Involvement In Behcet's Disease Research Articles

Significant association of HLA-A26 with uveitis and gastrointestinal involvement in patients with Behçet's disease in a multicenter study.

Specific HLA haplotypes are associated with Behçet's disease (BD). Because the effects of HLA-A26 and its combination with HLA-B51 on organ involvement in BD have not been well demonstrated, we aimed to examine them. This multicenter, cross-sectional, observational study enrolled patients with BD who visited Kyoto University Hospital between 2018 and 2021 or Kurashiki Central Hospital between 2006 and 2016 (n = 200). Disease severity was evaluated using the Krause score. Uveitis and gastrointestinal involvement were observed in 95/196 and 57/167 patients, respectively. The HLA alleles identified were HLA-B51 (n = 52/106), HLA-A26 (n = 25/88), and HLA-B51 and HLA-A26 (n = 6/88). In patients harboring HLA-B51, the presence of HLA-A26 was associated with higher frequencies of uveitis (p = 0.03) and coexistence of uveitis and gastrointestinal involvement (p = 0.002), and higher Krause scores (p = 0.02). Furthermore, the presence of HLA-A26 was associated with a higher frequency of uveitis in patients with gastrointestinal involvement (p = 0.001) and gastrointestinal involvement in patients with uveitis (p = 0.001). Since specific HLA haplotypes and their combinations are associated with organ involvement, both HLA-A and HLA-B haplotypes should be confirmed when screening for affected organs.

Behçet Hastalarında Kardiyak Tutulum: Retrospektif Tek Merkez Deneyimi

Behcet’s disease (BD) is well-known with mucocutaneous involvement, whereas the heart may rarely be involved, predicting morbidity and mortality. In this study, we aimed to reveal the clinical characteristics of cardiac involvement in BD. We retrospectively screened 800 BD patients diagnosed between 2000 and 2021 for cardiac involvement. 14 patients who met these criteria were recruited in this study. Demographic information, clinical features, treatment modalities, and prognosis were evaluated. All patients were male and smokers. The mean age for cardiac involvement was estimated at 32.5 ±7,8 years. Cardiac involvement developed in one-third of the patients before disease diagnosis. Patients were classified into three major groups: 8 of intracardiac thrombosis (ICT), 2 of coronary artery aneurysms, and 4 of myocardial infarction (MI). The majority of ICT was seen in the right ventricle (6 out of 8). In all MI cases, the left main coronary artery was totally occluded. Deep vein thrombosis was seen in 57% of patients. Apart from steroids, cyclophosphamide was the most common preferred agent, used in 9 patients. Azathioprine and interferon use were seen in 4 cases. Warfarin was used in 10 patients and 4 cases received an antithrombotic agent. Mortality was seen in 2 cases due to unknown causes. Cardiac involvement is rare, but a serious manifestation of BD. ICT was the most common type with mostly involved the right heart chambers. Male gender and smoking were found as the most important associated risk factors in this population.

Hypertension as a prominent manifestation secondary to renal artery lesions in pediatric Behcet’s disease

ObjectiveHypertension caused by vascular Behcet’s disease (BD) is an important prognostic factor of paediatric BD. However, much less is known about its clinical features. The objective of this study was to investigate the clinical characteristics of paediatric vascular BD complicated by hypertension.MethodsA retrospective study was carried out in paediatric BD patients complicated by hypertension treated in the Children’s Hospital Capital Institute of Paediatrics from Jan 2009 to Dec 2022.ResultsOf 65 BD patients, 6 (9.2%) were complicated by hypertension, 5 patients were female, and the median ages of onset and diagnosis were 9.8 years and 11.3 years, respectively. Three patients were found to have cardiac involvement and hypertensive retinopathy secondary to hypertension. Five of the 6 patients with hypertension had right renal artery involvement, and all of them were treated with glucocorticoids and immunosuppressants. Four patients were treated with biological agents. One patient with severe renal artery stenosis underwent unsuccessful vascular interventional therapy. After 3–6 years of follow-up, five patients were found to have renal atrophy, and one patient was at stable condition.ConclusionHypertension in paediatric BD is mainly caused by renal artery involvement. Early recognition and treatment of vascular involvement in BD is important to prevent poor prognosis.

