Search

Abstract Definition of the disease Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations. Epidemiology BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population. Clinical description The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations. Etiology The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated. Diagnostic methods Diagnosis is only based on clinical criteria. Differrential diagnosis It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn’s disease, Takayasu’s arteritis, polychondritis or antiphospholipid syndrome need to be considered. Management Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy. Prognosis The prognosis is severe due to the ocular, neurological and arterial involvement.

Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. It is a multifactorial disease, and several triggering factors including oral cavity infections and viruses may induce inflammatory attacks in genetically susceptible individuals. BD vasculitis involves different vessel types and sizes of the vascular tree with mixed-cellular perivascular infiltrates and is often complicated by recurrent thrombosis, particularly in the venous compartment. Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. These important therapeutic advances in BD have led us to propose French recommendations for the management of Behçet's disease [Protocole National de Diagnostic et de Soins de la maladie de Behçet (PNDS)]. These recommendations are divided into two parts: (1) the diagnostic process and initial assessment; (2) the therapeutic management. Thirty key points summarize the essence of the recommendations. We highlighted the main differential diagnosis of BD according to the type of clinical involvement; the role of genetics is also discussed, and we indicate the clinical presentations that must lead to the search for a genetic cause. [ABSTRACT FROM AUTHOR]

Background/aims Behcet's disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS). Data are lacking on the presentation and outcome of BCS related to BD. Methods We investigated the relationship between BD and BCS in 14 patients with both diseases and compared the results to 92 BCS patients without BD. Results Male gender (p = 0.003), North African origin (P = 0.007) and inferior vena cava obstruction (P <0.0001) were more frequent in patients with BD and BCS than in those with BCS alone and the plasma C-reactive protein level was higher (p = 0.003). Two of the patients with the combined diseases underwent recanalization of the vena cava and the hepatic veins, none received transjugular intrahepatic portosystemic shunts (TIPS), one received a surgical shunt and one underwent liver transplantation. TIPS were less frequent in patients with BD and BCS than in those with BCS alone (P = 0.019). Eighty six per cent of patients with BCS and BD received corticosteroids and immunosuppressive therapy. The 5-year transplantation-free survival rate was 63% in patients with BCS alone and 91% in those without BD (P = 0.11). In our series and in the literature, a high number of patients [12 (61.5%) and 11 (64.7%) respectively] treated with anticoagulation and corticosteroids and/or immunosuppressants did not require invasive treatment. Conclusion This study shows a higher frequency of IVC obstruction in patients with BCS and BD. Medical treatment with anticoagulation and immunosuppressive agents may improve the symptoms of BCS. Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD. [ABSTRACT FROM AUTHOR]

Abstract: Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal antiinflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvements, more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, and neurological and gastrointestinal involvements. However, some patients still have refractory disease, relapse, sight threatening eye disease, or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future biological therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of biotherapies provides the possibility of interfering with specific pathogenic pathways. Novel targeted biotherapies might be used in the future for BD. [Copyright &y& Elsevier]

Background: Behçet’s disease (BD) significantly increases morbidity and mortality. BD mainly affects young adults with a peculiar geographical distribution. It has been suggested that BD varies in its phenotypic expression in different ethnic groups. Methods: We investigated potential ethnicity-related differences relative to phenotype and prognosis of BD patients in a French multiethnic country. We included 769 consecutive patients fulfilling the international criteria of classification for BD, in the 3 largest ethnic groups of our cohort [European (n = 369), North African (n = 350) and sub Saharan African (n = 50)]. Factors that affect prognosis were assessed by multivariate analysis. Results: 535 (69.6%) patients were male and the median (IQR) age at diagnosis was of 30.9 (24.9-37.2) years. Sub Saharan African BD patients had a higher frequency of CNS involvement (48% vs 32.3% vs 29.5%, p = 0 .035), a higher rate of death (12% vs 6% vs 3.5%, p = 0.029) and a lower frequency of HLA B51 allele (29.4% vs 49.2% vs 55.8%, p = 0.009) compared to those from North Africa and Europe, respectively. Multivariate analysis showed that male gender (HR: 5.01, CI: 1.51-16.65), cardiovascular involvement (HR: 2.24, CI: 1.15-4.36), and sub Saharan African origin (HR 2.62 (0.98-6.97) were independently associated with mortality. The 15-year mortality rate was of 19%, 9% and 6% in sub Saharan African, North African and European BD patients, respectively (p = 0.015). Conclusion: We reported ethnicity-related differences with respect to phenotype of BD. Sub Saharan Africans patients exhibited a worse prognosis. [ABSTRACT FROM AUTHOR]

