International Study Group For Behçet's Disease Research Articles

The Clinical Feature of Behçet's Disease in Northeastern China

PurposeBehçet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China.Materials and Methods116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient.ResultsThe onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients.ConclusionThe clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.

Prevalence of Behcet’s syndrome in patients with recurrent aphthous ulcerations in Brazil

Behcet's syndrome (BS) is a clinical entity characterized by the appearance of recurrent oral and genital ulcers, in addition to a broad spectrum of clinical manifestations. The aim of the present study is to evaluate the prevalence of BS in patients with recurrent aphthous ulcerations (RAU) and to test the hypothesis that BS could be under diagnosed in Brazil. This is a cross-sectional investigation of the prevalence of BS based on the International Study Group for Behcet's Disease (ISGBD) criteria in patients with RAU attended in a stomatology service. Three hundred and six patients were attended at the ambulatory clinic in the study period, but the standard questionnaire was applied to 50 (16.6%) patients, 29 men and 21 women, identified as having RAU. Only one patient met the ISGBD criteria presenting RAU of the minor and complex type, recurrent genital ulcers, cutaneous, articular, ocular, and vascular manifestations, hypoacusia and fever. Thus, a prevalence of 2% of BS was defined in this subgroup of patients. In the present study, the frequency of 2% of BS in patients with RAU demonstrates the need for further population-based studies to be developed in order to definitively establish the real prevalence of this condition.

Association of Colonoscopic Findings and Clinical Symptoms of Intestinal Involvement in Behcet's Disease

Introduction: The frequency of intestinal involvement in the Behcet's disease is about 3 ∼ 25%. It shows geographic differences and higher incidences are reported in the east Asian countries including Korea. We tried to search reliable gastrointestinal symptoms and colonoscopic findings which can predict the intestinal involvement of Behcet's disease. Methods: We included total 186 patients diagnosed with Behcet's according to International Study Group for Behcet's Disease (ISGBD) from April 2004 to August 2007.

Serum homocystein level and vascular thrombosis in patients with Behçet disease

AbstractObjective: Behçet disease (BD) is a multisystemic inflammatory disorder of unknown aetiology that in some patients will present with thrombosis. However, the mechanism of thrombosis is unknown. In this study, we investigate the correlation between homocystein level in BD patients with vascular thrombosis.Patient and methods: One hundred and twenty‐three patients who fullfilled the criteria of the International Study Group for BD (n = 77, 62.6% females and n = 46, 37.4% males) were included in this cross‐sectional study. Patients were divided into two groups: with thrombosis (n = 23, 18.7%) and without thrombosis (n = 100, 81.3%). All patients underwent colour Doppler sonography for documentation of vascular thrombosis. Serum homocystein was measured by ELISA method, folate and B12 were measured by radioimmunoassay.Results: Patients with vascular involvement, vein and/or arterial thrombosis were enrolled. Mean serum homocystein level was significantly higher in the group with thrombosis (P = 0.012) and in male patients than females (18.10 ± 5.5 vs. 13.21 ± 4.1). In multiple regression analysis, the only factors that persisted for thrombosis were age and sex (P = 0.02, P < 0.0001, respectively). Serum folate and B12 were not correlated with thrombosis in BD.Conclusions: In this study, we found that mean homocystein levels were higher in BD patients with thrombosis and it may have a role in the process of thrombosis in BD.

Behçet's disease

Behçet's disease (BD) is a chronic, relapsing multisystem vasculitis with predominant involvement of the oral and genital mucosa. It has a worldwide distribution, but the prevalence is highest in Central Asia and the Far East (along the ancient 'Silk Route'). Genetic, environmental, immunological and haemostatic factors play a role in the aetiopathogenesis. The International Study Group for BD proposed criteria for the diagnosis of this condition, the essential feature being recurrent oral ulceration. Genital ulcers and skin manifestations are common, while ocular changes are the most important cause of morbidity. Almost any organ in the body can be involved, and systemic involvement may portend a poorer prognosis. There is no pathognomonic test for BD and the diagnosis is made on clinical findings. Treatment of BD would require multidisciplinary cooperation, and early referral to an ophthalmologist is advisable to prevent ocular morbidity. Topical and systemic agents (colchicine, dapsone and thalidomide) are useful in controlling exacerbation of the oral and genital ulcers. Severe disease may require immunosuppressive agents and, more recently, biological agents have been used successfully. It tends to follow an unpredictable course, and the eventual prognosis depends on the systemic involvement.

Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases

Vascular lesions can be the presenting sign of Behçet's disease (BD) preceding classical symptoms. The aim of this study was to evaluate the prevalence and types of vascular involvement in BD. Among 2319 patients diagnosed with BD according to the criteria of the International Study Group for BD, 332 patients (279 male, 53 female) with vascular involvement were included in this study. Prevalence of vascular involvement was 14.3%. Vascular involvement was found to be more common in males (P<0.001, male:female ratio 5.26:1). Mean age at onset of vascular involvement was 30.58+/-7.88. Vascular lesion was the presenting sign of BD in 50 patients (2.1%), whereas in 109 patients (4.7%) it started together with other clinical symptoms at the age of diagnosis. Superficial vein thrombophlebitis (SVT) was the most common vascular symptom (53.3%) followed by deep vein thrombosis (DVT) (29.8%). Arterial lesions were rare (3.6%). Of the patients 13.3% had more than one type of vascular involvement. Lower extremities were the most frequent localization in patients with SVT, whereas the femoral vein was mainly involved in patients with DVT and the pulmonary artery was mainly involved in the patients with arterial lesions. In countries like Turkey where BD is more frequent, young male patients presenting with vascular involvement should be evaluated for BD.

Clinical features of late‐onset Behçet's disease: Report of nine cases

Behçet's disease (BD) is usually diagnosed between the second and fourth decades. Onset after 50 years of age is extremely rare. We aimed to analyze the clinical features of late-onset patients with BD. The study was conducted from 439 patients diagnosed as BD according to criteria of the International Study Group for BD. Clinical features of patients who were asymptomatic or suffered from only recurrent aphthous stomatitis (RAS) until 50 years of age but fulfilled the diagnostic criteria of BD after this age were reviewed. The age-of-onset was more than 50 years in nine patients (1.56%). Two patients developed erythema nodosum, two developed pathergy positivity, one developed papulopustules, pathergy positivity and ocular symptoms, one developed papulopustules and pathergy positivity, one developed ocular symptoms, one developed papulopustules and ocular symptoms, and one developed erythema nodosum and pathergy positivity as well as oral aphthae and genital ulcerations after the age of 50 years. The neurologic system was involved in two patients. Mucocutaneous symptoms of two patients worsened after interruption of therapy. Two patients with neurologic and ocular involvement flared during the follow up. Since the course of the disease is regarded to be relatively mild in mature patients, it is noteworthy that systemic manifestations such as ocular and neurologic involvement and acute flares developed after the age of 50 years in the limited number of patients with late-onset BD in our series.

Is There Any Association between Hepatitis G Virus (HGV), Other Hepatitis Viruses (HBV, HCV) and Behçet's Disease?

Infectious agents, especially viruses, have been implicated in the pathogenesis of Behçet's disease (BD). The aim of this study was to determine whether BD is associated with hepatitis viruses. In this study, the serological markers of hepatitis (HBsAg, anti-HBs, anti-HBc and anti-HCV) and viral nucleic acid (HGV-RNA) were studied in the sera of 35 patients, all of whom fulfilled the diagnostic criteria of the International Study Group for BD, and the results were compared with those of 36 healthy controls. The prevalences of HBsAg, anti-HBs, anti-HBc in BD patients were 2.9%, 45.7%, and 31.4%, respectively, which were not significantly different from those in healthy controls. None of the subjects in either group were found to be positive for anti-HCV. HGV-RNA was detected in two patients with BD and in none of the healthy controls. In conclusion, BD does not seem to be associated with hepatitis viral infections including hepatitis B, C, or G.

Identification of an autosomal recessive mode of inheritance in paediatric Behçet's families by segregation analysis.

We have conducted a segregation analysis in order to characterise the transmission of Behçet Disease (BD), a multifactorial condition with a strong genetic component. Complete information about BD status and pedigree was obtained on 104 probands from our database. We used the criteria of the International Study Group for BD (ISBD) to delineate the clinical status of the sibs: possible BD (patients meeting two criteria), or ascertained BD (patients meeting at least three criteria). A proband was defined as "paediatric" when he/she completed ISBD criteria before/by the age of 16 years. Families were distinguished as paediatric (n = 67) (ascertained through a paediatric proband), and non-paediatric (n = 37) ones. An Expectation Maximization (EM) algorithm was used to estimate the Mendelian segregation ratio P in nuclear families (two parents and their offspring). The maximum likelihood estimate: Pcirc; = 0.248, calculated in the paediatric data set, was consistent with the theoretical value of P = (1/4) for autosomal recessive inheritance, whereas the Pcirc; value was 0.08 when using the non-paediatric data set. Our work provides the first evidence of genetic heterogeneity in BD, and of the existence of a Mendelian entity in the paediatric BD subgroup. Previous studies failed to show any simple mode of inheritance in BD, probably because they were performed on the whole BD population.