Indications for Magnetic Resonance Imaging in Patients With Behcet Uveitis.

Behcet disease is a systemic vasculitis, which may involve the eyes and central nervous system. The true prevalence of neurological involvement is not precisely known but may be associated with ocular involvement. This study investigates the association between Behcet uveitis and neuro-Behcet disease. A retrospective single-center analysis was conducted for consecutive patients with Behcet uveitis at the Massachusetts Eye Research and Surgery Institution. Uveitis characteristics, neurological symptoms, fluorescein fundus angiography, and MRI results were recorded. Our population included 108 patients with Behcet uveitis, and 26 (24.1%) were found to have neurological involvement associated with Behcet disease. Optic nerve leakage on fundus angiography and neurological symptoms were associated with an increased risk of neurological involvement. Three cases (11.5%) were nonparenchymal, while 23 (88.5%) were parenchymal with lesions in the cortex, subcortical white matter, thalamus, basal ganglia, and brainstem. There is a high comorbidity between ocular and neurological involvement in Behcet disease. Careful assessment of neurological symptoms and baseline fluorescein fundus angiography are recommended for patients with Behcet disease. MRI has a high diagnostic yield and should be pursued if there is concern for progressive or pre-existing neurological involvement.

Predictive factors for cardiovascular involvement in behcet disease: A retrospective Tunisian cohort

Predictive factors for cardiovascular involvement in behcet disease: A retrospective Tunisian cohort

Serum Elafin as a Potential Marker of Disease Activity in Behçet's Disease.

Elafin is a serine protease inhibitor with anti-inflammatory properties. It is expressed in various epithelial tissues with increased production under inflammatory conditions. Increased tissue elafin expression in Behçet's disease (BD) lesions has previously been demonstrated. We hypothesised that serum elafin might be increased in patients with BD and aimed to assess the relationship of serum elafin with disease activity and organ involvement in BD. Fifty-four BD patients (29 active, 25 inactive) and 30 healthy controls were included in this single-centre case-control study. Serum elafin was measured using ELISA. Complete blood count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) tests were also performed. Serum elafin levels were significantly higher in patients with BD (1.58 ± 0.47 ng/ml, range: 0.67-2.96) compared to controls (1.10 ± 0.28 ng/ml, range: 0.65-1.49) (P < 0.001). Patients with active BD had higher elafin levels than patients with inactive BD (P = 0.008). Active arthritis was associated with an increase in elafin (P = 0.012), while the presence of mucocutaneous symptoms was not. Serum elafin correlated significantly with ESR (P = 0.001). The ideal cut-off value for the diagnosis of BD was determined as 1.24 ng/ml with a sensitivity and specificity of 72.2% and 70.0%, respectively. Serum elafin is significantly increased in patients with BD. It may serve as a marker of disease activity, especially articular involvement.

Transthoracic echocardiographic assessment of cardiac valves in patients with Behçet's disease.

The main objectives of the current study are to investigate valvular involvement in patients with cardiac Behçet's disease (BD) and find out the risk factors of valvular involvement in cardiac BD. We retrospectively assessed the clinical and echocardiographic data in the medical records of 121 patients with BD admitted to Beijing Anzhen Hospital from January 2015 to January 2022. We evaluated the valvular structure and function mainly by echocardiography. A total of 77 BD patients (77/121, 63.64%) had cardiac valvular involvement. Valvular lesions occurred more frequently in males (p = 0.022). Aortic regurgitation (AR) (62/77, 80.52%) was the most common finding and severe AR occupied 80.65% (50/62). The most common manifestations of BD patients with severe AR was aortic valve prolapse (25/50, 50%), followed by echo-free spaces within the aortic annulus (11/50, 22%), vegetation-like lesions (10/50, 20%), and aortic root aneurysm (10/50, 20%). The incidence of paravalvular leaks (PVL) in BD patients was 14.29% (7/49). The diameter of the sinus of Valsalva and proximal ascending aorta, and total cholesterol (TCHO) were the independent risk factors of moderate-severe aortic valvular regurgitation (p < 0.01). Left ventricular end-diastolic dimension (LVEDD), left ventricular ejection fraction (LVEF) and brain natriuretic peptide (BNP) were significantly associated with moderate-severe mitral valvular regurgitation (p < 0.01). The most common valvular abnormality in BD is AR. Echocardiography has great value in the comprehensive evaluation and accurate diagnosis of valvular involvement in BD patients.

