Prevalence Of Behçet's Disease Research Articles

Gastrointestinal Behçet's disease: a review.

Behçet's disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet's disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.

The prevalence of Behçet's disease in a city in Central Anatolia in Turkey.

The prevalence of Behçet's disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. The present study aimed to investigate the prevalence of BD in Kayseri, a city in Central Anatolia in Turkey. This study investigated cross-sectional prevalences of BD in individuals aged >10years in Kayseri, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) through home visits, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The study was conducted using the criteria defined by the International Study Group for Behçet's Disease. The sample size was determined to be 4697 with an expected sampling error of 5.5 per 10,000, with a 95% confidence interval. A standard questionnaire was administered to a total of 5218 individuals. A history of ROU was recorded in 470 (9.0%) of the 5218 residents, and a previous diagnosis of BD was recorded in nine individuals. The prevalence rate of BD was estimated as 17 cases per 10,000 population in Kayseri, Turkey. The present study contributes towards estimations of prevalences of BD in Turkey and towards raising public awareness about the disease. It also supports previous studies that have reported the world's highest prevalences of BD in Turkey.

Épidémiologie de la maladie de Behçet

With more than 30 published prevalence estimates for Behçet's disease (BD), covering many different regions worldwide, the prevalence of BD is quite well described. Even though the interpretation of these data is complicated by between-study differences in methodology, which may substantially influence the results, these data suggest large geographic variations in frequency of BD, with prevalence rates of 20–420/100,000 inhabitants for Turkey, 2.1–19.5 for other Asian countries, 1.5–15.9 for southern Europe and 0.3–4.9 for northern Europe. Additional epidemiological studies or case series from North and South America, the Caribbean Islands, and individuals of sub-Saharan ancestry further suggest that the geographic distribution of BD is much wider than the boundaries of the ancient Silk Road. The few available incidence rates prevent from making strong inferences as to whether the frequency of BD has changed over time. Recent population-based studies of immigrants or migrant populations consistently indicate that migrants from areas of high BD prevalence remain at high risk for BD, which may even be close to the prevalence observed in their countries of origin. Genetic factors, which are not detailed in this review, seem to play a preponderant role in BD development, although they cannot explain the wide between-country disparities in BD prevalence. However, environmental risk factors, including infectious and non-infectious causes, remain poorly investigated and have not yet produced solid hints.

Outcome predictors for intestinal Behçet's disease.

Behçet's disease (BD) is a multisystem inflammatory disorder that presents as recurrent oral and genital ulcers in conjunction with other dermatological and ocular manifestations. The prevalence of BD is higher in Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. Similar to inflammatory bowel disease, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission that necessitate adequate maintenance therapy after achievement of clinical remission. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease. To date, 5-aminosalicylic acid, systemic corticosteroids, and immunosuppressants have been used anecdotally to treat intestinal BD. The clinical course of intestinal BD shows considerable variability, and the exact point at which more potent agents such as immunosuppressants should be used has not yet been elucidated. Given the difficulty in predicting which patients will experience complicated disease courses and the fact that these drugs are related with certain risk resulting from immunosuppression, proper identification of prognostic factors in intestinal BD may allow physicians to implement tailored medical therapy and individualized patient monitoring based on risk stratification. In this review, the impact of baseline characteristics on the long-term course of intestinal BD, prognostic factors during various medical therapies, and outcome predictors related to surgery will be discussed.

