Behçet’s Disease with Severe Arterial Involvement in a Child

Beh?et's disease is a vasculitis characterised by its thrombotic tendency. In some patients, manifestations of vascular lesions may dominate the clinical picture instead of the classic triad. We describe a 13-year-old boy with a 1.5-year history of Beh?et's disease presenting with complaints of acute abdominal pain, severe headache and decreased vision. His work-up revealed a saccular aneurysm of the distal abdominal aorta, thrombosis in the right common iliac, external iliac and femoral arteries, and thrombosis of the superior sagittal sinus. Aortoiliac bypass with a Dacron graft was performed with success. He also received cyclosporin A and anticoagulant therapy. Severe vasculitis may become overt at any age in patients with Beh?et's disease. Early diagnosis and management is important to prevent morbidity and mortality.     

Behçet’s syndrome: An update

The pathogenic mechanisms in Behçet’s syndrome are largely unknown. An autoantigen role for human leukocyte antigen B51 has been proposed. The reasons behind the thrombophilia are also not clear. Endothelial pathology could be the main culprit. The recently proposed association between familial Mediterranean fever and Behçet’s syndrome might not be well founded. The long-term prognosis is more guarded among the young and among males. However, the disease burns out in many cases. Clinicians are getting better at management, and have better understanding of the old drugs, such as colchicine, and have new and potent drugs like tumor necrosis factor-alpha inhibitors.     

Transient involvement of the cerebral cortex in a flare of Behçet’s syndrome

We describe a flare of Behçet’s syndrome in a 42-year-old man who presented with florid mucocutaneous manifestations, headache and vomiting, but without meningeal or neurologic signs. A single, non-enhancing cortical lesion was found in the frontal lobe by computed tomography and magnetic resonance (MR) imaging studies. Diffusion-weighted analysis and MR spectroscopy were consistent with focal inflammation. The patient improved with colchicine, and after 5 months, a repeat MR scan demonstrated resolution of the inflammatory changes suggesting that involvement of the cerebral cortex may be a self-limiting phenomenon, distinct from other more frequent and destructive parenchymal manifestations of neuro-Behçet’s disease.     

Coronary involvement in patients with Behçet’s disease

Clinical Rheumatology - To investigate the clinical features and potential risk factors of coronary involvement in Behçet’s disease (BD). In this case-control study, we retrospectively...     

Cerebral venous sinus thrombosis in Behçet’s disease: a retrospective case-control study

The objective of the study is to investigate the clinical features of Behçet’s disease (BD) complicated with cerebral venous sinus thrombosis (CVST). We retrospectively analyzed the clinical features of age- and gender-matched BD patients with and without CVST. Of the 840 BD patients enrolled, 21 (2.5%, 11 female, mean age 30.3 ± 9.5) were diagnosed as CVST. Eighteen patients (85.7%) developed neurologic manifestations after BD onset, with an average interval of 47.6 months (SD 36.1). Onset of neurologic symptoms was mostly progressive (89.5%), with headache (95.2%) being the most common symptom. Intracranial hypertension occurred in 17 cases (89.5%). Thrombosis was detected mostly in transverse (71.4%) and superior sagittal (66.7%) sinuses, with more frequently (66.7%) dual or multiple sinuses involved. In addition, extra cranial thrombosis was presented in 11 cases. Cerebral infarction was presented in four cases. Compared with BD patients without CVST, the prevalence of extra cranial vascular involvement (52.4 versus 20.6%; P = 0.005) and BDCAF 2006 score (3.7 ± 1.4 versus 1.8 ± 1.3; P = 1.7863 × 10^?7) was higher in BD patients with CVST. During a median 7-month follow-up (range 0.3–88.8 months), 18 patients (85.7%) achieved remission following glucocorticoid, immunosuppressant therapy, anticoagulants, and dehydration, although 3 patients experienced (14.3%) flare. No death was reported. CVST is rare complication of BD and predominately progressively developed in active BD and after BD onset. It typically features intracranial hypertension syndrome and is frequently complicated with extra cranial thrombosis; thus, systemic thrombus screening is highly recommended. Promptly, diagnosis and aggressive treatment are keys to improve the prognosis.     

Chylothorax and chylopericardium in Behçet’s diseases: case report and literature review

Behçet’s disease (BD) with chylothorax and/or chylopericardium is uncommon. Here, we report a case of a 32-year-old man suffering BD with chylothorax and chylopericardium complications. We also review the literature and discuss clinical characteristics, possible pathogenesis, and treatment strategy of patients suffering BD with chylothorax and/or chylopericardium complications.     

Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients

Behçet’s disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5?±?11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P?=?0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities (n?=?49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation (n?=?25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta (n?=?19), lower extremity arteries (n?=?15), pulmonary arteries (n?=?13), coronary arteries (n?=?5), and subclavian arteries (n?=?5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P?=?0.024; 54.9 vs 76.5 %, P?=?0.000; 19.6 vs 30.5 %, P?=?0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P?=?0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis.     

