analysis, Logrank test, and Cox's proportional hazards regression. Results1166 Behcet's patients were evaluated. 80 patients who had eye involvement as the first manifestation of the disease were excluded and 1086 participants entered the analysis. Among them, 647 patients (59.6%) developed ocular involvement 7.8 ± 6.7 years after the first symptom. Immunomodulatory treatment before ocular involvement reduced the risk by 3 times (P-value <0.001). ConclusionThis study demonstrated that the initiation of immunomodulatory treatment prior to eye involvement can reduce the risk of eye involvement in Behcet's patients. Therefore, reducing the onset time of disease symptoms and providing appropriate treatment can reduce Behcet's disease ocular complications.

Comparison of Serum Levels of SIL-2R, IL-6, IL-10, TNF-α, CRP, ESR and Fibrinogen in Patients with Active and Inactive Behçet’s Disease

Behçet's disease (BD) is a chronic inflammatory illness that affects the entire body and is characterized by recurring episodes of oral aphthae, ocular and cutaneous lesions, and scrotal or vaginal ulcerations. The involvement of other organs and systems increases mortality in addition to the significant morbidity. This study involved a total of 40 participants, 20 of whom were healthy controls and 20 of whom were patients (of the 20 Behçet's patients, 12 (60%) were in the active phase and 8 (40%) were in the inactive phase). There was no therapy being given to any of the 20 patients that would have affected their cytokine levels. Only young men made up the patient and control groups because both early onset and male sex are signs of poor prognosis. The ELISA method was used to measure the levels of serum cytokines. The statistical analysis of the derived numerical values employed the Mann-Whitney U Test. We found a significant correlation between serum cytokine levels and classical acute phase markers in active Behçet’s patients. ESR (P &lt; 0,001), CRP (P &lt; 0,001), fibrinogen (P &lt; 0,001), IL-10 (P &lt; 0,001), IL-6 (P &lt; 0,001), SIL-2R (P &lt; 0,001) and TNFα (P &lt; 0,001). There was no statistically significant difference in serum levels of classical acute phase markers ESR (P = 0,746), CRP (P = 0,476) and fibrinogen (P = 0,940) when inactive Behçet’s patients and healthy controls were compared. However, serum levels of IL-10 (P &lt; 0,001), IL-6 (P = 0,001), SIL-2R (P &lt; 0,001) and TNFα (P = 0,001) were statistically different between inactive Behcet's patients and the control group. Our research shows that even in the inactive phase, serum cytokine levels of Behçet’s patients are much higher than the healthy control group. However, the levels of ESR, CRP and fibrinogen, which are classical acute phase markers, were found at normal levels in Behçet’s patients in the inactive phase. These findings show that measurement of serum interleukin levels will enable us to take preventive measures for morbidity and mortality follow-up of Behçet's patients.

Gender-Specific Differences in Egyptian Behcet's Disease Patients

Gender-Specific Differences in Egyptian Behcet's Disease Patients

Scoping beyond pulmonary artery involvement; pulmonary involvement in Behcet's disease; a retrospective analysis of 28 patients.

Pulmonary involvement other than pulmonary artery involvement in Behcet's disease still remains an area of investigation. The aim of this study was to evaluate pulmonary involvement associated with Behcet's disease. We retrospectively investigated all Behcet's disease patients in terms of pulmonary involvement. Twenty-eight patients, whose radiologic examinations were consistent with Behcet's disease-related involvement after excluding other possibilities, were included in this study. Data regarding demographic characteristics, other clinical components of Behcet's disease, treatment modalities, and types of pulmonary involvement were analyzed. Pulmonary involvement was seen more common in male (82.1% vs 17.9%). Mean age for Behcet's disease diagnosis was found 32 years (SD 10.9) and mean age for pulmonary involvement was calculated 37 years (SD 11.4). Deep vein thrombosis (DVT) was the most common associated vascular involvement (53.6%). In our study population, alveolar hemorrhage and/or ground glass appearance were seen in 46.4% (13/28) of BD patients with pulmonary involvement. Totally, pulmonary artery aneurysm (PAA), small-sized pulmonary vasculitis (sPV), and pulmonary thrombosis (PT) were seen in 7 (25%), 13 (46.3%), and 18 (64.4%) of patients, respectively. Intracardiac thrombosis (ICT) in the right ventricle was present in 5 patients. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) in the first line. Warfarin was used in 18 patients. Overall mortality was seen in 3 patients: 1 due to PAA bleeding and others with unknown causes. Despite the importance of pulmonary artery involvement and pulmonary thrombosis in Behcet's disease, small-sized pulmonary vasculitis in the form of small vessel involvement is generally overlooked. Our study findings have shown that alveolar hemorrhage and/or ground-glass appearance in the absence of pulmonary artery aneurysm and pulmonary thrombosis are seen commonly as well. Key Points • The characteristics of pulmonary small vasculature involvement in Behcet's disease which is still an area of investigation warrant further attention. • The clinician should bear in mind that the spectrum of pulmonary involvement in Behcet's disease may be variable, but an extensive work up is still of great importance especially in atypical cases.