Intestinal Behcetʼs or Crohnʼs Disease

Purpose: Intestinal Behçet's disease (BD) and Crohn's disease (CD) are chronic inflammatory bowel diseases that are difficult to distinguish from each other. We present a case of intestinal BD which was very difficult to differentiate from CD. Case: 29-year-old male with history of occasional oral ulcers presented with diarrhea, abdominal pain and fevers after eating raw oysters. His partner developed the same symptoms which resolved after taking metronidazole (MTZ) and ciprofloxacin (Cip). Patients symptoms improved after taking MTZ and Cip, but returned 1 week later with diarrhea, painful scrotal ulcers (Figure 1), right wrist pain, sore throat and oral thrush. He also had ulcers on the uvula, cervical and inguinal lymphadenopathy, and multiple small pustular lesions over the body. His albumin was 1.8 mg/dl, hemoglobin 9.7 gm/dl and stool positive for Clostridium difficle toxin. A MR enterography showed diffuse thickening of descending colon and loss of haustra. Flexible Sigmoidoscopy with biopsy showed severe active colitis with cryptitis and ulcerations in the colon and mild patchy proctitis. Patient was started on IV MTZ, oral vancomycin and IV methylprednisolone 60 mg BID. There was immediate improvement in his symptoms and he was discharged from the hospital 4 days later. Discussion: The prevalence of intestinal BD is 1-2% in BD and can have similar presentation as Crohn's disease. The ulceration can be found throughout the colon, but mostly frequently found in the ileocecal region. Our patient met the criteria by international study group for BD with having recurrent oral ulcerations, genital ulcer and involvement of the colon. HLA-B51 allele positive patients have higher prevalence in Japan and eastern Asia and more severe disease. The cummulateive use of steroids and higher number of ulcers are associated in patients with Anti Alpha Enolase Antibody. Endoscopically a round, less than 6 ulcers and absence of cobblestoning is helpful to differentiate intestinal BD from CD. Corticosteriods are the drug of choice for active disease. Azathioprine, colchicine, infliximab, dapsone and tacrolimus have been used for maintenance of intestinal BD. This case highlights the difficulty in differentiating intestinal BD from CD.Figure: [1254] Scortal ulcer.

Incidence, prevalence and clinical characteristics of Behcet's disease in southern Sweden

To study the incidence, prevalence and clinical characteristics of Behçet's disease (BD) in a defined population in southern Sweden. The study area consists of three health-care districts with an adult population (≥15 years) of 809 317 on 1 January 2011 (25% of non-Swedish ancestry), situated in Skåne, the southernmost county in Sweden. Patients were identified using clinical registries in all the five hospitals within the study area. Only patients fulfilling the International Study Group criteria for diagnosis of BD were included. Forty patients (13 women) fulfilling the diagnosis criteria for BD (70% of non-Swedish ancestry) were identified. The point prevalence of BD on 1 January 2011 was 4.9/100,000 adults (95% CI 3.4, 6.5) and was higher among the population of non-Swedish ancestry (13.6 vs 2.0/100,000, P < 0.001), and higher among men (6.8 vs 3.2/100,000, P = 0.019). There were 20 incident cases (diagnosed in Sweden between 1997 and 2010). The annual incidence rate was 0.2/100,000 adults (95% CI 0.1, 0.3) and was higher among the population of non-Swedish ancestry (0.6 vs 0.1/100,000, P < 0.001). The incidence was 0.3/100,000 adults in men and 0.1/100,000 in women, P = 0.143. During the course of the disease, 100% of the patients developed oral ulceration, 80% genital ulcers, 88% skin lesions, 53% eye disease, 40% arthritis/arthralgia and 20% venous thrombosis. The prevalence of BD is higher in Sweden than previously reported, mainly due to immigration. The incidence of BD remains elevated for immigrants from high-prevalence regions even long after settling in Sweden.

