Materiały źródłowe

• #1 Behçet’s Disease: Epidemiology, Etiopathogenesis, Diagnosis and Treatment | Frontiers Research Topic

  https://www.frontiersin.org/research-topics/13770/behcets-disease-epidemiology-etiopathogenesis-diagnosis-and-treatment/magazine

  Behet’s disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology with the clinical features of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal, urogenital, pulmonary, and cardiac involvement. […] In this collection, we aim to bring the most recent developments in BD to a close. We will review the current state of knowledge regarding the epidemiology, etiopathogenesis, clinical features, diagnosis, differential diagnosis and therapeutic approaches for the treatment of BD. […] We will also discuss the epidemiological similarities and differences in BD among the countries with the most recent developments in the epidemiology.

• #1 Epidemiology and treatment of Behçet’s disease in the USA: insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry with a comparison with other published cohorts from endemic regions | Arthritis Research & Therapy | Full Te

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02615-7

  Behets disease (BD), a chronic systemic vasculitis, has distinct geographical and ethnic variation. Data regarding the epidemiology of patients with BD in the U.S. are limited; therefore, we sought to describe BD patient characteristics and medication use in the U.S., and compared them with data from patients from endemic regions. […] The prevalence of BD in the U.S. is increasing, which may be due to increased disease recognition and immigration from endemic areas; however, robust epidemiologic data about BD in the U.S. is scarce. […] The current study represents the largest dataset of U.S. BD patients reported to date. We confirmed the female predominance in the U.S. and found that a substantial proportion of BD patients were taking a biologic medication. In comparison to patients in endemic regions, U.S. BD patients were older and received more biological medications, which raises the possibility that there are a variety of epidemiologic and clinical differences in disease between patients in the U.S. and those from endemic areas.

• #1 Behcet’s disease: epidemiology, clinical manifestations, and diagnosis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/27351485/

  Behcet’s Disease (BD) is classified among vasculitides. […] Areas covered: a) Epidemiology: BD is rare, and is seen along the Silk Road, from 20 to 420/100,000 in Turkey and 80/100,000 in Iran, to 0.64/100,000 in the UK. […] This data was extracted from the five nationwide surveys and the largest case series from BD conference reports and a Pubmed search.

• #1 Clinical manifestations and diagnosis of Behçet syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-behcet-syndrome

  Behçet syndrome is more common along the ancient silk road, which extends from eastern Asia to the Mediterranean. It is most common in Turkey (80 to 370 cases per 100,000), while the prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia. […] Beyond the silk road, immigrant and refugee populations from areas of high Behçet syndrome prevalence demonstrate increased risk of disease development. Prevalence in Paris, France in 2003 was 7.1 per 100,000, with rates of 2.4 per 100,000 in those of European ancestry, 34.6 per 100,000 in those of North African ancestry, and 17.5 per 100,000 in those of Asian ancestry. […] Behçet syndrome is somewhat more common in males in the eastern Mediterranean area and in females in northern European countries. Estimates of prevalence in the United States and Europe have ranged from 0.12 to 7.5 per 100,000. Analysis of residents of Olmsted County, Minnesota over a 45-year period identified a prevalence of 5.2 per 100,000.

• #1 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease usually affects young adults 20 to 40 years of age and is also seen in children less frequently. Both genders are equally affected by the disease; a male predominance is observed in Arab populations, while female predominance is evident in Korea, China, the United States, and some northern European countries. The disease has a more severe course in males and younger populations. Most cases are sporadic, although familial clusters are also reported. Genetic anticipation, described as the earlier onset of disease in successive generations, has also been described. […] The geographic pattern of the disease suggests a distribution along the ancient „Silk Route” with the highest incidence in the Middle and the Far East. Turkey has the highest prevalence affecting 420 people per 100,000 population. In the United States, the prevalence is 5.2 people per 100,000 population.

• #1 Article: Behçet’s disease, hospital-based prevalence and manifestations in the Rotterdam area (full text) – December 2015 – NJM

  https://www.njmonline.nl/article_ft.php?a=1650&d=1093&i=189

  Behets disease, first described by Hulusi Behet in 1937, is an idiopathic systemic vasculitis with variable clinical manifestations. The highest prevalences of Behets disease are seen in Turkey (20-420 per 100,000, with about 70 per 100,000 in its European region). In Asian countries, such as Japan, Korea, China, Iran, and Saudi Arabia, prevalence varies from 13.5 to 20 cases per 100,000. In Western countries prevalences of approximately 1 per 100,000 have been reported. Morbidity is reported to be lower in Western populations, in comparison with Eastern Mediterranean and Middle and Far Eastern countries. Behets disease can develop at any age, but the disease appears most frequently between the second and fourth decade of life. In countries along the former Silk Road, Behets disease is more frequent among men, whereas in Western countries it is more prevalent in women. Demographic and morbidity data of Behets disease in Northern Europe and the Netherlands are sparse. The prevalence of Behets disease in the Rotterdam area differed per ethnicity: 1 per 100,000 for Dutch, 71 per 100,000 for Turkish and 39 per 100,000 for Moroccans. We present the first epidemiological and morbidity data of Behets patients living in the Netherlands. In this population the majority of patients are Dutch-Caucasian, Turkish or Moroccan. The prevalence of Behets disease in the Rotterdam area amongst these groups reflects epidemiological studies in comparable populations and does not appear to shift after migration. However, a substantial additional amount of patient data is necessary to elucidate migration effects on the occurrence and morbidity of Behets disease more robustly.

• #1 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Behet disease is not common in the United States. The US prevalence is reported as 0.12-0.33 case per 100,000 population. […] However, data from a study of residents of Olmsted County, Minnesota, over a 45-year period identified a prevalence of 5.2 cases per 100,000 population. […] The American Southwest has a prevalence of 8.9-10.6 cases per 100,000 population, with ethnic proportions of 49.2% Hispanic American, 31.7% European-American, 14.3% Native American, and 1.7% derived from Silk Road populations. […] Worldwide, the incidence and prevalence of Behet disease are highest along the Silk Road, the ancient trade passage that extended from China to the Middle East and southern Europe. This geographic pattern correlates with the prevalence of carriage of the HLA-B*51 allele of the major histocompatibility complex, which is associated with susceptibility to Behet disease.

