Prevalence Of Behçet's Disease Research Articles

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency

ObjectiveTo describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. MethodsA systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. ResultsTwelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. ConclusionsThe prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Clinical Manifestations of Behçet's Disease: A Retrospective Cross-Sectional Study.

Behçet's Disease (BD) is a systemic vasculitis, highly prevalent in Eastern Asia to Mediterranean countries. Iran is among the countries with the highest prevalence of BD, and previous studies in different countries have shown a broad range of clinical manifestations of the disease. The present study is conducted to evaluate the prevalence of the clinical manifestations of BD in patients referring to rheumatology clinics of two distinct referral hospitals in Tehran and Zanjan, Iran. In this retrospective, cross-sectional study, the medical records of patients with BD were reviewed, and age at onset, sex, the delay between the onset of symptoms and diagnosis, clinical manifestations, HLA B27, HLA B51, HLA B5, haematuria, proteinuria, leukocyturia, Erythrocyte Sedimentation Rate (ESR), and pathergy phenomenon were included in the study. The collected data were analysed by χ2 test using SPSS 23. A total of 188 patients (Male/female ratio = 1.47) were included in the study with mean ± SD age at onset of 27.98 ± 10.47 years and a mean ± SD of delay between the onset of symptom and diagnosis of 5.70 ± 7.16 years. The most common clinical manifestation was mucosal involvement (85.1%), followed by the ocular lesion (55.3%) and skin manifestations (44.7%). The Pathergy phenomenon was observed in 98 patients (52.1%). Moreover, 45.2% had positive HLA B5, followed by HLA B51 (35.1%) and HLA B27 (12.2%). This study demonstrated that male/female ratio and mean age at onset were comparable to the results of previous studies in Iran. Significant associations between HLAB5 and clinical manifestations underline the pivotal role of genetic factors in BD.

Diverse selection pressures shaping the genetic architecture of behçet disease susceptibility.

Behçet disease (BD) is a polygenic, multifactorial, multisystem inflammatory condition with unknown etiology. Global distribution of BD is geographically structured, highest prevalence observed among East Asian, Middle Eastern, and Mediterranean populations. Although adaptive selection on a few BD susceptibility loci is speculated, a thorough evolutionary analysis on the genetic architecture of BD is lacking. We aimed to understand whether increased BD risk in the human populations with high prevalence is due to past selection on BD associated genes. We performed population genetics analyses with East Asian (high BD prevalence), European (low/very low BD prevalence), and African (very low/no BD prevalence) populations. Comparison of ancestral and derived alleles’ frequencies versus their reported susceptible or protective effect on BD showed both derived and ancestral alleles are associated with increased BD risk. Variants showing higher risk to and more significant association with BD had smaller allele frequency differences, and showed less population differentiation compared to variants that showed smaller risk and less significant association with BD. Results suggest BD alleles are not unique to East Asians but are also found in other world populations at appreciable frequencies, and argue against selection favoring these variants only in populations with high BD prevalence. BD associated gene analyses showed similar evolutionary histories driven by neutral processes for many genes or balancing selection for HLA (Human Leukocyte Antigen) genes in all three populations studied. However, nucleotide diversity in several HLA region genes was much higher in East Asians suggesting selection for high nucleotide and haplotype diversity in East Asians. Recent selective sweep for genes involved in antigen recognition, peptide processing, immune and cellular differentiation regulation was observed only in East Asians. We conclude that the evolutionary processes shaping the genetic diversity in BD risk genes are diverse, and elucidating the underlying specific selection mechanisms is complex. Several of the genes examined in this study are risk factors (such as ERAP1, IL23R, HLA-G) for other inflammatory diseases. Thus, our conclusions are not only limited to BD but may have broader implications for other inflammatory diseases.

Epidemiology and clinical domains of Behçet's disease in the Cantabria region, Northern Spain.

The prevalence of Behçet's 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. 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 Behçet's 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 49.7±14.7 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 inhabitants 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 for the entire population. Clinical phenotypes observed are similar to those described in other world regions.

Epidemiologic and Etiological Features of Korean Patients With Behçet's Disease.