CEREBRAL VENOUS THROMBOSIS IN THE MEDITERRANEAN AREA IN ADULTS

Cerebral venous and dural sinus thrombosis (CVT) is a rare condition with a wide spectrum of clinical presentations. The epidemiology of the disease has evolved considerably during the recent decades with increasing oral contraceptive use in young and middle-aged women. CVT has various causes including genetic and acquired prothrombotic disorders and it usually has a favorable outcome with a low rate of thrombotic recurrence and mortality. Geographical and ethnic variations between populations may result in different distribution of CVT etiologies leading to different pathophysiological mechanisms and clinical presentations. In CVT series reported mostly from the Americas and the western European countries Behçet’s disease (BD) is not reported as a common cause of CVT. However it can be discerned as a frequent cause of CVT in BD series. Due to the high prevalence of BD in the southeast Mediterranean region BD is a frequent cause of CVT in the area. Discerning characteristics of patients with BD and CVT have been reported previously and these might be helpful in guiding diagnosis and treatment of CVT especially in this part of the world.

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.

Patient Characteristics in Behçet Disease

Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001-4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence of BD to keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.

Epidemiology and clinical characteristics of behçet's disease in the US: A population‐based study

Behçet's disease (BD) is a rare, multisystem inflammatory disorder of unknown cause. Although well-documented in Eastern populations, epidemiologic data is scarce in North American countries. Here we describe the incidence and prevalence of BD in Olmsted County, Minnesota over 45 years. We identified an incidence cohort of subjects age >/=18 years who had a clinical diagnosis of and/or fulfilled the International Study Group (ISG) criteria for BD from January 1, 1960 to January 1, 2005. Age- and sex-specific incidence and prevalence were estimated and age- and sex-adjusted to the 2000 US total population. The study population was comprised of 13 subjects with BD; 11 fulfilled ISG criteria between 1960 and 2005. Mean +/- SD age was 31 +/- 9 years, and 69% were white. Point prevalence in 2000 was 5.2 per 100,000 population (95% confidence interval [95% CI] 0.64-9.84). The overall annual age- and sex-adjusted incidence of BD was 0.38 per 100,000 population (95% CI 0.17-0.59), with a higher incidence in women (0.51 per 100,000; 95% CI 0.17-0.84) than in men (0.26 per 100,000; 95% CI 0.004-0.52). Dermatologic lesions included oral ulcers (100%), genital ulcers (62%), erythema nodosum (46%), and papulopustular lesions (54%). Ocular lesions, vascular complications, and central nervous system involvement were present in 8, 3, and 3 subjects, respectively. Our study shows an overall incidence of 0.38 per 100,000 population, which is comparable with other Western populations. The prevalence of 5.2 per 100,000 population is similar to estimates reported in Western countries, but lower than that in countries along the Silk Road.

Population‐based prevalence study of Behçet's disease: Differences by ethnic origin and low variation by age at immigration

To estimate the prevalence of Behçet's disease (BD) in a multiethnic population living in France, with particular focus on disease risk among immigrants. The study was conducted in a county in the Paris metropolitan area that is home to 1,094,412 adults (ages > or =15 years), of whom 26% are of non-European ancestry. Patients with BD living in this area during 2003 were identified using 3 sources (hospitals, community physicians, and the National Health Insurance database), and diagnoses were verified using the International Study Group criteria. Standardized, year-2003 prevalence rates were computed for the overall population and for each ethnic group. Stratified prevalence rates according to age at immigration to France were calculated to investigate the relationship between age at immigration and BD risk. Seventy-nine subjects fulfilled our search criteria. The overall prevalence per 100,000 adults was 7.1 (95% confidence interval [95% CI] 3.5-14.4), and the prevalence for populations of European, North African, and Asian ancestry was 2.4 (95% CI 0.6-7.2), 34.6 (95% CI 24.4-47.5), and 17.5 (95% CI 10.7-27.2), respectively. Within the migrant population of either North African or Asian ancestry, BD prevalences were similar for residents born in France, residents <15 years old at immigration, and residents > or =15 years old at immigration. Our findings indicate that the prevalence of BD among immigrants of North African or Asian ancestry is significantly higher than that in the European-origin population, and comparable with rates reported from North Africa and Asia. Moreover, our results suggest that BD risk is not related to age at immigration. These findings support the hypothesis that BD has a primarily hereditary basis.