Prevalences of hepatitis A, B, C and E viruses in Behçet's disease.

To determine whether Behçet's disease (BD), being a systemic vasculitis of unknown aetiology, is associated with hepatitis viruses (HAV, HBV, HCV and HEV). In addition to 124 patients [male:female (M/F): 73/51], all fulfilling the diagnostic criteria of the International Study Group for BD (1991), 14 patients with systemic necrotizing vasculitis (M/F: 7/7), 47 patients with ankylosing spondylitis (M/F: 36/11) and 51 healthy controls (M/F: 22/29) were also included in this study. Serological markers of four different types of hepatitis (anti-HAV IgM, total anti-HAV, HBsAg, anti-HBs, total anti-HBc, anti-HBc IgM, anti-HCV and anti-HEV) were studied in all cases. There was no difference between the groups with respect to HAV, HCV and HEV serologies. Anti-HBs positivity was observed less frequently in BD compared with healthy controls and systemic vasculitis (P<0.05). Serological evidence of previous HAV, HCV and HEV infections was not significantly different between Behçet's patients and other groups. However, previous HBV infection was found in a significantly lower number of BD patients as compared with healthy controls and systemic vasculitic patients.

Transcranial Doppler detection of microembolic signals in patients with Behçet's disease

We evaluated the prevalence of microembolic signals (MES) in patients with Behçet's disease (BD). We also attempted to determine the frequency of MES in BD patients with or without neurological involvement. This study enrolled 55 patients fulfilling the diagnostic criteria of International Study Group for BD. Bilateral transcranial Doppler ultrasound of the middle cerebral arteries was performed. MES were identified based on the criteria of International Consensus group on Microembolus Detection. Patients with BD were divided into two groups in respect of the presence of neurological involvement (n = 10) or not (n = 45), and counts of MES in the two were compared with each other and with normal subjects. We found MES in 16 patients (29%) with BD. The frequency was higher in patients with neurological involvement than in those without (80% vs. 17%, P< 0.001). In patients with neurological involvement there was a positive correlation in regression analysis between the prevalence of MES and disease duration (P = 0.025). There was a significantly higher prevalence of MES in BD patients than in control subjects. The frequency of MES was higher in patients with neurological involvement than in those without. TCD detection of MES may allow the recognition of subset of patients at high risk for the appearance of neurological involvement.

Clinical features of Behçet's disease in children: An international collaborative study of 86 cases

Objectives: The objective of this study was to characterize the clinical picture of Behçet's disease (BD) in children. Study design: A questionnaire was completed by five BD specialists from Turkey, France, Iran, or Saudi Arabia. We first reviewed 86 cases retrospectively with a specially designed computerized database and then selected 65 who met the criteria of the International Study Group for BD, which include buccal aphthosis plus at least two among recurrent genital aphthosis, eye lesions, skin lesions, and positive pathergy test. The remaining 21 patients, who had features suggestive of BD but did not fulfill the international criteria, were analyzed separately and then compared with the other 65 patients. Results: BD affected boys and girls equally. The clinical picture frequently included mucocutaneous lesions. Uveitis was less frequent than in adults but carried a poor prognosis, especially in male patients ( p < 0.001). The mortality rate (3%) was related to large vessel involvement. Familial cases were particularly frequent (15%). Erythema nodosum and skin hypersensitivity were common in Turkish patients, whereas neuro-BD was more frequent in French and Saudi Arabian patients. Patients who did not fulfill the international criteria had significantly less genital aphthosis ( p < 0.01), less skin lesions or hypersensitivity ( p < 0.01), and less uveitis ( p < 0.01). Conclusion: BD in children is similar to BD in adults. The high frequency of familial cases calls for further investigation of the immunogenetic factors that may favor early expression of the disease. (J Pediatr 1998;132:721-5.)

Diagnostic criteria of Behçet's disease: problems and suggestions.

There are no specific diagnostic laboratory tests for Behçet's disease. Diagnosis relies on proper history-taking and typical clinical manifestations. To provide more objectivity to the diagnosis, several diagnostic criteria have been introduced including one major set of guidelines by the International Study Group for Behçet's Disease (ISGBD) in 1990 which has made a significant contribution, although some disagreements exist in interpretation. Based on this criteria, recurrent oral ulceration is an obligatory manifestation for the diagnosis of Behçet's disease; however, some data indicates it is not a requirement for the diagnosis. In this article the author critically reviews different accepted diagnostic criteria or classifications of Behçet's disease and gives recommendations for a more thorough diagnosis.