Prevalence Of Behcet Research Articles

Pattern and Visual Prognostic Factors of Behcet's Uveitis in Northwest Iran.

PurposeTo investigate the pattern of ocular involvement in Behcet's disease (BD) with predictors of patients' final state of vision.MethodsThis historical cohort encompassed the clinical records of 200 patients diagnosed according to the International Criteria for BD (ICBD), over a period of 17 years between 2004 and 2021.ResultsThe prevalence of Behcet's uveitis (BU) was more common in females and patients in the fourth decade of life. Ninety-five patients (47.5%) had evidence of ocular involvement in the initial ophthalmologic evaluation, and 171 patients (85.5%) manifested evidence of BU during the follow-up visits of which bilateral non-granulomatous panuveitis was the most common anatomical pattern of involvement (32.9%) followed by posterior (27.6%), anterior (26.5%), and intermediate (13.8%) uveitis. The prevalent accompanying signs were oral aphthous (67%), skin lesions (29%), and genital ulcers (19.5%). Cystoid macular edema (CME) was the most frequent ocular complication (62%), followed by cataract (57.5%) and epiretinal membranes (ERM) (36.5%). Univariate analysis showed the following determinants: male gender, younger age at onset, panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis as poorer visual prognostic factors of the disease. Multivariate analysis demonstrated a higher chance of poor visual prognosis of BD in patients with panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis.ConclusionThis cohort study demonstrated an overview on epidemiological patterns of BU along with the visual prognostic factors in Iranian patients.

Behcet's syndrome in nonendemic regions.

Behcet's syndrome is more common in certain geographic regions, however, can be seen outside of these areas and need to be included in the differential diagnosis of many patients, as it has overlapping features with many rheumatologic conditions. Especially in regions with immigrant populations, there seem to be similarities to originating countries in Behcet's prevalence, but the syndrome is not limited to those from certain backgrounds and can be seen in others also. There is emerging evidence that even though the prevalence of Behcet's may be similar to that of endemic areas, in nonendemic regions the condition may be less severe, suggesting potential environment agents in determining the severity of the disease. In addition, women seem to be overrepresented in nonendemic areas and may explain part of the reason for less severe symptoms, as Behcet's tends to be more severe in men. The somewhat different presentation of Behcet's syndrome in nonendemic areas needs to be considered when thinking about Behcet's in the differential diagnosis of patients. Research into potentially less severe form of the disease in nonendemic areas may provide new clues to the pathogenesis of this condition.

Headache in Behcet's disease: types and characteristics.

BackgroundBehcet’s disease involves several systems in the body. Neurological involvement is identified by different symptoms. Headache is one of the common complaints of patients with Behcet’s disease. It might be a part of neurological involvement or may arise independently in the course of disease. Studies with small sample size have resulted in various findings in this field. Since the prevalence of Behcet’s disease is relatively high in Iran, this study was carried out to compare the features of headache between an acceptable number of patients with this rare disease and a control group.MethodsThe current case–control study was performed to compare the features of headache between 312 patients with definite Behcet’s disease who referred to a Behcet’s clinic and healthy individuals. Patients with Behcet’s disease were randomly selected. Controls were matched for age and sex. They were personally examined and interviewed meticulously using a questionnaire that met the standards of the International Headache Society classification for different types of headache.ResultsThe incidence of headache in the case and control groups was 28.3 % (n = 120) and 18.6 % (n = 59), respectively (p < 0.05; OR 2.73). Tension-type headache was observed in 12.2 % (n = 38) of cases which was significantly higher than control group (n = 6.3 %) (p = 0.011; OR 2.05). The most frequent type of headache in the case group was tension-type headache (12.2 %). In the control group, however, migraine without aura was the most common type (9.1 %). A correlation between ophthalmological involvement and headache was observed in 11 patients in the case group. In addition, a significantly higher systolic blood pressure was found in the case group compared to the controls (125.1 vs. 121.7 mmHg; p = 0.007). There was no major correlation between prednisolone consumption in patients with Behcet’s disease and the type and frequency of headache.ConclusionsHeadache, especially tension-type headache, is more common in patients with Behcet’s disease. This might be the result of specific types of uveitis-related and non-structural headaches seen in Behcet’s disease.