Prevalence of Behcet's disease in Iran: a WHO‐ILAR COPCORD stage I study

AbstractAim: To elucidate the prevalence of Behcet's disease (BD) during the World Health Organization‐International League of Associations for Rheumatology Community Oriented Program for Control of Rheumatic Disease study in Iran. The old estimate was 16 per 100,000 inhabitants, but this was just an estimation and a more precise figure was required.Materials and methods: Tehran, the capital of Iran with 1/10th of Iran's population, was selected as the field for the COPCORD study. The population of Iran is of mixed ethnicity (Caucasians 75.4%, Turks 22%, and Semites 2.6%). The same distribution was found in Tehran's general population. Tehran has 22 districts; 50 clusters were randomly selected from them. Interviewers passed an exam by interviewing 20 subjects. The observed agreement was 0.96. The chance expected agreement was 0.531325. The kappa coefficient was 0.919 (standard error: 0.112). The z‐score was 8.19. The one tailed P‐value was &lt; 0.0001. Rheumatologists were selected from the ‘Rheumatology Subspecialty’, that is, those who were scheduled to become a rheumatology professor in one of Iran's medical schools.Results: The response rate was 75%. From 10,291 subjects interviewed all subjects with positive questionnaires were examined; seven had definitive BD and five probable BD. The prevalence was calculated on the seven definitive cases. It was 68 for 100,000 inhabitants. The confidence interval was 33.5–137. Four patients were female and three were male. All had oral and genital aphthosis, four had ocular lesions, three had pseudo‐folliculitis, and one had joint manifestations.Conclusions: The prevalence of BD in Iran is 68 per 100,000 inhabitants, which is the second highest prevalence after Turkey (80–370) in the world, and far behind comes Saudi Arabia with 20, China 14, and Japan 13.4 per 100,000 inhabitants.

The migration pattern, patient selection with diagnostic methodological flaw and confusing naming dilemma in Behçet disease

Dear Editor, I have read with great interest the very recent review article of Leibowitz et al. ( European Journal of Echocardiography 2006) titled “Echocardiographic manifestations of Adamantiades–Behcet's disease” and congratulate the authors for their work. They specifically evaluated cardiovascular involvement in Behcet disease that is characterized by myocardial disease, venous disease and disease of the aorta and great vessels. The authors reviewed the spectrum of echocardiographic imaging findings in Behcet disease patients and then suggested the use of echocardiography in such patients to comprehensively examine the great vessels pathology as well as myocardial and valvular performance. However, I would like to make some contributions to this review article and have a few vital questions to be answered by the authors of the present study. First ; The authors state both in the abstract and the Introduction section of the article that “the prevalence of Behcet disease is increasing in Western countries due to migration patterns”. I think this statement should be proved and, therefore, be referenced with evidence based articles. Although this ubiquitous disorder exhibits a distinct geographic variation and is endemically higher particularly in Turkey, Asians and Japan,1,2 such a migration pattern stated by the authors has never been demonstrated for this disorder up to now. Indeed, Behcet disease accounts for up to one-fifth of cases of endogenous uveitis in some of these abovementioned countries that is most probably due to the association of environmental factors together with the histocompatibility antigen. Important in this respect is that Turkish individuals who have migrated to Germany demonstrate a lower risk of disease than those of the Turkish-origin population living in Turkey.2 Furthermore, the disease is virtually unknown in Japanese people who have emigrated to tropical Hawaii or the continental USA despite it having high prevalence in Japan.2 Therefore, I …