Effects of anti-TNF biologic drugs on uveitis severity in Behçet patients: systematic review and Meta-analysis.

To investigate effects of anti-TNF biologic drugs on uveitis severity (comparing visual acuity logMAR levels) in Behçet patients. Three databases PubMed, Scopus, and the Web of Science were searched for qualified papers focusing on the anti-TNF-α factors treatment in Behçet's disease (BD)-associated uveitis. Studies that were designed pre and post anti-TNF drug treatment, were selected. After determining the search strategy for this study, the relevant data were extracted. The initial search was performed in the target databases and a total of about 1458 articles were found. Fifteen articles were selected for systematic review and only 12 of them had inclusion criteria for Meta-analysis (with visual acuity data). The mean dose of prednisolone before and after biological treatments was reported in 5 studies (28.56 and 7.56 mg/kg, respectively). Also, the preliminary results indicate a significant reduction in visual acuity logMAR levels (MD=-1.5 IU/L, 95%CI: -2.1, -0.01). Biological drugs significantly reduce the dose of prednisolone and affect visual acuity values.

Global systematic review and meta-analysis of health-related quality of life in Behcet's patients.

Background:Behcet’s disease (BD) is a chronic fatal illness with a relapsing remitting nature and significant organ-threatening morbidity and mortality. The aim of this research was to examine studies which were conducted on investigation of prevalence of quality of life among patients with Behcet’s disease.Methods:A total of 13 articles were extracted from four main databases including PubMed, EMBASE, Scopus, and Web of Science from the onset of 2000 to January 2021. All studies published in English with the purpose of examining quality of life (QOL) among patients with BD or investigating its main determinants were included.Results:Totally, 1137 BD patients participated in 13 studies. Based on random effect analysis, the total score of physical health-related QOL was 46.7 (95% CI=41.26 to 52.13) and the total score of mental health-related QOL was 49.01 (95% CI=43.83 to 54.18) representing a moderate level of QOL among BD patients. Furthermore, weighted effect size analyses showed a significant correlation between QOL and variables such as patients’ age, gender, disease duration and depression (pvalue: 0.00).Conclusion: As the symptoms of BD worsen over time, patients confront with more severe body pain, mobility restrictions, and difficulties in chewing, eating, speaking and swallowing which negatively affect social interactions of patients and reduce their QOL. Furthermore, depression was proved to act as a deteriorating factor for Health-Related Quality of Life (HRQOL) among BD patients. Thus, patients need to be psychologically supported by a specialized team and be informed during the course of treatment to gain useful information about the disease, treatment approaches and coping strategies.

IL-17A and IFN-γ are Up-regulated in CD4 and γδ T Cells in Active Behcet's Disease Patients