• #1 The Epidemiology of Behçet’s Disease | IntechOpen

  https://www.intechopen.com/chapters/54638

  Behets disease (BD), a chronic vasculitis affecting any type of the blood vessels, was first described by a Turkish dermatologist Hulusi Behet. Although it has a worldwide distribution, it is commonly seen in the Silk Road countries around the Mediterranean Sea. The country in which the disease is most commonly seen is Turkey with a prevalence of 20602/100,000. BD has a worldwide distribution. But it is commonly seen in the Silk Road countries around the Mediterranean Sea, including Spain, Portugal, Turkey, Iran, and Far East countries like China and Japan. The prevalence of the disease along the silk route is 1420/100,000. BD is most common in Turkey with the prevalence of 20602/100,000. The other more prevalent countries are Iran, China, Tunisian, Korea, Israel, and Japan. In North European, American, and African countries BD is less frequent. Regional and ethnic factors may affect the time of the diagnosis of the illness. The diagnosis of the disease is delayed in areas where the prevalence of the disease is lower. BD is uncommon in children. Patients with initial symptoms at age 16 years or lower are classified as juvenile-onset BD. The prevalence of juvenile BD is unknown. However, in few series, it was reported that 3.3 and 26% of the patients with BD were juvenile-onset BD. BD disease affects both the gender equally. But the gender distribution may differ between different regions. A few studies showed that male predominance was seen in Middle Eastern countries, while female predominance was seen in Asian countries. Gender affects the clinical findings and the severity of the disease as well. BD has a worldwide distribution. But it is commonly observed along the Silk Road countries between the Mediterranean Sea. It is most common in Turkey. The prevalence of the disease, the frequency of the clinical findings, and genetics may differ between different regions.

• #1 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  A meta-analysis of 18 HLA-B*51 studies concluded that this association with Behet disease appeared to strengthen geographically eastward along the Silk Road. […] Turkey has the highest prevalence of Behet disease, with 420 cases per 100,000 population. The prevalence in Japan, Korea, China, Iran, and Saudi Arabia ranges from 13.5 to 22 cases per 100,000 population. The prevalence in North America and Europe is much lower, with 1 case per 15,000-500,000 population. […] The sex prevalence varies by country. In the Middle East, Behet disease is more common among males, with male-to-female ratios of 3.8:1 (Israel), 5.3:1 (Egypt), and 3.4:1 (Turkey). In Germany, Japan, and Brazil, the disease is slightly more common in females. In the United States, Behet disease is more common in females (5:1 female-to-male ratio). […] Behet disease is most common in persons aged 20-40 years. The mean age at onset is 25-30 years. Cases that develop before age 25 years are more likely to involve eye disease and active clinical disease.

• #1 POS0258 THE EPIDEMIOLOGY OF BEHÇET’S SYNDROME IN ENGLAND; A RETROSPECTIVE CASE CONTROL STUDY NESTED WITHIN THE CLINICAL PRACTICE RESEARCH DATALINK (CPRD) AND CPRD LINKED HOSPITAL EPISODES STATISTICS (HES) | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/Suppl_1/432

  CPRD and linked HES provide a national resource to further explore the epidemiology of Behcets syndrome (BS) in a western population. […] Annual incidence rate and prevalence per million person years will be calculated stratified by age, sex, ethnicity and patient level/area-based deprivation. […] A total of 4810 cases of BS were noted during the study period. Prevalence of BS was 19.07 per 100,000 (95% CI 19.93, 18.23) in 2020, rising compared to previous years (12.5/100,000 in 2006). […] The prevalence of BS appears higher than previously thought in the UK consistent with our previous observations.

• #1 Epidemiology of Behçet’s Disease in Northern Spain – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-behcets-disease-in-northern-spain/

  Epidemiology of Behets Disease in Northern Spain […] The prevalence of Behets disease (BD) has a considerable geographical and temporal variability. Data regarding epidemiology in Spain are limited. Our study aimed to assess the epidemiology and clinical domains of BD in a population-based cohort from northern Spain and to compare the results with other geographical areas. […] We conducted a cross-sectional study of a well-defined population in Northern Spain. Cases of suspected BD between January 1980 and December 2018 were identified. The diagnosis of BD was established according to the International Study Group (ISG) for Behets Disease. The incidence of BD between 1999 and 2018 was estimated by sex, age, and year of diagnosis. […] Of 120 patients with probable BD, 59 patients met ISG criteria and were finally included in the study, with a male/female ratio of 0.97; mean age 49.714.7 years. Incidence during the period of study was 0.492 per 100,000 people, observing an increase from January 1999 to December 2018. Prevalence was 10.14 per 100,000 in 2018. Clinical manifestations were relapsing aphthous stomatitis (100%), genital ulcers (78%), skin involvement (84.7%), joint involvement (64.4%), uveitis (55.9%), central nervous system (16.9%), vascular (10.2%), and gastrointestinal manifestations (6.8%). […] The prevalence of BD in Cantabria is higher than in other Southern-European countries. This difference may reflect a combination of geographic, genetic, or methodological variations, as well as the free accessibility to the Spanish Public Health System. Clinical phenotypes observed are similar to those described in other world regions.

• #1 Behçet’s disease in Wales: an epidemiological description of national surveillance data | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02505-4

  Behets disease is a rare, chronic, incurable, multisystemic disease. […] The aims were to identify population prevalence of Behets disease in Wales in comparison to other endemic and non-endemic regions, and provide an epidemiological profile of a case series of adult patients. […] Between 1995 and 2020, 347 adults and 5 children were recorded in Wales with a diagnosis of Behets disease. Population prevalence was calculated as 11.1 per 100,000 population. […] The prevalence of Behets disease varies geographically, with the condition being most common along the ancient Silk Road route in the Far East and Mediterranean basin. […] The current population prevalence of Behets disease in Wales is estimated to be 11.1 per 100,000 population. […] This is significantly lower than the countries of the world with the highest estimated prevalence (such as Turkey and Iran, reported as having prevalence of 380 and 68 per 100,000 population respectively).

• #1 Behçet’s disease in Wales: an epidemiological description of national surveillance data | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02505-4

  Our study identified that the majority (76.9%) of Behets disease cases in Wales were female. […] The 2020 UK cohort study also noted higher prevalence in females (63.8%). […] We did not find statistically significant differences in durations of survival between males and females, or cases residing in different WIMD 2019 quintiles. […] The strengths of this study included national population level coverage, data collection in keeping with well-established surveillance registry practices and the triangulation of sources to ensure verification of cases.

• #1 Behcet disease | MedLink Neurology

  https://www.medlink.com/articles/behcet-disease

  There is emerging evidence that Behcet disease in nonendemic regions may be less severe, suggesting potential environment agents in determining the severity of the disease. […] The symptoms of Behcet disease also show geographical and ethnic variation, with severe eye involvement and inflammatory bowel disease being more common in the Far East than in the Mediterranean basin.

• #1 Behçet’s Disease Epidemiology and Geographical Variations | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-031-77251-1_3

  Behets disease (BD) is a chronic, multifactorial, and systemic condition that progresses in the form of inflammatory attacks. Although BD can be seen all over the world, it is more common in the Middle East, the Far East, and countries around the Mediterranean. […] Trkiye has the highest prevalence of BD. […] Current evidence strongly suggests that genetic factors play a crucial role in predisposition to BD. […] While the prevalence of the HLA-B51 antigen is 2025% in the normal population and 5080% in BD patients in countries on the historical Silk Road, these rates are 28% and 15%, respectively, in countries where BD is rare. […] The clinical manifestations of BD can vary depending on the geographical region. While less eye involvement and vascular involvement have been reported in Trkiye and Japan, gastrointestinal involvement is more common in Japan and Korea than in other countries.