Behçet's disease (BD) is a multisystem disease in which environmental factors provoke an adverse immune response in patients with genetic susceptibility towards BD, subsequently leading to a cascade of dysregulated inflammation throughout the body. It is particularly prevalent in regions spanning the ancient Silk Road, including Korea, where the first known case of BD was reported in 1961. We summarize the history, epidemiology, and clinical presentation of BD in Korea, highlighting the clinical tendencies that are particularly seen in the Korean BD population as compared to European populations. Analysis of epidemiologic trends over the past three decades in Korea shows a decreasing prevalence of complete BD and a higher prevalence of intestinal BD. We also discuss the ever-evolving understanding of the pathogenesis of BD, noting the complex interplay among genetics, environment, and immunology. The HLA-B51 allele is the most significant known genetic risk factor in developing BD. We also discuss more recently studied associations between BD and immune factors such as IL-10, IL-23R-IL-12RB2, IL-1A-IL-1B, CCR1, ERAP1, and the GIMAP cluster, the last of which has been found to have an association with BD specifically in Korea. Environmental factors such as pollution and microbials are often the inciting event in developing BD, as they trigger an imbalanced immune response in genetically susceptible individuals, one that has been often found to exhibit an aberrant Th1/Th17 response. There would be value to further studying the pathogenesis and clinical characteristics of Korean BD.

Contribution of Arab countries to Behçet disease research: a PubMed-based bibliometric and altmetric analysis.

Behçet's disease (BD), a rare multisystemic disorder, has an unknown etiology. Interactions between genetic and environmental factors play a major role in this disorder. Human leukocyte antigen B51 allele is the strongest risk factor for the development of Behçet disease. The high prevalence of BD in the Arab world makes it necessary to undergo local research and publications, to target particular genetic, geographical, or even cultural risk factors. This study aims to assess the numerical contribution of the Arab world to research on Behçet disease. Using the PubMed platform, the number of BD-related publications from 2005 to 2019 was assessed for all 22 Arab countries, and countries having the highest prevalence of the disease. Number of publications on BD was normalized to the average population size and GDP for each Arab country. From 2005 to 2019, Arab countries published 198 articles related to BD. This accounts for 0.1% of the total number of BD-related articles published in this period (4170). A significant, positive correlation of moderate strength exists between the number of publications and the average population, while a non-significant, positive correlation of week strength exists between the number of publications and GDP. Concerning the top 15 countries according to Behçet disease prevalence, Turkey had the greatest number of articles, while the USA had the highest ratio of publications per prevalence. The high prevalence of BD in Arabic countries was faced by paradoxically low research activity in this field. Arab countries are lagging behind in the field of Behçet disease-related research, which highlights the need for improving research productivity.

Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With SLCO2A1 Gene, and Intestinal Behçet's Disease in Japan in 2017: A Nationwide Survey.

BackgroundCronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet’s disease (BD) are classified as intractable intestinal disorders in Japan. However, the national prevalence of these diseases remains unknown. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017.MethodsWe conducted a mail-based survey targeting hospitals across Japan to estimate the annual numbers of patients with CCS, CEAS, and intestinal BD in 2017. Using a stratified random sampling method, we selected 2,979 hospital departments and asked them to report the number of patients who met specific diagnostic criteria. The total number of patients for each disease was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. The corresponding prevalence rates per 1,000,000 population were calculated based on the mid-year population of Japan in 2017.ResultsThe overall survey response rate was 68.1% (2,029 departments). The estimated numbers of patients with CCS, CEAS, and intestinal BD were 473 (95% confidence interval [CI], 357–589), 388 (95% CI, 289–486), and 3,139 (95% CI, 2,749–3,529), respectively; the prevalence rates per 1,000,000 population were 3.7 (male: 4.0; female: 3.5), 3.1 (male: 3.0; female: 3.1), and 24.8 (male: 24.5; female: 25.0), respectively. The male-to-female ratios were 1.10, 0.94, and 0.93 for patients with CCS, CEAS, and intestinal BD, respectively.ConclusionsEstimates of the national prevalence of CCS, CEAS, and intestinal BD in Japan were generated and found to be higher than those previously reported.

Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature

Behcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases reported in the literature. The prevalence of BD is variable globally, with Turkey having the highest prevalence, with 370 cases/100,000 population. Out of the 17 criteria proposed for the diagnosis of BD, the most commonly used criteria are the International Study Group (ISG) on BD criteria and the “International Criteria of BD” (ICBD) criteria. We present a case of a 40-year-old female who presented with fever of unknown origin, oral ulcers, odynophagia, and a rash over the face, extremities, and trunk. She developed acute-onset bilateral painful red eyes in the hospital with marked diminution of vision and persistent tachycardia. On evaluation, she was diagnosed to have bilateral intermediate uveitis based on the clinical finding of conjunctival congestion, and 1+ cells in the anterior chamber of both eyes, dispersion of iris pigments on the anterior surface of lens bilaterally, and Grade 1 vitreous haze on slit-lamp examination. The patient had persistent tachycardia for which an electrocardiogram was done, which revealed nonspecific ST-changes. Echocardiography was done, which showed a left ventricular ejection fraction of 40%, with global hypokinesia, suggestive of myocarditis. Our patient fulfilled the ICBD criteria, and she tested positive for human leukocyte antigen B51. She also had persistent transaminitis, and on evaluation, she was found to have occult hepatitis B infection with possible reactivation. The final diagnosis of BD with myocarditis and occult hepatitis B infection was established. She was managed with oral glucocorticoids, colchicine, azathioprine, and entecavir with a favorable outcome. BD causing myocarditis is an infrequent presentation, and we could find only 15 cases reported in the literature since 1947 when Dr. Hulusi Behcet first described the disease.

The Prevalence of Familial Mediterranean Fever and Behçet's Disease: A Cross-Sectional Study.

Objectives This study aims to investigate the coexistence of familial Mediterranean fever (FMF) and Behçet’s disease (BD).Patients and methods This cross-sectional study was conducted between May 2009 and August 2009. The study included 14,881 randomized children (7,741 males, 7,140 females; mean age 13.0 years; range, 12 to 14 years) from sixth to eighth grades, in 72 primary schools in the center of Turkey’s Sivas province. Of these children, 985 were randomly selected and interviewed with their parents. During these interviews, the family trees up to second-degree relatives were drawn. The presence of a diagnosis of FMF or BD was questioned. Patient history, physical examination, eye examination, and pathergy test were performed when needed. The methods of this study were reported in accordance with the STrengthening the Reporting of OBservational studies in Epidemiology guidelines.Results Nine hundred and eighty-five students, 978 mothers, 953 fathers, and 1,876 relatives (4,792 in total) were included in the study. The ratio of consanguineous marriage ratio was 13.6%. Only 30 patients (0.6%) were diagnosed with FMF, while three (0.06%) were diagnosed with BD. In patients with FMF, consanguineous marriage was statistically significant (p=0.015). In terms of low back, heel, and joint pain and morning stiffness, there was a statistically significant difference between patients with and without FMF (p<0.05). Of the three BD patients, one had concomitant FMF.Conclusion The prevalence of FMF in Sivas province was higher than Turkey’s prevalence; however, the prevalence of BD was lower. According to these findings, it is not easy to conclude that the two diseases share a common pathogenesis.

Headache in Behçet’s Disease

Our objective is to describe the various neurologic manifestations of Behcet's syndrome with special attention to headache syndromes. Most recent studies have focused on data collection to better characterize the relatively rare disorder of neuro-Behcets (NBD). In patients with Behcet's disease (BD) who are experiencing headache, most are primary headache disorders, specifically tension type or migraines. Worsening headache can, however, be a sign of increased Behcets disease activity. Behcet's disease is a chronic, systemic, inflammatory disorder of unknown etiology. The prevalence of BD is most notable in countries along the ancient Silk Road, particularly the Middle East. BD occasionally has neurologic manifestations, further categorized into parenchymal and non-parenchymal syndromes. Headache is the most frequently reported neurological symptom of BD. Primary headaches are the most common headache type among patients with BD with comorbid headache disorders with debate regarding whether migraine or tension-type headache is most prevalent. Of the secondary causes of headache in BD, cerebral venous thrombosis is the most common.

HLA-B*51 allelic and carrier frequency in Kazakhstan: insights into Behçet's disease prevalence in Central Asia.