Involvement of γδ T cells is implicated in the pathogenesis of Behçet's disease (BD) as a bridge between innate and adaptive responses. IL-17 and IL-22 have also been shown to participate in the BD pathogenesis in addition to IFN-γ. Mainly CD4+ T cells are investigated previously for the production of these inflammatory cytokines. In this study, the role of γδ T cells in cytokine-related mechanisms was evaluated in BD in comparison to CD4+ T cells. Surface expression of markers for functional states of both CD4+ and γδ T cells were compared in ex vivo samples collected from patients with BD and healthy controls (HC).Sixteen active BD (a-BD), 9 inactive BD (i-BD) patients and 25 HC were investigated. The expression of CD161, CCR6 as markers for IL-17 producing cells were analyzed on γδ and CD4+ T cells. IFN-γ, IL-17A, IL-22, as well as CD107a (LAMP1) and CD16 (FcγRIII) were evaluated in both cell subtypes after in vitro stimulation.Only IFN-γ production was increased in γδ T cells of a-BD patients. There was no difference in increase of CD107a or decrease of CD16 surface expression on γδ T cells upon stimulation between the groups. Ex vivo IL-17A and both IL-17A/IFN-γ production and expression of CD161, CCR6 by CD4+ T cells were increased in a-BD.Along with CD4+ T cells, γδ T cells have complementary roles in cytokine production in BD. Higher IFN-γ production of γδ T cells suggests the role of an environmental triggers in BD pathogenesis, whereas IL-17 related activity is mainly provided by CD4+ T cells.

Histopathological comparison of pathergy positive and negative areas of newly diagnosed Behçet patients.

Behcet's disease is an inflammatory vasculitis, and pathergy reaction is a compulsory element of the diagnostic criteria. In the literature, there is no study comparing the histopathology of negative pathergy tests and positive pathergy tests in newly diagnosed Behcet's patients. The primary goal was to investigate the frequency of vasculitis, which is an important finding of Behcet's disease, in pathergy histopathology. The histopathology of patients with positive and negative pathergy tests revealed vasculitis or vasculopathy. In histopathology, neutrophils, lymphocytes, eosinophils, and mast cells in the perivascular area were counted and recorded as a percentage. Of the 88 Behcet's patients who participated in the study, 45 were female and 43 were male. The rate of vasculitis in the histopathology of those who were positive for the pathergy test was not statistically different than those of those with negative pathergy tests (P=.14). In our study, although the relationship between the male gender and uveitis was significant at the border, and the relationship between pathergy positivity and uveitis was not found to be significant, a significant relationship was found between vasculitis in histopathology and uveitis (P=.023). Based on the results of our study, we think that a pathergy test of histopathology will contribute to the clinical diagnostic evaluation in cases with suspicious clinical pathergy tests and especially in cases with negative pathergy tests.

Serum growth differentiation factor-15 (GDF-15) level in behcet's disease patients: Relation to clinical characteristics, musculoskeletal ultrasound findings and disease activity

Aim of the workThis study was conducted to assess serum level of growth differentiation factor-15(GDF-15) in Behcet’s disease (BD) patients and its relationship with clinical characteristics, ultrasound findings and disease activity. Patients and methodsStudy included 30 BD patients and 20 matched healthy controls. Visual analog scale (VAS)-pain of affected joints and Behcet’s disease current activity form (BDCAF) were assessed. The most affected joints and surrounding soft tissues were assessed by musculoskeletal ultrasound (MSUS).Serum GDF-15 level was measured. ResultsPatients were 23 males and 7 females (M:F 3.3:1) with a mean age of 34.6 ± 8.6 years and mean disease duration of 6.6 ± 4.5 years. 18(60%) had arthritis and arthralgia. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were 26.2 ± 21.2 mm/1sthr and 50.4 ± 37 mg/dl respectively. There was significant increase of GDF-15 levels in patients with articular affection (3492.3 ± 4618.8 ng/ml) compared to those without (338.9 ± 90.3 ng/ml) and controls (322 ± 78.8 ng/ml) (p < 0.001).As regard MSUS, knee (n = 8) and ankle joints (n = 8) were the most commonly affected joints. Synovitis was present in 13 patients, enthesopathy was present in 10. GDF-15 significantly correlated with ESR and CRP (r = 0.48;p = 0.008 and r = 0.73, p = 0.005) and both remained significant on regression analysis that ESR (p = 0.019 and p = 0.007 respectively). At a serum GDF-15 cut-off point of 570 ng/ml for patients with peripheral arthritis/arthralgia the sensitivity and specificity were 88.9% and 91.7% respectively. ConclusionSerum GDF-15 can be used as a marker for articular affection in BD patients and is associated with the MSUS imaging visualizing subclinical inflammatory changes.