• #1 Evaluation of HLA-B51 frequency and its relationship with clinical findings in patients with Behçet’s disease: 4-year analysis in a single center | Egyptian Rheumatology and Rehabilitation | Full Text

  https://erar.springeropen.com/articles/10.1186/s43166-023-00181-1

  Although BD generally affects the Middle East, Mediterranean, and East Asian societies on the ancient Silk Road, it can be seen all over the world due to migration. […] Turkey has the highest prevalence of BD. […] The present study aimed to investigate the association of HLA-B51 positivity in Turkish patients with BD and the relationship with the demographic and clinical findings. […] The frequency of HLA-B51 was 55.6%. […] Oral and genital ulceration, vascular involvement, and family history were more common in patients with BD with HLA-B51 positivity. […] The frequency of vascular involvement was higher in patients who were HLA-B51-positive. […] However, there were no statistically significant differences between the HLA-B51-positive and HLA-B51-negative groups. […] The prevalence of HLA-B51 may differ according to the populations investigated. […] The frequency of HLA-B51 was 54-82% in Turkish patients with BD, 44.5% in Japan, and 48.9% in Iran, and it has been reported as 15% in Northern Europe and America. […] Therefore, the usefulness of HLA-B51 positivity as a diagnostic and prognostic marker in BD remains in doubt.

• #1 Adamantiades-Behçet’s Disease: Epidemiology in Germany and in Europe | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-642-56455-0_23

  The German Registry of Adamantiades-Behets disease founded 1990 in Berlin provides current data on the epidemiology, the clinical manifestations and the course of the disease in Germany on a continuous basis. […] The prevalence of the disease evaluated in Berlin-West was 1.68/100,000 in 1989 and had risen to 2.26/100,000 in 1994. […] Familial occurrence was detected in 3.2% of German and 15.5% of Turkish patients (p=0.016). […] The relative risk of HLA-B51-positive German natives to develop the disease was 6.0 for males (p0.001), while females showed no association of HLA-B51 with the disease. […] HLA-B51 was confirmed as marker of severe prognosis. […] Epidemiological features of Adamantiades-Behets disease in Germany and in Europe.

• #1 Behçet’s Disease (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/behcets-disease-pro

  Behet’s disease epidemiology […] Behet’s disease is not commonly seen in Northern Europeans. The prevalence is highest in the Middle East, Mediterranean and Eastern Asia. […] The prevalence of Behet’s disease is 80 to 370 cases per 100,000 population in Turkey, 10 per 100,000 in Japan and 0.6 per 100,000 in Yorkshire. European cases are more often described – but not exclusively – in the migrant population. […] HLA-B51 is the most strongly associated known genetic factor. However, it accounts for less than 20% of the genetic risk, even in familial cases (less than 5%), which indicates that other genetic factors remain to be discovered. It is most common among patients aged 20-30 years. […] An age of onset younger than 25 years is associated with a higher prevalence of eye disease and active clinical disease. […] Males and females are equally affected. However, males tend to have a more active and more severe form of the disease.

• #1 The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet’s Disease in Brazil

  https://www.mdpi.com/2077-0383/12/22/7008

  The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet’s Disease in Brazil […] Objective: Modifications in the severity and clinical expression of Behçet’s disease (BD) have been described in some areas that are considered endemic for the disease. This study aims to evaluate the chronological changes in epidemiology and clinical characteristics of BD patients in a referral center in Brazil, which is considered a non-endemic area for the disease. […] BD is reported worldwide, but has a well-known distinct geographical distribution, with higher prevalence in countries along the ancient Silk Road. Formal incidence studies are rare for BD. On the other hand, the prevalence of BD shows considerable variability between countries. Iran and Turkey are the countries with the highest prevalence of BD in the world, and the frequency of BD increases from north to south in Europe. BD is relatively rare in South America. In Brazil, there are still no data available on the incidence of BD. A multicentric epidemiological study in Brazil, including patients with vasculitis under follow-up from nine hospital centers, showed that BD is the most frequent primary vasculitis in the country. The study involved seven centers from the southeast and two from the northeast of Brazil and was performed between 2015 and 2017. […] In recent decades, epidemiological data showed that the features of BD have changed over time. A decrease in the prevalence, severity, and clinical expressions of BD was described, mainly in endemic areas. A recent study carried out in Tunisia showed a decrease in the frequency of posterior uveitis between the two decades from 1995 to 2017. […] This study aimed to assess the evolution of epidemiological features and the frequency of clinical manifestations between BD patients divided into two groups according to the follow-up time. […] Our study supports the notion that BD is becoming milder in some regions. BD is a severe blinding disorder, and we found a lower frequency of ocular involvement over time. These findings may be attributed to a higher level of education of patients and a growing awareness of the disease. Newer immunomodulating and biologic agents may offer an improved prognosis in patients with BD with severe manifestations.

• #1 Clinical manifestations and diagnosis of Behçet syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-behcet-syndrome

  Behçet syndrome typically affects young adults 20 to 40 years of age but is infrequently also seen in children. The disease appears to be more severe in young, male patients from Middle- or Far-Eastern Asia. Most cases of Behçet syndrome are sporadic, although families with multiple affected members, which is known as familial clustering, have been reported, and having a first-degree relative with Behçet syndrome does increase risk for the disease. Earlier onset of disease in successive generations, known as genetic anticipation, has been described.

• #1 SciELO Brasil – Behçet’s disease: review with emphasis on dermatological aspects* Behçet’s disease: review with emphasis on dermatological aspects*

  https://www.scielo.br/j/abd/a/rHXgQGQ5FPgnn466Jnt9Kdr/?lang=en

  A recent Dutch study in patients with different ethnic origins living in the Rotterdam area showed a prevalence of 1/100,000 population among Dutch Caucasians, 71/100,000 among Turks, and 39/100,000 among Moroccans, suggesting that the prevalence among Turks and Moroccans is the same as in their countries of origin. […] It is believed that BD affects more males than females, in a proportion that varies from 1.5-5:1, and is in general more serious in male patients. […] In general, BD has a peak age of onset between 20-30 years, and patients who develop the disease from a very young age present with more severe forms, with more organs affected. […] The pediatric population is also affected, and a study performed in the UK in 2016 showed that the frequency of having a first-degree relative also affected was 17%. […] Positive skin pathergy test (SPT) results vary among geographic areas, with high sensitivity and specificity in patients from Turkey, some Mediterranean and Middle East countries, and Japan. However, current studies show a decline in this trend.