Objective To describe the interplay between Behçet's disease (BD) and pregnancy. Methods This retrospective study included 76 pregnancies in 46 patients fulfilling the international criteria for BD. The median age of the patients at the time of entry into the study was 28.4 years (interquartile range 22.8-30.9 years). Patients were used as their own historical controls to assess the incidence of BD flares during pregnancy and before or after pregnancy. Factors associated with the occurrence of complications during pregnancy were assessed. Results Among the 76 pregnancies with BD analyzed, 27 (35.5%) were associated with worsening of the symptoms of BD flare; oral and genital ulcerations (78.4% and 67.6%, respectively) as well as ocular complications (32.4%) were the most frequent. The mean ± SD annual rates of BD flares were 0.49 ± 0.72 during pregnancy and 1.46 ± 2.42 during the nonobstetric period ( P = 0.018). The proportion of BD flares tended to be lower in patients treated with colchicine (27.9% versus 45.4% of patients not treated with colchicine; P = 0.11). The overall rate of complications during pregnancy was 15.8%. The complications included miscarriage (5 patients), cesarean delivery (3 patients), medical termination of pregnancy (2 patients), hemolysis, elevated liver enzymes, and low platelets syndrome (1 patient), and immune thrombocytopenia (1 patient). There was a statistically significant association between a history of deep vein thrombosis in BD and the risk of obstetric complications (odds ratio 7.25, 95% confidence interval 1.21-43.46, P = 0.029). Neither gestational age at delivery nor neonatal outcome was influenced by BD. Conclusion The disease course in BD seems to improve during pregnancy, mostly in patients who are treated with colchicine. Pregnancy in patients with BD appears not to be associated with an increased rate of pregnancy-related complications. [ABSTRACT FROM AUTHOR]

Abstract: Behçet''s disease (BD) is a recurrent systemic inflammatory disorder of unknown origin characterized by oral and genital mucous ulcer, uveitis, and skin lesions. Involvement of large vessels, central nervous system (CNS), gastrointestinal tract and thrombotic events are less frequent but can be life threatening. The aim of this review is to provide new insights into the pathogenesis of BD. Over the past year substantial advances have been done in the understanding of the genetic [1,2] and immunology [3] of BD. BD is at the crossroad between autoimmune and autoinflammatory syndromes. In common with autoimmune diseases BD shares class I MHC association. However, in contrast to autoimmune disorders, BD has clinical features that seem to be mostly autoinflammatory. The pathogenesis of BD is still unknown, but major determinants of the genetic and immune system abnormalities have been reported recently. Triggering infectious factors are supposed to participate in the outbreak of BD in genetically predisposed patients. Two recent large genome-wide association study (GWAS) conducted in Turkey and Japan reported association between single nucleotide polymorphism (SNP) of interleukin (IL)-10 and IL-23R/IL-12RB2 genes and BD. New insights into the perturbations of T cell homeostasis of BD recently emerged. We have recently demonstrated the promotion of Th17 responses and the suppression of regulatory T cells (Tregs) that were driven by interleukin (IL)-21 production and that correlates with BD activity. Inflammatory cells within BD inflammatory lesions included mostly neutrophils, Th1 and Th17 cells, and cytotoxic CD8+ and γδ T cells. Altogether, the recent progresses in the knowledge of BD pathogenesis pave the way for innovative therapy. [Copyright &y& Elsevier]

Objective. To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD).Methods. We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD.Results. The median age was 39 (range 27–48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score &geq;3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%) that led to complete clinical and imaging remission in 60.9% of patients. Two (8.7%) of the 23 patients with pseudotumoural NBD died of bedridden state complications.Conclusion. The pseudotumoural form of NBD is a rare and life-threatening condition. [ABSTRACT FROM PUBLISHER]