Behçet's disease (BD) is a systemic vasculitis whose prevalence varies according to the ethnicity. HLA-B*51 is a well-known genetic factor predisposing to BD: populations with higher HLA-B*51 allelic frequencies are also those with a greater disease prevalence, as occurs in the Silk Road countries. However, the BD prevalence is unknown in many countries, including Kazakhstan and, in general, Central Asia, despite the location on or near the Silk Road. Due to the lack of clinical studies from this area, we analyzed the bone marrow donors registry in Kazakhstan, in order to assess the HLA-B*51 allelic and carrier frequencies, which resulted to be 8.14 and 15.28%, respectively. Through a comparison with the same parameters in other countries (by performing a data search in the medical literature and in the Allele Frequency Net Database), we might speculate a BD prevalence in Kazakhstan of 10-15/100.000 at least. This result should prompt a greater clinical consideration of this disease in Central Asia, as well as the publication of clinical data from this geographic area.

Behçet's disease in Egypt: a multicenter nationwide study on 1526 adult patients and review of the literature.

The present work was conducted to estimate the prevalence of adult Behçet's disease (BD) in adult Egyptian and to study the clinical pattern and influence of age at-onset and sex on disease phenotype. Also, we investigated the spectrum of presentation and frequencies along the north-to-south gradient of the country. The population-based, multicenter, cross-sectional study included 1526 adult BD patients from 26 specialized Egyptian rheumatology centers. Demographic, clinical, and therapeutic data are assessed for all patients. The mean age of patients was 35.7 ± 9.84years, disease duration 6.58 ± 5.25years, and age at onset 29.37 ± 8.6years; 91 were juvenile-onset (JoBD). There were 1102 males and 424 females (M:F 2.6:1). Regarding co-morbidities, 19.92% were diabetic, and 26.05% were hypertensive. The mean body mass index was 27.57 ± 5.24 (43.1% overweight; 25.9% obese). The mean BD current activity form was 4.48 ± 4.28. Regarding the medications use, systemic steroid and colchicine were the most common drugs used (947 (90.2%) and 611 (82.7%), respectively). The overall estimated prevalence of BD in Egypt was 3.6/100,000 population being highest in the two main cities: Alexandria (15.27) and Cairo (8.72). Pathergy test was positive in 43.4%. 90.2% were receiving systemic steroids and 8.3%, biologics. Disease characteristics were comparable between JoBD and adult-onset BD cases. Central nervous system (CNS), deep venous thrombosis (DVT), and gastrointestinal (GIT) involvement were significantly higher in males (p = 0.01, p = 0.001, and p = 0.001 respectively) while joint affection (p = 0.001) and disease activity (p = 0.011) were increased in females. This study provides current prevalence of BD in Egypt; 3.6/100,000 with no remarkable north-to-south gradient. The sex influences the disease phenotype with the CNS, DVT, and GIT involvement are higher in males, while the joint affection and disease activity were increased in females. • The prevalence and phenotype of Behçet's disease across Egypt is presented in a multicenter nationwide study. • The potential influence of the age at onset and sex on disease phenotype is highlightened. • A review of the literature worldwide is presented allowing comparisons with studies from other nations.

Update on the epidemiology, risk factors and disease outcomes of Behçet's disease.

Behçet's disease (BD) may be regarded as a polygenic autoinflammatory disease although adaptive immune system has also been implicated in pathogenesis. Different classification criteria sets exist for BD, including the new "International Criteria for BD." The pooled prevalence of BD was calculated as 10.3 per 100,000 population globally. BD is common along the Silk Road, including Turkey. Male sex and early onset are associated with a more severe disease course. For the follow-up of BD, there are five disease activity scales, one disease severity scale, and one QOL scale.

Frequency of Behçet's disease among a group of visually impaired adults.