One-year results of stent graft repair for carotid artery pseudo-aneurysm in patients with Behcet's disease.

Behcet's disease is a multisystem disorder of unknown etiology with vascular complications. This study reviewed the mid-term outcome of Behcet's disease patients with carotid artery pseudo-aneurysms treated by endovascular stent-graft repair at our unit. During a period of 11 years, six cases were included. Postoperative ultrasound duplex results were recorded along with computed tomography angiography report done a year after intervention. The mean age (±SD) was 38 (±5.2) years. The mean (±SD) pseudo-aneurysm size was 33 (±12.2) mm. Technical success was 83%; failed cannulation of the internal carotid artery was encountered in one case. On day 2 post-operative, a duplex ultrasound revealed complete exclusion and thrombosis of the false aneurysm in all cases. A year later, a computed tomography angiography revealed a primary patency rate of 80%, and only one case had a recurrent pseudo-aneurysm at the distal margin of the stent graft. All cases, however, had complete thrombosis in the pseudo-aneurysms lumen with a mean (±SD) regression in size of 18 (±6) mm. The mean (±SD) percentage of in-stent stenosis was 34.5% (±11.73%). Stent graft repair for carotid artery pseudo-aneurysm in Behcet's disease patients might be the preferable first line of treatment since it had a high technical success and mid-term primary patency rates, with additional fact that it obviously avoids the hazardous complications of surgery.

Increased Concentrations of Circulating Soluble MHC Class I-Related Chain A (sMICA) and sMICB and Modulation of Plasma Membrane MICA Expression: Potential Mechanisms and Correlation With Natural Killer Cell Activity in Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a severe autoimmune disease of unknown etiology. The major histocompatibility complex (MHC) class I-related chain A (MICA) and B (MICB) are stress-inducible cell surface molecules. MICA and MICB label malfunctioning cells for their recognition by cytotoxic lymphocytes such as natural killer (NK) cells. Alterations in this recognition have been found in SLE. MICA/MICB can be shed from the cell surface, subsequently acting either as a soluble decoy receptor (sMICA/sMICB) or in CD4+ T-cell expansion. Conversely, NK cells are frequently defective in SLE and lower NK cell numbers have been reported in patients with active SLE. However, these cells are also thought to exert regulatory functions and to prevent autoimmunity. We therefore investigated whether, and how, plasma membrane and soluble MICA/B are modulated in SLE and whether they influence NK cell activity, in order to better understand how MICA/B may participate in disease development. We report significantly elevated concentrations of circulating sMICA/B in SLE patients compared with healthy individuals or a control patient group. In SLE patients, sMICA concentrations were significantly higher in patients positive for anti-SSB and anti-RNP autoantibodies. In order to study the mechanism and the potential source of sMICA, we analyzed circulating sMICA concentration in Behcet patients before and after interferon (IFN)-α therapy: no modulation was observed, suggesting that IFN-α is not intrinsically crucial for sMICA release in vivo. We also show that monocytes and neutrophils stimulated in vitro with cytokines or extracellular chromatin up-regulate plasma membrane MICA expression, without releasing sMICA. Importantly, in peripheral blood mononuclear cells from healthy individuals stimulated in vitro by cell-free chromatin, NK cells up-regulate CD69 and CD107 in a monocyte-dependent manner and at least partly via MICA-NKG2D interaction, whereas NK cells were exhausted in SLE patients. In conclusion, sMICA concentrations are elevated in SLE patients, whereas plasma membrane MICA is up-regulated in response to some lupus stimuli and triggers NK cell activation. Those results suggest the requirement for a tight control in vivo and highlight the complex role of the MICA/sMICA system in SLE.