Behçet's Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement.

Behcet's disease (BD) is a chronic systemic inflammatory disorder characterized by underlying chronic vasculitis of both large- and small-caliber vessels. Thoracic involvement in BD can occur with various types of manifestations, which can be detected with contrast-enhanced MSCT scanning. In addition, MR can be useful in diagnosis. Characteristic features are aneurysms of the pulmonary arteries that can cause severe hemoptysis and SVC thrombosis that manifests as SVC syndrome. Other manifestations are aortic and bronchial artery aneurysms, alveolar hemorrhage, pulmonary infarction, and rarely pleural effusion. Achieving the right diagnosis of these manifestations is important for setting the correct therapy and improving the patient's outcome.

Characterisation of circulating endothelial microparticles in Behçet's disease: new markers of chronic endothelial damage?

Endothelial cell-derived microparticles (EMPs) are directly indicative of endothelial cell activation or apoptosis, and may also reflect endothelial inflammation, increased coagulation, and vascular tone. The aim of this study is to investigate whether EMPs would be able to evaluate system involvement and be a new indicators of disease activity in Behçet's disease (BD). Thirty-nine consecutive BD patients (who fulfilled the modified 1990 International Study Group on Behçet's disease or the 2006 International Criteria for Behçet's Disease) and 30 age- and sex-matched healthy controls were enrolled. The plasma levels of EMPs were measured by flow cytometry utilising specific labels for endothelial MPs (CD31+ and CD42b-). The levels of circulating EMPs (CD31+ and CD42b-) were significantly elevated in the case group compared with the healthy control group (p =0.000). Moreover, BD patients plasma EMPs were positively correlated with BD current activity form (r=0.802, p =0.000). Vascular and gastrointestinal involvement in BD patients were significantly increased (p=0.004 and p=0.011, respectively) with respect to patients without vascular and gastrointestinal EMPs. Levels of circulating EMPs are elevated in BD patients and correlate with the disease activity; the elevated EMPs may be a potential indicator to predict disease activity of BD. The plasma level of EMPs was increased, which indicated the increased risk of vascular and digestive tract involvement in BD.

Alterations in right ventricular mechanics in patients with Behcet's disease.

Manifest myocardial involvement is somewhat rare in patients with Behcet's disease (BD), although echocardiographic studies suggest that subclinical alterations in left ventricular (LV) contractility is rather common. Data on right ventricular (RV) involvement in BD is rather scarce.This study aims to determine whether RV systolic performance is affected in BD patients, and to understand the clinical and echocardiographic correlates of RV contractility in these patients.Forty-five patients who fulfilled criteria for BD and 45 age and gender matched controls were enrolled. All participants underwent a comprehensive echocardiographic examination, including deformation imaging, to characterize RV mechanics.Conventional morphologic and echocardiographic indicators of RV morphology and function were not different between groups, but RV apical strain and RV free wall strain (FWS) were significantly lower in BD patients as compared to the controls (P < 0.001 and P = 0.02, respectively). The only significant correlates of FWS were tricuspid regurgitation velocity and related indices in healthy controls, while FWS correlated with LV global longitudinal strain (GLS), morphologic measures of left and right atria and ventricles, and with conventional measures of right ventricular contractility. The relationship between FWS and GLS remained statistically significant after adjusting for other clinical and echocardiographic parameters (β = 0.379, P = 0.01).In patients with BD, there is a subclinical alteration in RV contractility and the degree of alteration in the RV systolic performance paralleled that of LV. Thus, present results support the presence of RV involvement in these patients.

Clinical characteristics and predictors for cardiovascular system involvement in patients with Behçet's disease.