• #1 Behçet’s Disease and Neuro-Behçet’s Syndrome – EMJ

  https://www.emjreviews.com/neurology/article/behcets-disease-and-neuro-behcets-syndrome/

  Behets disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin, which usually presents with orogenital ulceration and uveitis and is identified as the triple-symptom complex. […] The epidemiology of BD varies geographically, with a higher prevalence along the ancient Silk Road countries, extending from the Mediterranean region to Japan. This is coupled by a similar variation in the human leukocyte antigen (HLA)-B51, which is strongly associated with the disease in high-prevalence areas. […] The usual onset of BD is in the third or fourth decade of life; however, although rare, onset in children has also been reported. […] While BD shows equal frequency between each sex, males have a more severe disease course. […] Neurologic manifestations have been reported in 449% of cases; however, when large series are studied, their prevalence remains between 3% and 9% of all patients with BD.

• #1 Behçet’s Disease and Neuro-Behçet’s Syndrome – EMJ

  https://www.emjreviews.com/neurology/article/behcets-disease-and-neuro-behcets-syndrome/

  The age of onset of NBS, when excluding paediatric cases, is usually late within the third decade of life, with the mean duration between the onset of BD and NBS being approximately 5 years. […] The second most common form of neurologic involvement is cerebral venous sinus thrombosis (CVST), which may be seen in up to 1220% of the patients with BD who have primary neurological involvement. […] The presence of brainstem atrophy, particularly in the midbrain tegmentum and pons, have also been reported and correlated with a progressive form of the disease. […] Brainstem or spinal cord involvement, frequent relapses, early disease progression, and high CSF pleocytosis are poor prognostic features for p-NBS.

• #1 Behçet’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Beh%C3%A7et%27s_disease

  The syndrome is rare in the United States, Africa and South America, but is common in Asia, suggesting a possible cause endemic to those areas. An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 1 case for every 100,000 people. Globally, males are affected more frequently than females. […] In an epidemiologic study, 56 percent of patients with Behet’s disease developed ocular involvement at a mean age of 30. Ocular involvement was the first manifestation of Behet’s disease in 8.6 percent of patients. Ocular Behet’s disease with involvement of the optic nerve is rarely reported. Among patients with ocular Behet’s disease funduscopic findings of optic atrophy, and optic disc paleness have been identified with a frequency of 17.9 percent and 7.4 percent, respectively. Other fundoscopic findings include vascular sheathing (23.7%), retinal hemorrhage (9%), macular edema (11.3%), branch retinal vein occlusion (5.8%), and retinal edema (6.6%). However, optic atrophy was the most significant cause of visual impairment identified in 54 percent of patients with ocular Behet’s disease and permanent visual impairment.

• #1 British Paediatric Surveillance Unit Study

  https://behcetsuk.org/british-paediatric-surveillance-unit-study/

  A study of children and young people with Behçet’s disease in the UK that was funded by Behçet’s UK (then Behçet’s Syndrome Society) and Alder Hey Children’s Charity started in April 2015. […] The report has been published by Oxford University Press: Behçet’s syndrome in children and young people in the United Kingdom & Republic of Ireland: a prospective epidemiological study.

• #1 Epidemiology and Treatment of Behçet’s Disease Insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-and-treatment-of-behcets-disease-insights-from-the-rheumatology-informatics-system-for-effectiveness-rise-registry/

  Epidemiology and Treatment of Behets Disease Insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry […] Behets disease (BD), a rare multisystem vasculitis, is prevalent among Middle Eastern populations but remains rare in North America. Data for patients with BD in the U.S. are based on cohorts from single centers and limited by small sample sizes. Therefore, we sought to characterize BD in the U.S., analyzing patient characteristics and medication use across the RISE registry. […] We report on the largest population-based BD cohort in the U.S. using the RISE registry. Our findings suggest that U.S. patients with BD are predominantly female, which is in contrast to cohorts in the Middle East. Differences in medication use may be due to practice patterns or differences in the manifestations of BD. Further research is needed to explore the reasons for the increased prevalence of BD among women in the U.S. and its possible impact on disease severity and management.

• #1 Epidemiology, morbidity and mortality in Behçet’s disease: a cohort study using The Health Improvement Network (THIN). — The Kennedy Institute of Rheumatology

  https://www.kennedy.ox.ac.uk/publications/1087913

  The epidemiology of Behet’s disease (BD) has not been well characterized in the UK. […] We used a large UK primary care database to investigate the epidemiology of BD. […] The prevalence of BD was 14.61 (95% CI 13.35-15.88) per 100 000 population in 2017. […] There was significantly increased risk of ischaemic heart disease [aHR 3.09 (1.28-7.44)], venous thrombosis [aHR 4.80 (2.42-9.54)] and mortality [aHR 1.40 (1.07-1.84)] in patients with BD compared with corresponding controls. […] Physicians should be aware of the increased risk of developing ischaemic heart disease, stroke/transient ischaemic attack and deep venous thrombosis in patients with BD at an earlier age compared with the general population.

• #1

  https://www.msjonline.org/index.php/ijrms/article/view/13331

  Behcetss disease is a systemic vasculitis involving small to large veins and arteries. It is a sporadic disease, mostly prevalent among the ancestors of the silk route. […] Though overall mortality is around 5%, delay in diagnosis and treatment may lead to significant morbidity. […] Being uncommon in south India it is liable to be wrongly diagnosed and treated. Delay in the diagnosis and treatment may lead to severe complications. […] This article also elaborated on the latest developments in the treatment of Behcets disease.

• #1 Predictive Factors of Severe Behçet’s disease: A Longitudinal, Prospective Cohort Followed Between 1981–2020 | Reumatología Clínica

  https://www.reumatologiaclinica.org/es-predictive-factors-severe-behcet-s-disease-articulo-S1699258X21001212

  The presence of erythema nodosum as a prognostic factor for the severe disease was only described in patients with ocular disease. […] Our study shows that erythema nodosum is independently associated with the most severe manifestations of BD and that this association is not limited to ocular BD cohorts. […] In our study, the increase of the current age is associated with severe BD, with the odds for severe BD increasing 2% per year, without considering interaction with other factors. […] However, when age was analyzed together with other factors, we found no association between severe BD and the patient’s age. […] The authors believe that, in the future, the identification of such biomarkers can help to distinguish these diseases, and possibly allow an earlier diagnosis. […] Male gender, erythema nodosum, and pseudofolliculitis were associated with severe DB.