Etiological factors and histopathological features in erythema nodosum: a 6-year retrospective cross-sectional study

Erythema nodosum (EN) is the most common type of panniculitis. The most frequent etiological factors are streptococcal pharyngitis, sarcoidosis, Behçet's disease, and tuberculosis. Our objective was to identify the etiological factors and to evaluate the patients' clinical, laboratory, and histopathological findings. Eighty-eight patients diagnosed with EN at our clinic between 2013 and 2019 were evaluated retrospectively. Sixty-five patients were evaluated histopathologically. The patients' ages ranged between 17 and 76 (mean age: 41.91 ± 13.07 years). EN was 7.8 times more frequent in women. Patients presenting with idiopathic EN were significantly older than secondary cases (p < 0.05). Sixty-one patients (69.3%) had an underlying disease (secondary EN). The most common etiological factors were upper respiratory tract infections (n = 26), followed by Behçet's disease (n = 20). Septal panniculitis was present in 89.2% of cases evaluated histopathologically. Mixed or lobular panniculitis was present in 35.7% of Behçet's disease patients with EN-like lesions. Vasculitis was also noted in 35.7% of Behçet's disease patients. Our data confirm the predominance of upper respiratory tract infections and Behçet's disease among patients with EN in Turkey. Behçet's disease patients presenting with EN-like lesions may show mixed panniculitis and vasculitis, whereas classic EN patients predominantly show septal panniculitis.

Serum interleukin-37 (IL-37) and its gene polymorphism in Iranian Behcet's disease patients: Association with disease manifestations and activity

IntroductionThe etiopathogenesis of autoimmune inflammatory Behçet's disease (BD) has not been well understood but cytokines and genetic factors might play a role. Interleukin (IL)-37 is an anti-inflammatory cytokine that suppresses both innate and adaptive immunity. This study aimed to assess the possible association between serum levels of IL-37 and its genetic polymorphisms with susceptibility to BD. MethodsSerum levels of IL-37 and its genetic variants were determined using enzyme-linked immunosorbent assay (ELISA) and allele-specific polymerase chain reaction, respectively. ResultsThe results revealed significantly lower level of IL-37 in BD patients (82.8 ± 2.8 pg/ml) compared to the healthy subjects (110.3 ± 11.8 pg/ml, p < 05). Furthermore, significantly lower level of IL-37 (77.2 ± 2.3 pg/ml, p = 0.048) was shown in patients with ocular involvement. In addition, genotypic analysis demonstrated more rs4241122 AA genotype frequency among male BD patients with an active form of disease and those with gastrointestinal manifestations (p = 0.01). However, this genotype was more frequently seen among female patients with an inactive form of disease (p < 0.001). Besides, allelic analysis showed a higher frequency of rs4241122 A allele among male patients with an active form of disease (p = 0.006), as well as among female patients with vascular involvement (p = 0.03). ConclusionOur data indicated that decreased levels of IL-37 might act as a susceptibility factor for BD and ocular involvement among patients. We also found gender-biased associations between genotypes and alleles of rs4241122 and disease manifestations. However, further studies with larger sample sizes are needed to verify these results.

Fragmented QRS complexes are associated with subclinical left ventricular dysfunction in patients with Behcet's disease: Four-dimensional speckle tracking echocardiography.

Behcet's disease (BD) is a chronic systemic inflammatory disease in which early detection of cardiac involvement is essential. The aim of this study was to assess the left ventricular (LV) functions in BD patients using four-dimensional (4D) speckle tracking echocardiography (STE) and to test the correlation between LV dysfunction and the presence of QRS fragmentation. This cross-sectional study included 64 Behcet's patients and 48 healthy volunteers. The BD group was divided into two subgroups depending on the presence (fQRS+) or absence (fQRS-) of fragmented QRS (fQRS). In both groups, left ventricular global area strain (LV-GAS), global radial strain (GRS), global longitudinal strain (GLS), and global circumferential strain (GCS) were obtained with 4D echocardiography. GAS, GRS, GLS, and GCS values were significantly different in Behcet's patients and in healthy volunteers. GLS and GAS values were lower in the fQRS+ than in the fQRS- group (-15.8 ± 1.8 and -17.9 ± 1.6, P = .001 vs -25.0 ± 3.1 and -29.2 ± 4.2, P < .001, respectively). The duration of disease was longer in fQRS+ than in fQRS- patients (120.8 ± 67.4 vs 71.0 ± 40.5, P < .001). Multiple linear regression analysis showed that fQRS and disease duration were independent predictors of LV-GAS. Four-dimensional STE may be helpful for the prediction of early cardiac dysfunction in patients with BD. The presence of fQRS may be an indicator of subclinical LV dysfunction.