The purpose of this study is to analyse the clinical characteristics of Behçet's disease (BD) patients in China with or without cardiovascular system involvement, and to develop a risk model to identify factors related to cardiovascular involvement in BD. This retrospective cohort study, using the information on BD in Shenzhen People's Hospital from January 2000 to December 2021, included 95 patients; BD patients without cardiovascular system involvement (n=63) and with cardiovascular system involvement (n=32). Patients with BD who were males and had a combination of hypertension and a longer duration of disease were more likely to have cardiovascular involvement (p < 0.05). Compared to patients without cardiovascular involvement, manifestations of genital ulcers are rarely observed in those with cardiovascular involvement (60.32% vs. 37.50%, p=0.035). The binary logistic regression analysis found that ascending aortic widening and a history of hypertension were independent risk factors for BD with cardiovascular system involvement (OR=1.277, 95% CI 1.09, 1.495, p=0.002; OR=11.578, 95% CI 1.308, 102.639, p=0.028). The established prediction model indicated that can help to predict the likelihood of cardiovascular involvement in a patient with BD. Cardiovascular involvement in BD is not rare at all, however, it is often underreported due to a lack of specificity. BD patients who are male, have a history of combined hypertension, and have a long duration of disease should be focused on the presence of combined cardiovascular system involvement, with particular attention to the patient's ascending aortic internal diameter.

Clinical characteristics and radiographic outcomes of vascular Behçet's disease involving the aorta or its major branches.

To describe the clinical characteristics and radiographic outcomes of vascular Behçet's disease (BD) involving the aorta or its major branches. This retrospective cohort study was performed in patients with vascular BD involving the aorta or its major branches. All included patients underwent computed tomography angiography (CTA) at least two times with a 2- to 5-year interval. Radiographic progression was defined as newly developed and/or aggravated (> 20%) characteristic features on CTA. The cohort included 22 patients with BD with a median interval of 3.65years between the initial and follow-up CTA. Five patients (22.7%) showed radiographic progression. Patients with radiographic progression had a longer disease duration at baseline than those without (6.67 vs. 0.26years, p = 0.028). Of all patients, 21 (95.5%) had vascular aneurysms/pseudoaneurysms and 11 (50.0%) had thrombosis. The most frequently involved artery with aneurysmal change was the abdominal aorta (8/21, 38.1%), followed by the iliac arteries (5/21, 23.8%). In the case of thrombosis, the most frequently involved arteries were the femoral (4/11, 36.4%) and iliac (4/11, 36.4%) arteries. The characteristics and locations of vascular involvement did not significantly differ according to the radiographic outcome. A considerable proportion of patients with BD with arterial involvement showed radiographic progression within 2-5years. Patients with radiographic progression had a longer disease duration at baseline. The most common form of arterial involvement of BD was aneurysmal change, followed by thrombus formation. • This study evaluated for the first time the radiographic outcomes of 22 patients with Behçet's disease involving the aorta or its major branches. • A considerable proportion of patients (5/22, 22.7%) showed radiographic progression. • Patients with radiographic progression had a longer disease duration at baseline than their counterparts; however, no other clinical factors were significantly different. • The most frequent form of vascular involvement was pseudoaneurysm followed by thrombosis.

INTRA-CARDIAC THROMBUS IN BEHCETS DISEASE: TWO CASE REPORTS

Behcets disease (BD) is a multi-systemic inflammatory disorder characterized by a recurrent oral and genital aphthosis associated with multiple organ disorders: ophthalmic, neurological, vascular, and digestive.Cardiac involvement in BD especially Intra-cardiac thrombus (ICT) is extremely rare only 50 cases have been registered worldwide [1-5]. We report a rare case of two patients aged respectively 18-year-old and 50-year-old who are diagnosed with Behcets disease complicated by right-sided heart thrombi extended to the pulmonary artery.

Clinical presentation and treatment outcomes of arterial involvement in Behçet's disease: a single-centre experience.

Arterial involvement, although rare, accounts for significant mortality and morbidity in patients of Behçet's disease (BD). There is paucity of data on arterial BD. The objective of this5-year retrospective cohort study was to examine the clinical presentation, pattern of arterial involvement, and treatment outcome in Indian arterial BD patients. Data on demography, clinical presentation, radiology, instituted therapy, vascular interventions and treatment outcomes were recorded and analyzed. Ten (16.9%) out of 59 patients with BD had arterial involvement in 13 vascular territories [mean age 30 (8) years, 9 (90%) males]. Pulmonary artery was most commonly involved (46%), followed by abdominal aorta (15%), femoral artery (15%), descending thoracic aorta (8%), common iliac (8%), and dorsalis pedis artery (8%). Two patients had multi-territory involvement. The median interval between disease onset and development of arterial aneurysms was 3years (3months-12years). Concomitant deep vein thrombosis was seen in 60% cases. Prednisolone and cyclophosphamide were the most common immunosuppressive therapy used; one patient who relapsed on cyclophosphamide responded to infliximab. Five surgical or endovascular interventions were performed. Four patients (40%) died due to aneurysm rupture-all had a delayed diagnosis, and three had pulmonary artery involvement, with death due to massive hemoptysis. Based on the present study, we concluded thatarterial involvement in BD is seen predominantly in males and has a high mortality. Early detection and aggressive treatment with immunosuppression and surgical or endovascular interventions are essential for good outcomes.