Characteristics and phenotype heterogeneity in late-onset Behçet’s syndrome: a cohort from a referral center in China

Clinical Rheumatology - To evaluate the demographics, clinical aspects, and major organ involvement of patients with late-onset Behçet’s syndrome (BS) in a tertiary center in China. We...     

Smoking and Behçet’s Disease

It has recently been claimed that some of the symptoms in patients with Beh?et's disease (BD) can be activated after the patient has stopped smoking. In this study we investigated the effect of smoking on the symptoms of Beh?et's disease. Fifty asymptomatic current smokers (CS) who promised to stop smoking (group 1) and 60 current non-smokers (NS) (group 2) (21 of them ex-smokers) with BD were examined at the beginning and a week later for the presence of symptoms of BD. Forty-seven of the 50 CS completed the study. Oral aphthous ulcers were observed in 31 (65.9%) of them at the end of the study period. Besides oral aphthous lesions, genital ulcers were detected in two and erythema nodosum in two other patients. Only 15 (25%) group 2 patients developed oral aphthous ulcers during the study period. The difference between the frequencies of oral aphthous lesions in these groups was significant (p=0.0002). We concluded that cessation of cigarette smoking can activate the mucocutaneous symptoms, especially oral aphthous lesions, in patients with BD.     

Arterial aneurysms in Behçet’s disease patients: Frequency,clinical characteristics and long-term outcome

IntroductionVasculitis in Behçet’s disease (BD) is common and unique as it can involve blood vessels of any type and any size. Literature on the characteristics of arterial aneurysms (AA) is scanty.Aim of the workTo present the clinical characteristics and long-term outcome of BD patients with AA compared to those without.Patients and methodsThe medical records of 146 BD patients admitted to the Rheumatology Department, Cairo University Hospitals (2004–2017) were retrospectively revised. The frequency and anatomical location of arterial aneurysms were recorded and the associated clinical characteristics analyzed.ResultsThe mean age of the BD patients was 34.3 ± 8.02 years and they were 127 males and 19 females (6.7:1) with disease duration of 6.81 ± 3.67 years. Apart from oro-genital ulcerations; deep venous thrombosis was the most common associated manifestation while the musculoskeletal manifestations were the least. Twenty-seven (18.4%) patients had arterial aneurysms. All of them were males. Pulmonary artery was the most common artery involved (44.4%) followed by abdominal aorta. Surgical intervention was done for 40.7% patients. The mortality rate was 14.8% (4 patients) in those with AA while it was 2.5% (3 patients) in those without.ConclusionArterial aneurysms are common in Egyptian BD patients mostly males. Increased awareness of the rheumatologists and including appropriate imaging techniques for early detection of AA in BD are mandatory. Early detection and prompt treatment with immunosuppressives may improve the disease outcome.     

Behçet syndrome

PURPOSE OF REVIEW: Interest in Beh?et syndrome (BS), although the condition is rare in many parts of the world, is increasing as judged by the growing number of related publications and scientific meetings. RECENT FINDINGS: Recent work reconfirmed the following without further insight into their meaning in pathogenesis: males have a more severe course; one third of the patients have a thrombophilia; papulopustular lesions and arthritis go together; gammadelta T are increased; and HLA B51 is still the main genetic association. Presence of increased levels of antibodies to Saccharomyces cerevisiae and the proposal of alpha-enolase as the target antigen of antiendothelial cell antibodies, known to be present also in BS, are observations that might help in delineating the unknown pathogenesis. For management, preliminary yet promising experience with the use of anti-tumor necrosis factor agents is being reported. SUMMARY When compared with 2 decades ago, we know considerably more and can do more about BS.     

Behçet’s disease and Chiari’s network

Behçet’s disease (BD) is a chronic, multisystemic, inflammatory process of uncertain origin. Diagnosis criteria consist of recurrent oral ulceration plus two of the following: genital ulceration, ocular and skin lesions, and positive pathergy test. Pulmonary embolism and, especially intracardiac thrombi, are very rare. We report the case of a patient with BD presenting with bilateral pulmonary embolism and intracardiac mass in right atrium.     