Behçet's disease (BD) is one of the reasons of acquired visual impairment among young adults. Ocular involvement is a significant cause of disability in BD. The objective of this study is to assess the prevalence of BD among a group of adults who has visual impairment. Ankara Metropolitan Municipality Education and Technology Center is one of the official institutions which records and follows the demographic data of visually impaired people in Turkey. In November 2014, there were 675 visually impaired people recorded at this center. Medical history was taken from 294 adults by phone in November and December of 2014. Participants were asked if the visual impairment had been either acquired or congenital. If the patients had BD or suspicious BD, they were recalled for detailed investigation which would be carried out by an internist, a rheumatologist and an ophthalmologist. Two hundred thirteen of 294 (72.4%) visually impaired adults were male. One hundred nine of 294 (37.1%) had acquired visual impairment. Six (5.5%) of those 109 patients had BD. Overall prevalence of BD among study group was 2.04%.The median age of people with BD was 35years. The median age at BD diagnosis was 16.5years and the median duration from diagnosis to visual loss was 2.5years. BD is still one of the causes of acquired visual impairment in Turkey. In this study, BD prevalence among a visually impaired adult group was 2.04%. BD accounted for 5.5% among adults who had acquired visual impairment. In a study of 1965, BD prevalence among people with acquired blindness was 12%. However, this study was conducted at pre-immunosuppressive period. Our prevalence is obviously lower than those studies. Extended population-based studies are needed for population estimations.

Behçet disease: a rare systemic vasculitis in Poland.

INTRODUCTION Behçet disease (BD) is an immune‑mediated small‑vessel systemic vasculitis, which is rarely seen in Poland. OBJECTIVES The aim of this study was to evaluate the incidence and prevalence of BD, as well as to assess the sex and geographical distribution of BD in Poland during a 7‑year follow-up. To our knowledge, this is the first evaluation of this rare disease in Poland, based on a hospital morbidity database. PATIENTS AND METHODS We analyzed population‑based administrative data obtained from a national hospital morbidity study conducted between January 2008 and December 2014 by the National Institute of Public Health in Poland. The annual incidence rates and point prevalence of BD were calculated using the number of patients with BD and corresponding census data for the overall Polish population. RESULTS Data included 316 hospitalization records. The final study sample comprised 130 patients (54 men [42%] and 76 women [58%]) with first‑time hospitalizations for BD. The mean (SD) age was 41.6 (18.7) years (95% confidence interval [CI], 38.3-44.8; range, 5-85 years). The incidence rate of BD was estimated at 0.5 per million persons per year (95% CI, 0.35-0.61). The point prevalence at the end of 2014 was 3.4 per million persons. The incidence rate of BD did not vary significantly between more urban and more rural regions, and BD was observed more often in women than in men. CONCLUSIONS BD is endemic in Eastern and Central Asian countries, but is also seen in Poland. However, its incidence and prevalence rates are lower in Poland than in other European countries.

Exploring the variability in Behçet’s disease prevalence: a meta-analytical approach

Surveys of Behçet's disease (BD) have shown substantial geographic variations in prevalence, but some of these differences may result from methodological inconsistencies. This meta-analysis explored the effect of geographic location and study methodology on the prevalence of BD. We systematically searched the literature in electronic databases and by handsearching to identify population-based prevalence surveys of BD. Studies were eligible if they provided an original population-based prevalence estimate for BD with the number of prevalent cases identified in the study area. Pooled prevalence proportions across all studies were computed by using random effects models based on a Poisson normal distribution. Pre-defined subgroup analyses and meta-regression were used to investigate the effect of covariates on the prevalence proportions. We included 45 reports published from 1974 to 2015 and covering worldwide areas. The pooled estimates of prevalence proportions (expressed as cases/100 000 inhabitants) were 10.3 (95% CI 6.1, 17.7) for all studies and 119.8 (59.8, 239.9) for Turkey, 31.8 (12.9, 78.4) for the Middle East, 4.5 (2.2, 9.4) for Asia and 3.3 (2.1, 5.2) for Europe. Subgroup analyses showed a strikingly greater prevalence for studies with a sample survey design than a census design [82.5 (95% CI 47.3, 143.9) vs 3.6 (2.6, 5.1)]. Metaregression identified study design as an independent covariate significantly affecting BD prevalence proportions. Differences in BD prevalence proportions likely reflect a combination of true geographic variation and methodological artefacts. In particular, use of a sample or census study design may strongly affect the estimated prevalence.

Evaluation of olfactory function in Behçet's disease.