Cardiac Manifestations in Iranian Patients with Behçet's Disease.

Background: Behcet’s disease (BD) is a vasculitis with multisystem and multiorgan involvement. Cardiac involvement in BD is a rare complication with a poor outcome that manifests itself in different forms. In this study, we aimed to investigate cardiac involvement in BD. Methods: This is a retrospective study based on cardiac manifestations in BD according to the data of the Behçet’s Disease Unit, the Rheumatology Research Center, Tehran University of Medical Sciences, from registered patients from 1975 until June 2017. Cardiac manifestations consisted of pericarditis, myocardiopathy, myocardial infarction, stable ischemic heart disease, endomyocardial fibrosis, thrombosis, and valvular and coronary involvement. All the patients’ baseline and demographic data were recorded in a designed questionnaire. The laboratory workups, imaging, and pathological tests were also performed. Results: We studied 7650 patients with BD, of whom 51% were male. In the entire study population, 47 patients manifested cardiac involvement: valvular involvement in 6.1%, myocardial infarction in 23.4%, stable ischemic heart disease in 20%, pericarditis in 21.3%, intracardiac thrombosis in 2.1%, coronary aneurysm in 2.1%, heart failure in 12.8%, and dilated cardiomyopathy in 4.3%. Conclusion: The prevalence of cardiac involvement in our patients with BD was 0.6%. A multidisciplinary approach can reduce mortality and morbidity rates. Consequently, we suggest that echocardiography and other cardiac diagnostic tests be routinely considered for early diagnosis and subsequent treatment.

Incidence of ophthalmic involvement in Behçet's disease in the United Kingdom: a British Ophthalmic Surveillance Unit (BOSU) study.

Behçet's disease (BD) is a relapsing-remitting vasculitis, which can manifest in different organ systems including the eyes. There is currently limited published data describing the incidence of ophthalmic disease within the United Kingdom. The primary aim of this study was to survey the incidence and manifestations of ophthalmic BD prospectively, with a secondary aim of reviewing treatment modalities initiated in first-line therapy. Using the British Ophthalmic Surveillance Unit reporting system between October 2016 and November 2018, we prospectively surveyed the number of cases of BD presenting to UK ophthalmologists. A total of 89 cases of ophthalmic manifestations of BD were reported and complete information was collected on 58 patients. 93 eyes of 58 patients were affected. The median age of reported cases was 31 years (range 13-55 years) who were born in 15 different countries. Most cases (n = 35, 60%) had bilateral involvement. Vitritis was the most common ocular manifestation (68%; n = 63) followed by anterior uveitis (46%; n = 43). The greatest causes of visual morbidity were cystoid macular oedema, vitritis and retinal ischaemia. Most patients were prescribed either topical or oral corticosteroids (59%; n = 34), with some given intravitreal or intravenous corticosteroids. Five patients (8.6%) were initiated on disease-modifying anti-rheumatic drugs and one given an anti-TNF monoclonal antibody. This is the first prospective study to analyse the incidence of ophthalmic involvement in BD over a 2-year period, finding an annual incidence of 0.04 per 100,000 individuals in the UK.

Neuro-Behçet is a rare disease but should be considered in all kinds of neurological findings, even in childhood.