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

To investigate the epidemiology and clinical course of Behçet's disease (BD) over a 17-year period in a defined area of northern Italy. All patients with incident BD diagnosed over a 17-year period (from January 1, 1988 to December 31, 2004) living in the Reggio Emilia area were identified through the following sources: physicians at Reggio Emilia Hospital, medical practitioners, and community-based specialists. We identified all patients registered in a centralized index and in the Reggio Emilia district database for rare diseases. Patients were followed up from the time of diagnosis until either their death or April 1, 2005. Eighteen patients (9 men and 9 women) had complete BD. Mean +/- SD age at diagnosis was 33 +/- 7 years. The incidence rate of BD was 0.24 per 100,000. The prevalence of BD on January 1, 2005 was 3.8 per 100,000. No patients died during the followup period. Although all patients developed oral ulceration during the disease course, 22.2% had no oral lesions at disease onset. Eye disease occurred in 55.6%. Ocular disease was more common in men and appeared at disease onset or within the first few years of disease onset (median 3 years). Only 1 patient had loss of useful vision in at least 1 eye at the end of followup. In all affected patients, visual acuity improved once treatment was started. This population-based study is the first to report the prevalence and incidence of BD in Italy. In Italian patients, BD is nonfatal and the prognosis of eye disease is good.

Prevalence and clinical aspects of Behcet’s disease in the north of Israel

Behcet's disease (BD) has a higher prevalence in countries along the ancient silk route, but the actual prevalence in Israel is unknown. We evaluated the occurrence and clinical expression of BD in the northern region of Israel: in the whole population and by ethnic groups. The sample included all adult patients with BD (International Study Group criteria) treated at three medical centers in northern Israel. Patient data were collected by file review and physician survey. Relevant demographic data for the population served by the medical centers were obtained from the official Israeli authorities. A total of 112 patients were identified. The overall prevalence of BD was 15.2/100,000 and was similar in men and women. The prevalence rates among the Jewish, Arab, and Druze populations were 8.6, 26.2, and 146.4 per 100,000, respectively. Age at disease onset was similar in all ethnic groups and significantly lower in males (28.6+/-9.7 vs 32.9+/-11.3, p=0.03). There were no differences in disease manifestations by sex or ethnicity. All Druze patients were HLA-B5 positive, compared to 80.8% of the Arab patients and 72.0% of the Jewish patients. Recurrent oral ulcers in family members were more common in Arab patients (p=0.004). The BD severity index was significantly lower in Druze patients (p=0.05), mainly in males (p=0.03). This study confirms the high prevalence of BD in Israel and the variability in disease rates and expression by ethnic origin. Our findings, particularly regarding the Druze population, call for further field surveys and genetic studies.

MEFV gene is a probable susceptibility gene for Behçet's disease

Objective: Behçet's disease (BD) is a rare, chronic, multisystem inflammatory disorder. The prevalence of BD is higher in the Middle Eastern and Mediterranean populations. Another chronic inflammatory disease, familial Mediterranean fever (FMF), is also known to be highly prevalent in these populations. The prevalence of BD is higher in the FMF patient population than in populations known to be rich in BD . Both BD and FMF have some pathophysiological features in common and they result from inappropriate activation of neutrophils. Clinical manifestations of both diseases can mimic each other and the coexistence of both diseases in the same patient has been reported. Given that BD and FMF have similar pathophysiological, epidemiological, and clinical features, we hypothesized that the gene responsible for FMF, MEFV, may also play a role in the pathogenesis of BD.Methods: Forty‐two BD patients who had no symptoms and family history for FMF and 66 healthy controls were screened for common MEFV gene mutations (E148Q, M680I, M694V, and V726A).Results: Fifteen patients (36%) displayed MEFV mutations (nine M694V, five E148Q, and one M680I) and mutation rates were significantly elevated compared to 66 (11%) healthy controls (p = 0.0034).Conclusion: The occurrence of frequent MEFV mutations in BD patients suggests that the MEFV gene is involved in the pathogenesis of Behçet's disease.

Prevalence of Behçet's disease in Istanbul, Turkey.