Prevalence of Behcet’s disease among adult patients consulting three major clinics in a Druze town in Israel

To evaluate the prevalence of Behçet's disease (BD) in a Druze community in Israel, we conducted a two-stage clinic-based survey in an Israeli Druze town. The first stage aimed to identify patients with recurrent aphthous stomatitis (RAS) in all patients who visited three of the largest clinics in the town during a period of 6 months. The second stage aimed to identify those patients with RAS who fulfilled the diagnostic criteria for BD according to the International Study Group (ISG) criteria. One thousand and eighty-three out of about 4,000 registered subjects were interviewed, 63 of whom had RAS (5.8%). Two patients fulfilled the ISG criteria for BD, resulting in a calculated prevalence in the range of 2:1,083-2:4,000, i.e., 50-185:100,000. Another two patients with oral and genital aphthosis but without eye or skin lesions were diagnosed as suspected BD. The very high prevalence of BD, as found in our study, places the Druze among the populations with the highest prevalence of the disease all over the world, though selection biases could account for overestimation as well as underestimation of the actual BD prevalence. Our findings call for genetic studies to explore whether there is a genetic predisposition to BD in this population.

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.

Behçet's syndrome is not so rare: Why do we need to know?

The study by Mahr and colleagues, which is reported in this issue of Arthritis & Rheumatism (1), gives us important new insight into the prevalence of Behcet’s disease (Behcet’s syndrome [BS], as we prefer to call this condition) that will be useful in resource allocation and aid in the understanding of disease mechanisms, the traditional aims of epidemiology. In addition, their data allow us to consider how we can improve our diagnostic abilities using epidemiology as a guide. Mahr et al (1) report a BS prevalence rate of 7.1 per 100,000 adults in a suburb of Paris, France. As expected, non-European subgroups, including subjects of Asian and North African descent, had substantially higher prevalence rates compared with the native European population. The same group of investigators previously examined the frequencies of other vasculitides in the same geographic area, using almost identical sources of case ascertainment (2). In both instances, the authors used a capture–recapture analysis to check the validity of their sources of case ascertainment and to estimate the missing-case rate. The capture–recapture method, which has thus far been used infrequently in rheumatology, is an epidemiologic tool that allows the use of multiple samples from a single population, providing a shortcut to traditional whole population studies (3). The following 3 important conditions must be met for the capture– recapture method to have a workable validity: the population studied must be closed, like a fish pond; the likelihood of representation of the trait being examined should be equal in each sample; and capture by 1 source should not increase or decrease the likelihood of capture by another source. It is often argued that, unlike in veterinary work where the method has found wide use, the capture– recapture method generally underestimates the true disease rate when used in human medicine (4), where the fulfillment of the above 3 conditions is understandably difficult. Nevertheless, the method does give us an approximation of the underestimation. The underestimation was 20% in the study by Mahr et al published in this issue of Arthritis & Rheumatism (1) and 25% in their previous study (2), and the nonresponder rates were also quite similar (43% in the present study and 47% in the previous study). Despite the possible shortcomings of the methodology used, the fact that nearly identical methodology was used in an identical population in both studies gives us the unique opportunity to compare the frequency of BS with the frequencies of the other vasculitides in the heart of Europe. In the area near Paris, BS is several times more common than each of the 4 other vasculitides, while it is almost as common as all of them combined (Table 1). The formal designation of a disease as “orphan” in Europe requires a frequency of 1 per 2,000 inhabitants (5), and it is clear that the frequencies of BS and the other vasculitides are well within the boundaries of this definition. However, a disease can also be an “orphan” in the eyes of the physician, and we have reason to suggest that BS is considerably more orphan in this sense when compared with the other vasculitides. This contention is supported by a comparison of the space allocated to BS in standard textbooks of rheumatology with that allocated to the other vasculitides. One such well-known source (6) has 5 pages allocated to BS compared with 12 for Wegener’s granulomatosis and 7 for Churg-Strauss syndrome. In light of the new information reported by Mahr and colleagues, we hope that BS will become less “orphan” and receive more attention in textbooks, medical education and training, public policy and government, and foundationand pharmaceutical industry–supported research. Both genetic and environmental variables have been described in the etiology of BS. It has been almost Hasan Yazici, MD, Emire Seyahi, MD, Sebahattin Yurdakul, MD: University of Istanbul, Istanbul, Turkey. Address correspondence and reprint requests to Hasan Yazici, MD, Professor of Medicine, Chief, Division of Rheumatology and Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Safa sok. 17/7, Kadikoy, Istanbul 81310, Turkey. E-mail: hyazici@attglobal.net. Submitted for publication July 18, 2008; accepted in revised form September 17, 2008.