• #2 Predictive Factors of Severe Behçet’s disease: A Longitudinal, Prospective Cohort Followed Between 1981–2020 | Reumatología Clínica

  https://www.reumatologiaclinica.org/es-predictive-factors-severe-behcet-s-disease-articulo-S1699258X21001212

  Behet’s disease (BD) is a systemic vasculitis of unknown cause. […] Although BD has a worldwide distribution, its prevalence is higher along the ancient silk route extending from the Mediterranean region to the Middle East and Far East countries. […] BD prevalence varies from 20420/100,000 inhabitants in Turkey to 0.270.63/100,000 inhabitants in the United Kingdom. […] The severity of BD manifestations is known to be greater in men due to an increase in morbidity related to ocular, vascular, and neurological manifestations of BD and in younger patients at the onset of disease. […] This study revealed the association of the male gender as well as the presence of erythema nodosum and pseudofolliculitis, as clinical characteristics that are independently associated with manifestations of severe BD.

• #2 The Epidemiology of Behçet’s Disease | IntechOpen

  https://www.intechopen.com/chapters/54638

  Behets disease (BD), a chronic vasculitis affecting any type of the blood vessels, was first described by a Turkish dermatologist Hulusi Behet. Although it has a worldwide distribution, it is commonly seen in the Silk Road countries around the Mediterranean Sea. The country in which the disease is most commonly seen is Turkey with a prevalence of 20602/100,000. BD has a worldwide distribution. But it is commonly seen in the Silk Road countries around the Mediterranean Sea, including Spain, Portugal, Turkey, Iran, and Far East countries like China and Japan. The prevalence of the disease along the silk route is 1420/100,000. BD is most common in Turkey with the prevalence of 20602/100,000. The other more prevalent countries are Iran, China, Tunisian, Korea, Israel, and Japan. In North European, American, and African countries BD is less frequent. Regional and ethnic factors may affect the time of the diagnosis of the illness. The diagnosis of the disease is delayed in areas where the prevalence of the disease is lower. BD is uncommon in children. Patients with initial symptoms at age 16 years or lower are classified as juvenile-onset BD. The prevalence of juvenile BD is unknown. However, in few series, it was reported that 3.3 and 26% of the patients with BD were juvenile-onset BD. BD disease affects both the gender equally. But the gender distribution may differ between different regions. A few studies showed that male predominance was seen in Middle Eastern countries, while female predominance was seen in Asian countries. Gender affects the clinical findings and the severity of the disease as well. BD has a worldwide distribution. But it is commonly observed along the Silk Road countries between the Mediterranean Sea. It is most common in Turkey. The prevalence of the disease, the frequency of the clinical findings, and genetics may differ between different regions.

• #2 Clinical manifestations and diagnosis of Behçet syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-behcet-syndrome

  Behçet syndrome is more common along the ancient silk road, which extends from eastern Asia to the Mediterranean. It is most common in Turkey (80 to 370 cases per 100,000), while the prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia. […] Beyond the silk road, immigrant and refugee populations from areas of high Behçet syndrome prevalence demonstrate increased risk of disease development. Prevalence in Paris, France in 2003 was 7.1 per 100,000, with rates of 2.4 per 100,000 in those of European ancestry, 34.6 per 100,000 in those of North African ancestry, and 17.5 per 100,000 in those of Asian ancestry. […] Behçet syndrome is somewhat more common in males in the eastern Mediterranean area and in females in northern European countries. Estimates of prevalence in the United States and Europe have ranged from 0.12 to 7.5 per 100,000. Analysis of residents of Olmsted County, Minnesota over a 45-year period identified a prevalence of 5.2 per 100,000.

• #2 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  A meta-analysis of 18 HLA-B*51 studies concluded that this association with Behet disease appeared to strengthen geographically eastward along the Silk Road. […] Turkey has the highest prevalence of Behet disease, with 420 cases per 100,000 population. The prevalence in Japan, Korea, China, Iran, and Saudi Arabia ranges from 13.5 to 22 cases per 100,000 population. The prevalence in North America and Europe is much lower, with 1 case per 15,000-500,000 population. […] The sex prevalence varies by country. In the Middle East, Behet disease is more common among males, with male-to-female ratios of 3.8:1 (Israel), 5.3:1 (Egypt), and 3.4:1 (Turkey). In Germany, Japan, and Brazil, the disease is slightly more common in females. In the United States, Behet disease is more common in females (5:1 female-to-male ratio). […] Behet disease is most common in persons aged 20-40 years. The mean age at onset is 25-30 years. Cases that develop before age 25 years are more likely to involve eye disease and active clinical disease.

• #2 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Behet disease is not common in the United States. The US prevalence is reported as 0.12-0.33 case per 100,000 population. […] However, data from a study of residents of Olmsted County, Minnesota, over a 45-year period identified a prevalence of 5.2 cases per 100,000 population. […] The American Southwest has a prevalence of 8.9-10.6 cases per 100,000 population, with ethnic proportions of 49.2% Hispanic American, 31.7% European-American, 14.3% Native American, and 1.7% derived from Silk Road populations. […] Worldwide, the incidence and prevalence of Behet disease are highest along the Silk Road, the ancient trade passage that extended from China to the Middle East and southern Europe. This geographic pattern correlates with the prevalence of carriage of the HLA-B*51 allele of the major histocompatibility complex, which is associated with susceptibility to Behet disease.

• #2 A Short Overview of Behcet’s Disease | IntechOpen

  https://www.intechopen.com/chapters/80897

  Traditionally known as the silk route disease, Behets disease is seen mainly along the historical Silk Route, which joined the East to the West suggesting that an inherited tendency to develop Behets disease was spread by merchants who traveled these trading routes. The highest prevalence was seen in Turkey with 20420 in 100,000 inhabitants while the lowest was in UK with a prevalence of 0.64. However, due to migration of people from various counties and environmental factors the disease is now prevalent worldwide. Predominant age of occurrence is between 3rd and 4th decades. Disease is more predominant in males with more severe disease manifestations in some Mediterranean areas. […] The etiology of Behets disease remains uncertain but various studies suggest an infectious trigger with inflammatory mediators and immune deregulation as the cause in a genetically susceptible host. Studies have shown an association between Behets disease and the allele HLA-B*51 (chromosome 6p21), which is relatively common in many ethnic groups.

• #2 Behçet’s disease epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Beh%C3%A7et%27s_disease_epidemiology_and_demographics

  The prevalence of Behet disease is approximately 0.12 to 7.5 per 100,000 individuals in the United States. […] The prevalence of Behet disease is approximately 80 to 370 per 100,000 individuals in Turkey. […] The prevalence of Behet disease is approximately 13.5 to 20 per 100,000 individuals in Iran, Japan, Korea, China and Saudi Arabia. […] The prevalence of Behet disease is approximately 7.1 per 100,000 individuals in Paris, France. […] The prevalence of Behet disease is approximately 2.4 per 100,000 individuals in European ancestry. […] The prevalence of Behet disease is approximately 34.6 per 100,000 individuals in North African ancestry. […] The prevalence of Behet disease is approximately 17.5 per 100,000 individuals in Asian ancestry. […] Behet disease commonly affects young adults 20 to 40 years of age.