Purpose: To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a).Design: Retrospective study.Methods: Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppressive agents were included. IFN-alpha2a was administered subcutaneously (three million units thrice a week). Ophthalmologic examination and fluorescein angiography associated with laboratory tests were performed at regular intervals. Main outcome measures were visual acuity and recurrence of uveitis attacks before, during, and after initiation of IFN-alpha2a therapy.Results: Control of inflammation was achieved in 28 of 32 patients (88%). Mean observation period of the 28 responder patients was 70.6 months (range, 30.3 to 129.2 months). Median visual acuity improved from 0.52 to 0.33 (logarithm of the minimum angle of resolution units; P = .005) two years after initiation of IFN-alpha2a therapy. The relapse rate decreased significantly during IFN-alpha2a treatment from 1.68 +/- 1.22 relapses/patient/year to 0.11 +/- 0.20 relapses/patient/year (P < .0001). IFN-alpha2a was discontinued in 19 of 28 patients (68%) after 32 months of treatment (range, 16 to 50 months). Mean follow-up after IFN-alpha2a discontinuation was 43 months (range, 11 to 84 months). After IFN-alpha2a discontinuation, the relapse rate increased from 0.08 +/- 0.21 relapse/person/year to 0.74 +/- 1.40 relapse/person/year (P = 0.04).Conclusions: IFN-alpha2a is efficient and safe for the long-term management of severe uveitis associated with Behçet disease. Meanwhile it seems to be a suspensive therapeutic strategy, even though long-term remission is possible in some patients. [ABSTRACT FROM AUTHOR]

The article discusses a population-based prevalence study of Behçet's disease (BD) conducted in Paris, France with a focus on disease risk among immigrants. The prevalence of BD is higher among immigrants of North African or Asian ancestry compared to the European-origin population. BD risk is not related to age. The findings support the hypothesis that BD is hereditary.

Purpose: To evaluate the efficacy of interferon alfa-2a (IFN alfa) in severe uveitis associated with Behçet's disease, that is refractory to steroids and conventional immunosuppressive agents.Patients and Methods: Patients with Behçet's disease (according to the International Study Group criteria), who relapsed despite steroids and immunosuppressive agents, were included in this retrospective study. Ophthalmological examination, laser flare photometry, and fluorescein angiography associated with laboratory tests were performed at regular intervals. IFN alfa (3 millions units thrice a week) was injected subcutaneously.Results: Eight patients (sex ratio: 1) were included between May 1995 and January 1999. The mean age was 29.1 years (14-54 years) and the disease was present between 11 and 167 months before the administration of IFN alfa. IFN alfa was efficient in all cases with a mean follow-up of 22 months (10-55 months). Steroids were tapered from a mean dosage of 47 mg/d to a mean dosage of 8.5 mg/d. Ocular inflammation was controlled and visual acuity improved in all cases. Treatment was generally well tolerated despite a constant but transient flu-like syndrome. IFN alfa was tapered in three patients and stopped in one case without any relapse after five months.Conclusions: Within the limitations of this retrospective study, low-dose IFN alfa seems to be well tolerated, promising in the management of refractory forms of uveitis due to Behçet's disease, and effective in allowing a reduction of steroid dosage. A prospective controlled study is necessary to confirm these preliminary results. [ABSTRACT FROM AUTHOR]

Reports cases of patients with Behcet's disease associated with endomyocardial fibrosis involving the left ventricle of the heart. Features of Behcet's disease; Causes of endomyocardial fibrosis; Sequelae of vasculitis.

Describes a case of central venous thrombosis during the pregnancy of a woman with Behcet's disease. Signs and symptoms; Treatment with heparin and corticosteroids.

[(18)F]fluorodeoxyglucose-positron emission tomography (FDG-PET) has demonstrated its usefulness in giant cell arteritis, Takayasu's arteritis, and unclassified aortitis. FDG-PET imaging could be more effective than CT-scan or magnetic resonance imaging in detecting the earliest stages of vascular wall inflammation. Data are lacking regarding its accuracy for diagnosis and follow-up of vascular lesions in Behçet's disease. We report the case of a 32-year-old woman with history of Behçet's disease and fever of unknown origin revealing voluminous pulmonary arterial aneurysms. FDG-PET scan revealed a peripheral uptake of pulmonary aneurysms, underlying the inflammatory status of arterial aneurysms wall. Our patient was treated by glucocorticoids and intravenous cyclophosphamide and achieved remission of Behçet's disease. During follow-up, FDG-PET scan enabled to detect an early radiologic therapeutic response after 4 months, while CT angiography remained unchanged. We report the first evidence of usefulness of FDG-PET scan for therapeutic evaluation of arterial aneurysms in Behçet's disease. Further studies are warranted to determine the accuracy of FDG-PET scan in larger cohort of Behçet's disease patients with vascular involvement. [ABSTRACT FROM AUTHOR]