The prevalence of Behçet's disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Three previous epidemiologic surveys have been carried out in different regions of Turkey. This study investigated the cross-sectional prevalence of BD in individuals aged > 12 years in Istanbul, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) by visiting them in their homes, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The sample size was determined to be 24,000 with an expected BD prevalence rate of 1/1000 and a sampling error of 4/10,000, with a 95% confidence interval (CI) of 6-14/10,000. The number of individuals to be screened in each district was determined in proportion to the population of all districts in Istanbul. The standard questionnaire was applied to a total of 23,986 individuals at their homes. A history of ROU was recorded in 2289 individuals (9.5%), and a previous diagnosis of BD was recorded in 47. The diagnosis of ROU was confirmed in 700, and the diagnosis of BD was established in 101 according to the International Study Group criteria. The prevalence rate of BD was estimated as 42/10,000 (95% CI, 34-51/10,000) in Istanbul, Turkey. This survey conducted in Istanbul, the largest cosmopolitan city in Turkey with immigrants from all over the country, has a larger sample size than other previous studies, and therefore the reported prevalence rate of BD has a more acceptable confidence interval. This study aids in the estimation of the prevalence of BD in Turkey, and supports previous findings that Turkey has the highest prevalence rate of the disease in the world.

Behçet's disease and renal failure.

The aims of this study were (i) to investigate the prevalence of Behçet's disease (BD) among dialysis patients in Turkey, (ii) to report the clinical characteristics of patients with BD and end-stage renal disease (ESRD), (iii) to evaluate the effect of ESRD on course and activity of BD and (iv) to analyse the published data about BD and renal failure. A questionnaire investigating BD among dialysis patients was submitted to 350 dialysis centres and we obtained the data for 20 596 patients from 331 dialysis centres. We submitted a second questionnaire regarding clinical characteristics of the patients with BD and ESRD. The PubMed and Web of Science databases were used for the analysis of BD and renal failure. Fourteen patients with BD were determined and the prevalence of BD was 0.07% among 20 596 dialysis patients in Turkey. None of the patients has had a new manifestation of BD after initiation of haemodialysis treatment. The analysis of previous data about renal BD demonstrated 67 patients with renal failure. The most common cause of renal failure in BD is amyloidosis. Routine urine analysis and measurement of serum creatinine and blood urea nitrogen levels are needed for early diagnosis. Vascular access-related problems are common and the activity of BD appears to decrease in patients with ESRD after initiation of haemodialysis.

Behçet's disease in Familial Mediterranean Fever:Characterization of the association between the two diseases

Objectives: Familial Mediterranean fever (FMF) is a genetic disease, characterized by attacks of fever and painful manifestations. Several vasculitides are more common in FMF than in the general population. The aim of the study was to define and characterize the association between FMF and Behçet's disease (BD), a form of vasculitis not previously related to FMF. Methods: We conducted a retrospective study in which FMF patients, alsosuffering from BD (FMF-BD), were recruited from about 4,000 patients registered in our clinic, using a computer survey. Patients identified by the screening process were examined, and those meeting the published criteria for the diagnoses of FMF and BD were classified as FMF-BD cases and compared with unselected FMF and BD controls. Results: The prevalence of BD was higher in FMF than in populations knownto be rich in BD (eg, 16 per 4,000 in FMF compared with 1 per 104 in Japan, P < .001). FMF-BD cases and FMF or BD controls were comparable in most demographic, clinical, and laboratory aspects studied. However, more cases than FMF-controls were of Iraqi/Turkish origin and responded less favorably to colchicine. A higher proportion of cases than BD controls had skin, central nervous system, and gastrointestinal manifestations, originated from North Africa, and had family history of BD. In most cases, as in most respective controls, the severity of FMF was of intermediate grade and the extensiveness of BD was limited. The HLA 135 antigen was present in 53% of BD cases and 40% of BD controls. Conclusions: BD should be included among the vasculitides complicating FMF BD and FMF in patients with FMF-BD, and in patients suffering from each of these entities alone, are clinically and demographically comparable.