Behçet's disease (BD) is a vasculitis characterised by eye, musculoskeletal, neurological and gastrointestinal involvement, in addition to recurrent oral ulcers. Neuro-Behçet is the term used to define the nervous system involvement in BD and is very rarely seen in childhood. This study aims to show that neuro-Behçet can manifest a clinical course involving all kinds of neurologic findings in the paediatric population. The Clinic of Paediatric Neurology at Uludag University provides tertiary treatment for children up to eighteen years of age in Bursa, Turkey. Five patients who were clinically diagnosed with Neuro-Behçet in the last 5 years were included in the study. Seizure, myopathy, transverse myelitis, polyneuropathy, venous thrombosis and facial nerve paralysis were respectively seen in the patients. Neuro-Behçet is rare in children, but it is important to know that it can cause various neurological findings, and also systemic findings should be taken into consideration in the diagnosis of neurological diseases. Studies on the neurological involvement of BD in children are inadequate. We believe that paediatric neurologists should be more aware of the neuro-Behçet condition.

Successful treatment of refractory mucocutaneous involvement in a Behçet disease patient with apremilast

Abstract Rationale: Apremilast is currently approved and used for oral ulcers in Behçet disease (BD). The direct comparisons of the efficacy between tumor necrosis factor (TNF) inhibitors and apremilast has not been conducted, and the guidelines do not address the order in which therapies should be used. Colchicine is more often recommended to be used first, and the other drugs are recommended to be chosen based on the overall condition of the patient. Patient concerns: A 44-year-old male with relapsing genital ulcers, oral ulcers, arthritis, and pustular skin lesions for unknown reasons for several years was transferred to our hospital presenting with generalised seizures. Diagnoses: Laboratory results showed an elevated inflammatory response. The elevated cell count and interleukin-6 levels were detected in cerebrospinal fluid. After excluding the other causes, the patient was diagnosed with meningoencephalitis due to acute neuro-BD. Interventions: Encephalitis and mucocutaneous lesions were ameliorated by glucocorticoids (prednisolone), but the mucocutaneous lesions flared-up after reducing the dose of glucocorticoids. TNF inhibitors and immunosuppressive drugs were not effective. Hence apremilast at a dose of 60 mg was initiated. Outcomes: Apremilast markedly attenuated mucocutaneous lesions, which disappeared within one month. There was no recurrence of mucocutaneous lesions. Apremilast was continued but glucocorticoids were discontinued and has not been re-administered for more than a year. Lessons: Apremilast might improve refractory mucocutaneous involvement, even in cases where TNF inhibitors and glucocorticoids are ineffective. Although the guidelines do not address the order in which therapies are used, apremilast can be considered beforeTNF inhibitors in selected cases of mucocutaneous involvement in BD.

Variation of red blood cell parameters in Behcet's disease: association with disease severity and vascular involvement.

Behcet's disease (BD) is a systemic and chronic inflammatory vasculitis with unknown etiology. Diagnosis is determined by evaluating several clinical criteria, but the lack of specific laboratory diagnostic markers makes the diagnosis of BD more difficult. Therefore, the aim of this study was to determine the changes in hematological parameters in BD patients to investigate their relationship with BD clinical features. A total of 48 BD patients and 96 healthy controls were included in this study. The severity of each BD patient was associated to a severity score according to the entire spectrum of disease manifestations. Several laboratory tests were assessed, and the difference in their results between BD patients and healthy controls was evaluated. Correlation analysis was performed to reveal the interaction of these parameters. C-reactive protein (CRP), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), neutrophil count, platelet count, and plateletcrit significantly increased in BD patients compared with healthy controls (P < 0.05). CRP was higher in patients with skin lesions, MCH and MCHC were lower in patients with vascular involvement, and the neutrophil count was higher in patients with skin lesions and genital ulcers. In addition, higher CRP and lower MCH and MCHC were associated with a severe condition. Besides, MCH and MCHC were negatively correlated with the platelet count, plateletcrit, and neutrophil count. This study revealed that MCH and MCHC are valuable parameters for BD. Their levels help assess the disease severity and indicate the vascular involvement in BD. Key Points • This is the first study reporting MCH and MCHC as important biomarkers in BD. • BD patients with vascular involvement and thrombosis potential have lower levels of MCH and MCHC. • MCH and MCHC are negatively correlated with platelet count, plateletcrit, and neutrophil count. • Lower MCH and MCHC are associated with a severe condition.

Mucocutaneous Involvement in Behcets Disease

Mucocutaneous Involvement in Behcets Disease