• #2 SciELO Brasil – Behçet’s disease: review with emphasis on dermatological aspects* Behçet’s disease: review with emphasis on dermatological aspects*

  https://www.scielo.br/j/abd/a/rHXgQGQ5FPgnn466Jnt9Kdr/?lang=en

  A recent Dutch study in patients with different ethnic origins living in the Rotterdam area showed a prevalence of 1/100,000 population among Dutch Caucasians, 71/100,000 among Turks, and 39/100,000 among Moroccans, suggesting that the prevalence among Turks and Moroccans is the same as in their countries of origin. […] It is believed that BD affects more males than females, in a proportion that varies from 1.5-5:1, and is in general more serious in male patients. […] In general, BD has a peak age of onset between 20-30 years, and patients who develop the disease from a very young age present with more severe forms, with more organs affected. […] The pediatric population is also affected, and a study performed in the UK in 2016 showed that the frequency of having a first-degree relative also affected was 17%. […] Positive skin pathergy test (SPT) results vary among geographic areas, with high sensitivity and specificity in patients from Turkey, some Mediterranean and Middle East countries, and Japan. However, current studies show a decline in this trend.

• #2 Epidemiological clinical behavior of Behçet’s disease | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-epidemiological-clinical-behavior-behcet39s-disease-S2444440516300206

  Epidemiological clinical behavior of Behet’s disease […] Behet’s disease is defined as a vasculitis affecting all types of vessels, presenting with local and systemic clinical manifestations that may involve concern any organ or organ system. […] A worldwide distribution is described; being reported more frequently in the countries corresponding to the antique Silk Road (Iran, Iraq, Uzbekistan, Turkmenistan, China and Turkey) and less frequently in Northern Europe and the United States. It is estimated that Turkey is the country with the highest incidence, with 80420 cases per 100,000 inhabitants. In countries like Japan, Korea, China, Iran and Saudi Arabia it fluctuates between 13 and 22 cases per 100,000 inhabitants. In the American countries it has been reported as a rare disease. […] It is reported that BD mainly affects young adults between the third and fourth decades of life. In relation to the gender, is described that it varies from country to country; for example, in the Middle East of Asia, including Israel, Egypt and Turkey, is more common in males, with a ratio of 35 men for every woman; in the reports from Germany, Japan, the United States and Brazil is more frequent in women.

• #2 Epidemiology and treatment of Behçet’s disease in the USA: insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry with a comparison with other published cohorts from endemic regions | Arthritis Research & Therapy | Full Te

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02615-7

  Behets disease (BD), a chronic systemic vasculitis, has distinct geographical and ethnic variation. Data regarding the epidemiology of patients with BD in the U.S. are limited; therefore, we sought to describe BD patient characteristics and medication use in the U.S., and compared them with data from patients from endemic regions. […] The prevalence of BD in the U.S. is increasing, which may be due to increased disease recognition and immigration from endemic areas; however, robust epidemiologic data about BD in the U.S. is scarce. […] The current study represents the largest dataset of U.S. BD patients reported to date. We confirmed the female predominance in the U.S. and found that a substantial proportion of BD patients were taking a biologic medication. In comparison to patients in endemic regions, U.S. BD patients were older and received more biological medications, which raises the possibility that there are a variety of epidemiologic and clinical differences in disease between patients in the U.S. and those from endemic areas.

• #2 Behçet’s Disease (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/behcets-disease-pro

  Behet’s disease epidemiology […] Behet’s disease is not commonly seen in Northern Europeans. The prevalence is highest in the Middle East, Mediterranean and Eastern Asia. […] The prevalence of Behet’s disease is 80 to 370 cases per 100,000 population in Turkey, 10 per 100,000 in Japan and 0.6 per 100,000 in Yorkshire. European cases are more often described – but not exclusively – in the migrant population. […] HLA-B51 is the most strongly associated known genetic factor. However, it accounts for less than 20% of the genetic risk, even in familial cases (less than 5%), which indicates that other genetic factors remain to be discovered. It is most common among patients aged 20-30 years. […] An age of onset younger than 25 years is associated with a higher prevalence of eye disease and active clinical disease. […] Males and females are equally affected. However, males tend to have a more active and more severe form of the disease.

• #2 Medical Summary of Behcet’s Disease

  https://www.behcets.com/medical-summary

  Recent data from Olmsted County, Minnesota, reveals a prevalence rate of 6.6 per 100,000 suggesting that the disease may be more common in the United States than previously suspected. […] Prevalence rates in all areas of the world are increasing, likely due to improved disease recognition and reporting. […] The disease occurs primarily in young adults. The mean age of onset is between 25 and 30 years. […] Earlier reports from eastern countries have shown a male predominance in patients with Behcet’s Disease, but recent reports reflect a male:female ratio much closer to one. In western countries, females predominate. […] In high prevalence areas, Behcet’s Disease is associated with the HLA-B*5101 allele of HLA-B51, a split product of HLA-B5. […] Familial Behcet’s Disease is uncommon, and no clear pattern of inheritance can be determined in most cases but familial disease was noted in 15% of affected children.

• #2 Epidemiology and clinical domains of Behçet’s disease in the Cantabria region, Northern Spain.

  https://www.clinexprheumatol.org/abstract.asp?a=18564

  The prevalence of Behets disease (BD) has a considerable geographical and temporal variability. Data regarding epidemiology in Spain are limited. Our study aimed to assess the epidemiology and clinical domains of BD in a population-based cohort from Northern Spain and to compare the results with other geographical areas of other countries. […] The incidence of BD between 1999 and 2018 was estimated by sex, age, and year of diagnosis. […] Incidence during the period of study was 0.492 per 100,000 people, observing an increase from January 1999 to December 2018. Prevalence was 10.14 per 100,000 inhabitants in 2018. […] The prevalence of BD in Cantabria is higher than in other Southern European countries. This difference may reflect a combination of geographic, genetic, or methodological variations, as well as the free accessibility to the Spanish Public Health System for the entire population.

• #2 The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet’s Disease in Brazil

  https://www.mdpi.com/2077-0383/12/22/7008

  The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet’s Disease in Brazil […] Objective: Modifications in the severity and clinical expression of Behçet’s disease (BD) have been described in some areas that are considered endemic for the disease. This study aims to evaluate the chronological changes in epidemiology and clinical characteristics of BD patients in a referral center in Brazil, which is considered a non-endemic area for the disease. […] BD is reported worldwide, but has a well-known distinct geographical distribution, with higher prevalence in countries along the ancient Silk Road. Formal incidence studies are rare for BD. On the other hand, the prevalence of BD shows considerable variability between countries. Iran and Turkey are the countries with the highest prevalence of BD in the world, and the frequency of BD increases from north to south in Europe. BD is relatively rare in South America. In Brazil, there are still no data available on the incidence of BD. A multicentric epidemiological study in Brazil, including patients with vasculitis under follow-up from nine hospital centers, showed that BD is the most frequent primary vasculitis in the country. The study involved seven centers from the southeast and two from the northeast of Brazil and was performed between 2015 and 2017. […] In recent decades, epidemiological data showed that the features of BD have changed over time. A decrease in the prevalence, severity, and clinical expressions of BD was described, mainly in endemic areas. A recent study carried out in Tunisia showed a decrease in the frequency of posterior uveitis between the two decades from 1995 to 2017. […] This study aimed to assess the evolution of epidemiological features and the frequency of clinical manifestations between BD patients divided into two groups according to the follow-up time. […] Our study supports the notion that BD is becoming milder in some regions. BD is a severe blinding disorder, and we found a lower frequency of ocular involvement over time. These findings may be attributed to a higher level of education of patients and a growing awareness of the disease. Newer immunomodulating and biologic agents may offer an improved prognosis in patients with BD with severe manifestations.