Serum homocysteine level and eye involvement in Egyptian patients with Behçet’s disease

Aim of the workTo investigate the possible role of serum homocysteine in the eye involvement in Egyptian patients with Behçet’s disease (BD).Patients and methodsA case–control study was made on 27 patients with BD (17 males and 10 females with a mean age of 32.11 ± 7.16 years) and 19 healthy control subjects. All patients fulfilled the criteria of the International Study Group for BD. The patients were categorized as BD with eye involvement (n = 12), or BD without eye involvement (n = 15). Serum homocysteine and C-reactive protein levels were studied in all patients and controls.ResultsThe mean serum homocysteine concentrations were significantly higher in BD patients than in healthy controls (15.56 ± 3.52 and 7.32 ± 1.38 μmol/L, respectively; P < 0.001). Also, the mean serum homocysteine concentrations were significantly higher in BD patients with eye involvement compared to those without eye involvement (18.50 ± 3 and 13.2 ± 1.61 μmol/L, respectively; P < 0.001). CRP, as a marker of disease activity was significantly higher in BD patients than in controls (14.33 ± 8.28 and 3.21 ± 1.72 mg/L, respectively; P < 0.001), however, no significant difference (P = 0.213) in CRP levels was found between BD patients with or without eye involvement.ConclusionHomocysteine may play a role in BD patients with ocular involvement. Assessment of homocysteine may be important in the investigation and management of patients with BD, especially with ocular disease.     

Retinal venous occlusion in Behçet disease

PURPOSE: The aim of our study is to report three cases of retinal venous occlusion in patients with Beh?et disease and to discuss the physiopathology of this vascular accident. METHODS: In this retrospective study, out of a series of 32 patients suffering from Beh?et disease, we report three cases of retinal venous occlusion. General and ophthalmologic features are described. Treatment strategy is detailed for each case. RESULTS: There were 2 cases of branch retinal venous occlusion and one case of central retinal venous occlusion. Relapses and retinal neovascularization were the most common complications. CONCLUSION: The retinal venous occlusion in Beh?et's disease is a non-common but severe complication. Early and appropriate treatment is required to improve the functional prognosis.     

Etanercept therapy in Behçet’s disease

Study objective

The goal of the present study was to investigate patient outcome when using the TNF receptor fusion protein etanercept in addition to conventional immunosuppressive drugs in ameliorating disease intensity and reducing relapses in refractory Behçet’s disease (BD).

Patients and methods

A single center, prospective study was conducted over 1 year. A total of 15 patients with the established diagnosis of BS were enrolled (mean age: 36.5?±?6.75 years, mean disease duration: 3.86?±?1.30 years). Clinical features were classified as refractory if the patients failed to achieve the desired response within 6 months of immunosuppressive and oral glucocorticoid therapy or flare of lesions developed while on the maximum tolerable doses of these drugs. The study included 2 patients who were on previous infliximab therapy for refractory disease. Inflammatory biomarkers (ESR and CRP) were investigated.

Results

Baseline clinical features in the study prior to inclusion showed recurrent oro-genital ulcers were observed in 100?% of patients, the pathergy test was positive in 17.6?%, ocular involvement was observed in 86.7?%, and acne lesions were recorded in 73.3?%. The following values were also recorded: mean ESR 22?±?16.97 mm/h, mean CRP level 6.87?±?4.44 mg/l, mean visual analog score 5.46?±?1.55, and mean patient global score 5.13?±?1.30. At the beginning of the study, all patients were on oral prednisolone (mean dose: 20.16?±?11.81 mg/day), azathioprine (mean dose: 126.66?±?25.81 mg/day), and oral colchicine (mean dose: 1.08?±?0.10 mg/day), then etanercept was added at a regular weekly dose of 50 mg subcutaneously for 1 year. By 8 weeks, 100?% of the patients achieve the primary endpoint, which included clinical resolution of refractory mucocutaneous, joint, and active ocular lesions with normalization of the acute phase symptoms.

Conclusion

Patients with refractory BD who received a 12-month treatment with etanercept in addition to conventional immunosuppressive therapy achieved a good therapeutic response with successful reduction of oral prednisolone to a mean dose of 6.66?±?2.24 mg/day. No serious infections or drug-related adverse events reported.     

Major vessel involvement in Behçet disease

PURPOSE OF REVIEW: Large vessel vasculitis occurs in a subgroup of patients with Beh?et disease at high risk for disease-related morbidity and mortality. Recognition of patients at risk, early detection of vasculitis, and the need for aggressive treatment are essential for optimal care of these patients. The authors review the clinical spectrum and management of large vessel problems in Beh?et disease, highlighting contributions over the past year. RECENT FINDINGS: Vasculo-Beh?et patients are at risk for multiple vessel-related complications including thromboses, stenoses, occlusions, and aneurysms. A number of factors may contribute to thrombosis in individual cases, but the primary reason for clot seems to reside in the inflammatory process in the arterial wall, still incompletely understood. An appreciation for the challenges in the perioperative period requires the joint efforts of physicians and surgeons, and fuels the study of alternate, less invasive procedures for Beh?et patients. SUMMARY: Because of earlier recognition, aggressive medical treatment, and novel surgical procedures, the morbidity and mortality of large vessel vasculitis in Beh?et disease are beginning to change. In the absence of controlled treatment studies, reports of clinical experience remain an important source of information for clinicians. Identification of patients at risk for vascular complications remains a priority.