Behçet's syndrome: from aetiology to treatment.

Behcet’s syndrome is an inflammatory disorder of unknown cause‚ pathologically characterised by a vasculitis‚ with a predilection for small venules. A T-helper 1 polarisation of the immune response has been suggested. Hulusi Behcet himself suggested herpes simplex as an aetiological agent and more recently‚ superantigens‚ heat shock proteins and streptococcal antigens have been suggested as putative aetiological factors. Clustering of cases along the ancient Silk Road is often cited to support a genetic basis for Behcet’s syndrome‚ but that is clearly not the whole story. The prevalence of Behcet’s syndrome amongst Turks in Germany is lower than that of Turks in Turkey‚ but is higher than that in the indigenous German population‚ suggesting a strong environmental influence. Similarly‚ the rarity of Behcet’s syndrome in Japanese migrants in Hawaii and California contrasts with the higher incidence in mainland Japan. A family history is present in up to 12% of non-caucasoid cases‚ and a sibling risk ratio of 11.2 is reported . The genetic susceptibility to Behcet’s syndrome has been linked with HLA-B51‚ MIC-A6 and more recently‚ 6p22-23. However‚ HLA-B51 is not the elusive “Behcet’s gene”‚ as there is a marked geographical variation in relative risk‚ up to 75% of patients worldwide are HLA-B51 negative‚ and HLA-B51 transgenic mice do not develop the Behcet’s phenotype. Nevertheless‚ these disease markers are all adjacent to suggesting a key role for this cytokine. This is supported by the efficacy of anti-TNF

Epidemiologic and clinical survey of Behcet's disease in Korea: the first multicenter study.

The prevalence of Behcet's disease is the highest in the East Asian and the Mediterranean countries. Behcet's disease is also distributed in the Asian countries, but the nationwide survey has not been performed in Korea yet. The Korean Study Group for Behcet's Disease, founded in 1999, conducted a multicenter, retrospective survey on epidemiologic and clinical features of the patients with Behcet's disease from 20 hospitals around the nation from 1997 to 1999. Of 3,497 patients, 1,527 were classified into complete or incomplete type of Behcet's disease according to the revised Shimizu's classification. The sex ratio was 1:1.75 with the female predominance. Geographical distribution showed the highest frequency in Seoul (38.5%). Clinically, 98.8% had oral ulcers, 83.2% had genital ulcers, 84.3% had skin lesions and 50.9% had ocular lesions. As for the minor clinical manifestations, articular symptoms were the most frequent. The pathergy test showed positive in 15.4% of patients and revealed a higher positive rate in males (20.2%) than in females (12.7%). In conclusion, we performed the first multicenter study on Behcet's disease in Korea and revealed the female predominance, higher frequency of ocular lesions, and lower positivity of pathergy test in the patients.

Cerebral Venous Sinus Thrombosis- An Update

DOI: http://dx.doi.org/10.3329/jom.v12i2.8421 JOM 2011; 12(2): 149-152