Sleep Quality, Depression Level and Affecting Factors of Behçet's Patients

Çalışma, Behçet hastalarının uyku kalitesi, depresyon düzeyleri ve etkileyen faktörleri belirlemek amacıyla tanımlayıcı olarak yapıldı. Çalışmaya katılmayı kabul eden 44 Behçet hastası çalışmaya dahil edildi. Çalışmanın verileri, araştırmacılar tarafından hazırlanan “Hasta Bilgi Formu”, “Pitsburg Uyku Kalitesi İndeksi (PUKİ)”, “Beck Depresyon Ölçeği (BDÖ)”, Behçet Hastalığı Yaşam Kalitesi Ölçeği (BHYKÖ)” ve “Behçet Hastalığı Güncel Aktivite Formu” ile toplandı. Verilerin değerlendirilmesinde, Mann Whitney U ve Spearman korelasyon analizleri yapıldı. Hastaların yaş ortalamasının 37,38±8,33 yıl, %90,9'unun erkek ve hastalık süresi ortalamasının 7,54±4,72 yıl olduğu belirlendi. Genital ülseri olan hastaların PUKİ puanının anlamlı olarak yüksek olduğu saptandı (p0,05). Hastalık aktivite skoru ile PUKİ, BDÖ, BHYKÖ puanları arasında anlamlı ilişki olduğu belirlendi (p

Nail Fold Capillaroscopy Abnormality in Behcet's Disease and Relation to Disease Activity among Egyptian Patients

Background: Behcet’s disease is a systemic autoimmune disease. Treatment mainly is immunosuppression. It has no sure diagnostic tests and relies mainly on clinical diagnosis. There are few scores to assess Behcet's disease activity (Behcet's Disease Current Activity Form) BDCAF score. Nailfold Capillaroscopy is a technique to visualize capillaries in nailfold area. It can correlate many diseases with the abnormalities in shape of blood capillaries. Objectives: The aim of the work was to find out Nail Fold Capillaroscopy abnormality in Behcet's Disease and its relation to the disease activity. Patients and Methods: A cross sectional-case control study which was done on 50 Behcet’s patients and 30 healthy controls. We assessed nailfold capillaroscopic changes in Behcet’s patients in comparison to controls and the correlation of BDCAF score with nailfold capillary abnormalities. Results: we found that 70% of Behcet's Disease patients had nailfold capillaroscopy abnormalities , a highly statistically significant changes in the capillaries in the form of capillary dilatation (24%), hemorrhage (54%), tortuosity (64%) in comparison to the control group (P<0.001). However, pattern was not specific as BDCAF Score wasn’t related to the capillary changes. Conclusion: Nailfold capillaroscopy showed nonspecific pattern in 70% Behcet's disease patients. Tortuosity, hemorrhages, dilation were not related to disease activity. Capillaroscopy is a good primary test for Bechet’s disease and can reflect the presence and extent of microvascular involvement and thereby might have diagnostic and prognostic value.

Clusters in Pediatric Rheumatic Diseases.

Rheumatic diseases usually have very diverse and complex pathophysiology resulting in different clinical phenotypes. Some certain phenotypes cluster together with a common presentation, course, and outcome. Our primary aim is to review the known disease clusters in certain rheumatological conditions. Cluster analysis has been reported in several rheumatic diseases, Behcet disease being the most common. Five individual clusters together with some clinical associations have been reported in Behcet patients. Certain antibody-phenotype associations and damage clusters have been reported in systemic lupus erythematosus (SLE) patients as well. Although there is no proper cluster analysis, two distinct phenotypes are evident in deficiency of ADA2 (DADA2): those with a polyarteritis nodosa-like vasculopathy and hematological involvement with pure red cell aplasia or bone marrow failure. The variants are claimed to be different regions of this gene for these 2 phenotypes. Lastly, there is a recent paper grouping ANCA-associated vasculitis according to disease phenotype and antibody associations. There are distinct clusters in certain rheumatic diseases which might help us to predict comorbidities, disease course, and treatment response.