• #2 Evaluation of HLA-B51 frequency and its relationship with clinical findings in patients with Behçet’s disease: 4-year analysis in a single center | Egyptian Rheumatology and Rehabilitation | Full Text

  https://erar.springeropen.com/articles/10.1186/s43166-023-00181-1

  Although BD generally affects the Middle East, Mediterranean, and East Asian societies on the ancient Silk Road, it can be seen all over the world due to migration. […] Turkey has the highest prevalence of BD. […] The present study aimed to investigate the association of HLA-B51 positivity in Turkish patients with BD and the relationship with the demographic and clinical findings. […] The frequency of HLA-B51 was 55.6%. […] Oral and genital ulceration, vascular involvement, and family history were more common in patients with BD with HLA-B51 positivity. […] The frequency of vascular involvement was higher in patients who were HLA-B51-positive. […] However, there were no statistically significant differences between the HLA-B51-positive and HLA-B51-negative groups. […] The prevalence of HLA-B51 may differ according to the populations investigated. […] The frequency of HLA-B51 was 54-82% in Turkish patients with BD, 44.5% in Japan, and 48.9% in Iran, and it has been reported as 15% in Northern Europe and America. […] Therefore, the usefulness of HLA-B51 positivity as a diagnostic and prognostic marker in BD remains in doubt.

• #2 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  Behcets disease is most common along the Old Silk Route, which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran. […] The disease is not rare in regions along the Old Silk Route, but the diseases epidemiology is not well understood. […] In Japan, Behcets disease ranks as a leading cause of blindness. […] Despite the predisposition to Behcets conferred by HLAB51, familial cases are not the rule, constituting only about 5% of cases.

• #2 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease usually affects young adults 20 to 40 years of age and is also seen in children less frequently. Both genders are equally affected by the disease; a male predominance is observed in Arab populations, while female predominance is evident in Korea, China, the United States, and some northern European countries. The disease has a more severe course in males and younger populations. Most cases are sporadic, although familial clusters are also reported. Genetic anticipation, described as the earlier onset of disease in successive generations, has also been described. […] The geographic pattern of the disease suggests a distribution along the ancient „Silk Route” with the highest incidence in the Middle and the Far East. Turkey has the highest prevalence affecting 420 people per 100,000 population. In the United States, the prevalence is 5.2 people per 100,000 population.

• #2 Clinical manifestations and diagnosis of Behçet syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-behcet-syndrome

  Behçet syndrome typically affects young adults 20 to 40 years of age but is infrequently also seen in children. The disease appears to be more severe in young, male patients from Middle- or Far-Eastern Asia. Most cases of Behçet syndrome are sporadic, although families with multiple affected members, which is known as familial clustering, have been reported, and having a first-degree relative with Behçet syndrome does increase risk for the disease. Earlier onset of disease in successive generations, known as genetic anticipation, has been described.

• #2 Behcet disease | MedLink Neurology

  https://www.medlink.com/articles/behcet-disease

  There is emerging evidence that Behcet disease in nonendemic regions may be less severe, suggesting potential environment agents in determining the severity of the disease. […] The symptoms of Behcet disease also show geographical and ethnic variation, with severe eye involvement and inflammatory bowel disease being more common in the Far East than in the Mediterranean basin.

• #2 Novel genetic variants of HLA gene associated with Thai Behcet’s disease (BD) patients using next generation sequencing technology | Scientific Reports

  https://www.nature.com/articles/s41598-024-58254-w

  While numerous HLA alleles have been reported as susceptible in various Asian and European countries, limited studies have explored their association with BD in the Southeast Asian (SEA) population. […] High-resolution HLA typing is instrumental in pinpointing specific HLA alleles, offering a more precise understanding of an individual’s HLA profile and its correlation with BD. […] This study in Thailand is among the first to explore the association of both class I and II HLA molecules with BD in high resolution (6-digits) using NGS technology.

• #3 Behçet’s disease in Wales: an epidemiological description of national surveillance data | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02505-4

  Behets disease is a rare, chronic, incurable, multisystemic disease. […] The aims were to identify population prevalence of Behets disease in Wales in comparison to other endemic and non-endemic regions, and provide an epidemiological profile of a case series of adult patients. […] Between 1995 and 2020, 347 adults and 5 children were recorded in Wales with a diagnosis of Behets disease. Population prevalence was calculated as 11.1 per 100,000 population. […] The prevalence of Behets disease varies geographically, with the condition being most common along the ancient Silk Road route in the Far East and Mediterranean basin. […] The current population prevalence of Behets disease in Wales is estimated to be 11.1 per 100,000 population. […] This is significantly lower than the countries of the world with the highest estimated prevalence (such as Turkey and Iran, reported as having prevalence of 380 and 68 per 100,000 population respectively).

• #3 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  BD is famously known to be prevalent along the Silk Road, which extends from eastern Asia to the Mediterranean. It is most commonly noted in Turkey, but has high prevalence also in Japan, Korea, China, Iran, Iraq, and Saudi Arabia. […] In North America and Northern European countries, the prevalence is much lower. Onset is typically in the second to fourth decades of life. BD is most commonly sporadic.

• #3 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Behet disease is not common in the United States. The US prevalence is reported as 0.12-0.33 case per 100,000 population. […] However, data from a study of residents of Olmsted County, Minnesota, over a 45-year period identified a prevalence of 5.2 cases per 100,000 population. […] The American Southwest has a prevalence of 8.9-10.6 cases per 100,000 population, with ethnic proportions of 49.2% Hispanic American, 31.7% European-American, 14.3% Native American, and 1.7% derived from Silk Road populations. […] Worldwide, the incidence and prevalence of Behet disease are highest along the Silk Road, the ancient trade passage that extended from China to the Middle East and southern Europe. This geographic pattern correlates with the prevalence of carriage of the HLA-B*51 allele of the major histocompatibility complex, which is associated with susceptibility to Behet disease.