Behçet's disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology and is characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous system manifestations. Few cases involving the nasal mucosa have been reported in the literature, and the true prevalence of BD remains unknown. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement, but sufficient clinical data are not available on the effect of BD on olfaction in adults. We therefore evaluated the olfactory function of patients diagnosed with BD. Patients were chosen from among a consecutive patient group population who visited the internal medicine rheumatology polyclinic and otolaryngology departments of Ondokuz Mayıs University Hospital. A total of 50 patients (both males and females) aged 18 to 60 years with a diagnosis of BD and 46 healthy controls (matched to the study group in terms of age and gender) were included. BD was diagnosed based on the criteria defined by the International Study Group for BD. A complete clinical history was taken for and a physical examination was performed in all participants. Patients with other rheumatic diseases; obstructive nasal pathologies leading to conductive-type olfactory dysfunction (e.g., septum deviation or nasal polyp); advanced systemic disease (e.g., hypertension or malignancy); a history of antithyroid, antihistamine, antidepressant, or steroid medication use within the past month; or who were current smokers, had an active upper respiratory infection, or had a history of otolaryngologic operations were excluded. The results of the "Sniffin' Sticks" (SS) olfactory test were compared between the two groups. The mean age of the 50 BD patients was 35.3±10 years; that of the 46 health controls was 36.9±11 years. There was no significant group difference in age or gender distribution (p>0.05). Odor identification and overall scores were significantly lower in the BD group than in the control group. There were no significant differences in odor discrimination scores between the BD and control groups (p>0.05). To our knowledge, this is the first study to evaluate olfactory function in patients diagnosed with BD using the SS test. Odor identification was more impaired in BD patients than in healthy controls, but there was no group difference in odor discrimination. BD patients should also be assessed for the involvement of olfactory function and may require treatment due to a malfunction of the olfactory system that affects the quality of life.

Bibliometric analysis on global Behçet disease publications during 1980-2014: is there a Silk Road in the literature?

Behçet disease (BD) is a chronic and multisystemic vasculitis characterized with recurrent oral and genital ulcers, uveitis, arthritis and skin manifestations. The highest prevalence of the disease has been reported in regions historically involved in the Silk Road routes. We aimed to analyse Behçet literature and evaluate whether there is a concordance between ancient Silk Road regions and the distribution of publication productivity. The bibliometric analysis of the publications published during 1980-2014 using Thomson Reuters Web of Science database was performed. We generated infographics of productivity and also analysed the correlations among economical productivities, technological advancement levels and humanity index and productivity performances of 78 countries in Behçet literature. Turkey ranked first in Behçet literature with 1837 articles followed by Japan and the USA. Turkey, Tunisia and Israel occupied the first three places in productivity. Significant correlations were noted between 2014 gross economic and technological indices and publication numbers of the countries. We found that European countries had high productivity, although they had low prevalence of BD. We detected no concordance between the productivity density of the countries and the ancient Silk Road routes. Most publications were reported from developed countries although undeveloped or developing countries had higher prevalence of BD. Physicians in undeveloped and developing countries should be supported and encouraged to perform novel studies on BD.

Investigation of Behçet's Disease and Recurrent Aphthous Stomatitis Frequency: The Highest Prevalence in Turkey.

The Recurrent Aphthous Stomatitis (RAS) is the most frequently observed painful pathology of the oral mucosa in the society. It appears mostly in idiopathic form; however, it may also be related with systemic diseases like Behçet's Disease (BD). Determining the prevalence of RAS and BD in the Northern Anatolian Region, which is one of the important routes on the Antique Silk Road. Cross-sectional study. Overall, 85 separate exemplification groups were formed to reflect the population density, and the demographic data of the region they represent. In the first stage, the individuals, who were selected in random order, were invited to a Family Physician Unit at a certain date and time. The dermatological examinations of the volunteering individuals were performed by only 3 dermatology specialists. In the second stage, those individuals who had symptoms of BD were invited to our hospital, and the Pathergy Test and eye examinations were performed. The annual prevalence of RAS was determined as 10.84%. The annual prevalence was determined to be higher in women than in men (p=0.000). It was observed that the prevalence was at the peak level in the 3(rd) decade, and then decreased proportionally in the following decades (p=0.000). It was also observed that the aphtha recurrence decreased in the following decades (p=0.048). The Behçet's prevalence was found to be 0.60%. The prevalence in women was found to be higher than in men (0.86% female, 0.14% male; p=0.022). While the RAS prevalence ratio was at an average value when compared with the other societies; the BD prevalence was found as the highest ratio in the world according to the literature.

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.