Search results for "Behcet's disease"
showing 10 items of 20 documents
Associated arterial and venous cerebral manifestations in Behçet's disease
2018
Behcet's disease is a rare multisystemic vasculitis with an etiology that is still unknown. Neurological manifestations may be seen in approximately 5-15% of patients, and both parenchymal and extraparenchymal neurological involvement has been described. When cerebral venous thrombosis (CVT) is the main extraparenchymal manifestation of Behcet's disease, the condition is then dubbed "angio-Behcet's syndrome". However, arterial involvement is extremely rare, with only one reported case of vasculo-neuro-Behcet's disease, characterized by both venous and intracranial arterial involvement - until now. This report is of two patients diagnosed with Behcet's disease characterized by the concomitan…
Risk factors for cognitive impairment in subjects with Behçet's disease
2004
In the present study we evaluated the prevalence and risk factors for cognitive impairment in subjects with Behçet's Disease (BD). The following risk factors were studied: age, education, disease duration, activity of disease, prednisone dosage, and anxiety and depression levels. Twenty-six BD out-patients without overt neurological involvement and 26 sex-matched controls completed a comprehensive neuropsychological battery. Compared to controls, BD subjects were significantly impaired on tasks evaluating long-term verbal and nonverbal memory, and visuospatial skills. Cognitive impairment was evident in 46.1% of BD patients compared with none of control subjects (p<.0001), with memory repr…
Massive gastrointestinale Blutung eines 28-jährigen Patienten mit intestinalem Befall eines Morbus Behçet
2006
A 28-year-old man was admitted to the emergency room of our hospital after syncope and acute gastrointestinal bleeding. On the basis of numerous oral and genital ulcerations as well as uveitis anterior and erythema nodosum, the diagnosis of Behcet's disease had been confirmed previously. The bleeding focus could not be detected by endoscopy. During the next days recurrent massive hemorrhages occurred in spite of immunosuppressive therapy with steroids. Angiography revealed a circumscribed bleeding source in the colon transversum near the left colonic flexure, which was treated by superselective coil embolization. A massive hemorrhage reoccurred and required a surgical approach with a Hartma…
A national cohort study on pediatric Behçet's disease: Cross-sectional data from an Italian registry
2017
Abstract Background Behçet’s disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years, but it may be diagnosed before the age of 16. It is not very rare in Italy, even though there are limited data concerning epidemiology. Aim of this study is to describe the baseline data of an Italian cohort of patients with as having BD or probable BD. Methods We described the baseline data of the first national epidemiological study on children coming from 16 Italian Pediatric Rheumatologic Centers diagnosed by the treating physicians as having Behçet’s Disease. …
Migraine without aura as a possible sign of covert neurological involvement in Behçet's disease: A case-control study
2004
In the present study we evaluate the prevalence of headache and the frequency of different primary headaches in patients with Bebçet's Disease (BD) without neurological involvement. Furthermore, we investigate the relationship between headache with other clinical, and behavioural variables. Twenty-seven BD patients and 27 control subjects underwent a validated semi-structured questionnaire based on the International Headache Society criteria. Levels of anxiety and depression, disease activity, and current medication were collected. Headache occurred in about 90% of BD patients. There was no difference in the prevalence of the different headache syndromes between BD patients and controls. O…
Computer tomography features of pulmonary vascular involvement in Behçet's disease
2019
Behçet’s disease is a chronic and multisystemic inflammatory vascular disease of unknown etiology, affecting vessels of all kinds and sizes. The involvement of the vascular system is observed in approximately 5-30% of patients. The most frequent vascular manifestations are venous thrombosis followed by arterial aneurysms and arterial thrombosis. The identification of vascular involvement is very important for prognosis, indeed the rupture of an arterial aneurysm represents the leading cause of sudden death in patients with Behçet’s disease. Computed tomography (CT) plays a crucial role in the identification and in the follow-up of vascular involvement. In this paper, we report the case of a…
Number VII Behcet's disease (Adamantiades syndrome)
2006
Behçet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations may also be seen. BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101). Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated. An inflammatory disorder, BS is now considered as a systemic vasculitis, c…
New approaches in the treatment of Adamantiades-Behçet's disease.
2005
Purpose of review To update clinicians on the recent advances in the treatment of Adamantiades-Behcet's disease. Recent findings Interferon-α-2a and infliximab have proved able to induce prompt remission in the vast majority of Adamantiades-Behcet's patients with DMARD-resistant uveoretinitis. Efficacy of interferon-α-2a has also been reported for mucocutaneous lesions, arthritis, and (more anecdotally) for neuro-Behcet, while results from small case series suggest that infliximab is beneficial for mucocutaneous lesions and (more anecdotally) for arthritis and gastro-intestinal manifestations. Two cases of neuro-Behcet treated with infliximab showed a complete resolution. Finally, in a rand…
Factor V Leiden and prothrombin gene G20210A mutations in Italian patients with Behçet's disease and deep vein thrombosis
2004
Objective To evaluate the frequency and type of vascular lesions and to study the association of factor V gene G1691A (Leiden) and prothrombin gene G20210A polymorphisms with venous thrombosis in Italian patients with Behcet's disease (BD). Methods Included were 118 consecutive Italian BD patients followed over a 3-year period (1997–1999) who satisfied the International Study Group criteria for BD. The control group consisted of 132 healthy Italian blood donors. All BD patients and controls were genotyped by polymerase chain reaction and allele-specific restriction enzyme techniques for factor V Leiden and prothrombin gene G20210A polymorphisms. Results Vascular lesions were observed in 37 …
Role of Human Leukocyte Antigens (HLA) in Autoimmune Diseases
2018
Since the discovery of HLA 60 years ago, it has contributed to the understanding of the immune system as well as of the pathogenesis of several diseases. Aside from its essential role in determining donor-recipient immune compatibility in organ transplantation, HLA genotyping is meanwhile performed routinely as part of the diagnostic work-up of certain autoimmune diseases. Considering the ability of HLA to influence thymic selection as well as peripheral anergy of T cells, its role in the pathogenesis of autoimmunity is understandable. The aim of this paper is to provide a brief overview of the role and current clinical relevance of HLA-B27 in spondyloarthritis and HLA-B51 in Behçet's disea…