• #3 Behçet’s Disease (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/behcets-disease-pro

  Behet’s disease epidemiology […] Behet’s disease is not commonly seen in Northern Europeans. The prevalence is highest in the Middle East, Mediterranean and Eastern Asia. […] The prevalence of Behet’s disease is 80 to 370 cases per 100,000 population in Turkey, 10 per 100,000 in Japan and 0.6 per 100,000 in Yorkshire. European cases are more often described – but not exclusively – in the migrant population. […] HLA-B51 is the most strongly associated known genetic factor. However, it accounts for less than 20% of the genetic risk, even in familial cases (less than 5%), which indicates that other genetic factors remain to be discovered. It is most common among patients aged 20-30 years. […] An age of onset younger than 25 years is associated with a higher prevalence of eye disease and active clinical disease. […] Males and females are equally affected. However, males tend to have a more active and more severe form of the disease.

• #3 Behçet’s disease epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Beh%C3%A7et%27s_disease_epidemiology_and_demographics

  The prevalence of Behet disease is approximately 0.12 to 7.5 per 100,000 individuals in the United States. […] The prevalence of Behet disease is approximately 80 to 370 per 100,000 individuals in Turkey. […] The prevalence of Behet disease is approximately 13.5 to 20 per 100,000 individuals in Iran, Japan, Korea, China and Saudi Arabia. […] The prevalence of Behet disease is approximately 7.1 per 100,000 individuals in Paris, France. […] The prevalence of Behet disease is approximately 2.4 per 100,000 individuals in European ancestry. […] The prevalence of Behet disease is approximately 34.6 per 100,000 individuals in North African ancestry. […] The prevalence of Behet disease is approximately 17.5 per 100,000 individuals in Asian ancestry. […] Behet disease commonly affects young adults 20 to 40 years of age.

• #3 POS0258 THE EPIDEMIOLOGY OF BEHÇET’S SYNDROME IN ENGLAND; A RETROSPECTIVE CASE CONTROL STUDY NESTED WITHIN THE CLINICAL PRACTICE RESEARCH DATALINK (CPRD) AND CPRD LINKED HOSPITAL EPISODES STATISTICS (HES) | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/Suppl_1/432

  CPRD and linked HES provide a national resource to further explore the epidemiology of Behcets syndrome (BS) in a western population. […] Annual incidence rate and prevalence per million person years will be calculated stratified by age, sex, ethnicity and patient level/area-based deprivation. […] A total of 4810 cases of BS were noted during the study period. Prevalence of BS was 19.07 per 100,000 (95% CI 19.93, 18.23) in 2020, rising compared to previous years (12.5/100,000 in 2006). […] The prevalence of BS appears higher than previously thought in the UK consistent with our previous observations.

• #3 Behcet disease | MedLink Neurology

  https://www.medlink.com/articles/behcet-disease

  Epidemiology of Behcet disease shows a geographical variation. It is more common in Eurasian countries between the latitudes of 30N and 45N in countries along the previously called Silk Route, which extends from the Mediterranean region to Japan. This distribution is associated with a similar variation in HLA-B51, which correlates with disease high prevalence areas such as the Middle East and Far East. […] The prevalence of Behcet disease has been reported to be between 0.5 to 5/105 in the United States, between 0.5 to 3/105 and 1/105 in northern and central Europe, 2.5 to 7.1/105 in the northwestern Mediterranean region, and increases further in the eastern Mediterranean and Middle Eastern regions. […] In a multihospital-based prevalence study from the Netherlands, patients with Behcet disease from different ethnic backgrounds living in the Rotterdam area were recorded and compared to the number of people with the same ethnic background living in the same area. A prevalence rate for Behcet disease was calculated to be 1/105 among Dutch-Caucasians, 71/105 among Turks, and 39/105 among Moroccans.

• #3 Predictive Factors of Severe Behçet’s disease: A Longitudinal, Prospective Cohort Followed Between 1981–2020 | Reumatología Clínica

  https://www.reumatologiaclinica.org/es-predictive-factors-severe-behcet-s-disease-articulo-S1699258X21001212

  Behet’s disease (BD) is a systemic vasculitis of unknown cause. […] Although BD has a worldwide distribution, its prevalence is higher along the ancient silk route extending from the Mediterranean region to the Middle East and Far East countries. […] BD prevalence varies from 20420/100,000 inhabitants in Turkey to 0.270.63/100,000 inhabitants in the United Kingdom. […] The severity of BD manifestations is known to be greater in men due to an increase in morbidity related to ocular, vascular, and neurological manifestations of BD and in younger patients at the onset of disease. […] This study revealed the association of the male gender as well as the presence of erythema nodosum and pseudofolliculitis, as clinical characteristics that are independently associated with manifestations of severe BD.

• #3 Evaluation of HLA-B51 frequency and its relationship with clinical findings in patients with Behçet’s disease: 4-year analysis in a single center | Egyptian Rheumatology and Rehabilitation | Full Text

  https://erar.springeropen.com/articles/10.1186/s43166-023-00181-1

  The clinical findings of Behets disease (BD) differ according to the country and race investigated. […] The most important genetic factor known in the pathogenesis of BD is HLA-B51, and this positivity is high in countries on the Silk Road where BD is as frequent as it is in Turkey. […] Although the positivity of HLA B51 is proven to be high in Turkey, there are no studies in the area of the western Black sea demonstrating its relation to the demographic. […] Our study aimed to investigate the association of HLA-B51 positivity in Turkish patients diagnosed as having BD and the relationship between the demographic and clinical findings of the patients. […] BD has recurrent oral and genital ulcers as a prominent feature and usually follows a relapsing-remitting course. […] The etiopathogenesis of BD is still unknown, but it is a multifactorial chronic disease triggered by environmental, microbiologic, and immunologic factors in individuals with a genetic predisposition such as human leukocyte antigen (HLA-B51).

• #4 Epidemiological clinical behavior of Behçet’s disease | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-epidemiological-clinical-behavior-behcet39s-disease-S2444440516300206

  Epidemiological clinical behavior of Behet’s disease […] Behet’s disease is defined as a vasculitis affecting all types of vessels, presenting with local and systemic clinical manifestations that may involve concern any organ or organ system. […] A worldwide distribution is described; being reported more frequently in the countries corresponding to the antique Silk Road (Iran, Iraq, Uzbekistan, Turkmenistan, China and Turkey) and less frequently in Northern Europe and the United States. It is estimated that Turkey is the country with the highest incidence, with 80420 cases per 100,000 inhabitants. In countries like Japan, Korea, China, Iran and Saudi Arabia it fluctuates between 13 and 22 cases per 100,000 inhabitants. In the American countries it has been reported as a rare disease. […] It is reported that BD mainly affects young adults between the third and fourth decades of life. In relation to the gender, is described that it varies from country to country; for example, in the Middle East of Asia, including Israel, Egypt and Turkey, is more common in males, with a ratio of 35 men for every woman; in the reports from Germany, Japan, the United States and Brazil is more frequent in women.

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