Behçet’s Disease Research Articles

The influence of HLA-B51 on clinical manifestations among Japanese patients with Behçet’s disease: A nationwide survey

Objectives: To scrutinize the influence of HLA-B51 to each clinical manifestation of patients with Behçet’s disease (BD) using a database of the Ministry of Health, Labour and Welfare of Japan.Methods: The database of newly registered patients with BD was obtained from the Japanese Ministry of Health, Labour and Welfare. Patients who met International Criteria for Behçet's Disease (ICBD) and had data for HLA-B51 were selected and analyzed.Results: Among the 3044 analyzable cases, 1334 (43.8%) were men and 1710 (56.2%) were women; the median age was 38 years (IQR 29–48). HLA-B51 was positive for 1334 (44.5%). Prevalence of selected manifestations was 98.5% for oral ulceration, 85.5% for skin lesion, 42.1% for ocular lesion, 69.1% for genital ulceration, and 29.0% for gastrointestinal symptom. HLA-B51-positive patients had higher risk for ocular lesion (OR 1.59, 95%CI: 1.37–1.84; p < .001) and lower risk for genital ulceration (OR 0.72, 95%CI: 0.62–0.84; p < .001) and gastrointestinal symptom (OR 0.65, 95%CI: 0.55–0.77; p < .001). No significant difference was observed for other organ involvement; oral ulceration, skin lesion, positive pathergy test, arthritis, epididymitis, vascular lesion, or neurological manifestation. Subgroup analyses revealed that HLA-B51 was not related to genital ulceration in the cases with an ICBD score of 6 or higher and that HLA-B51 tended to more largely affect the risk of three manifestations for men compared to that for women.Conclusion: HLA-B51 positive is a risk factor for ocular lesion and vice versa for genital ulceration and gastrointestinal symptoms in patients with Japanese BD.

MICA*049, not MICA*009, is associated with Beh\xe7et\u2019s disease in a Chinese population

Behçet’s disease (BD) is a multi-systemic inflammatory disease. Previous reports indicated that MICA*009 confers susceptibility to BD. MICA*049 differs from MICA*009:01, a major MICA*009 subtype, only at codon 335 in exon 6. However, the potential association of MICA*049 with BD has not been addressed. In this study, we differentiated association among MICA*049, MICA*009 and HLA-B*51 with BD. A Han Chinese cohort consisting of 41 BD patients and 197 ethnically matched controls were examined with sequencing and T-ARMS-PCR for genotyping of MICA, and ARMS-PCR for HLA-B*51. The phenotype frequency of MICA*049 (41.5% versus 8.1%, OR = 8.01, P = 1.91 × 10−8) and HLA-B*51 (46.3% versus 15.7%, OR = 4.62, P = 1.21 × 10−5) were significantly higher in BD patients than those in controls, whereas MICA*009 showed no significant difference between the two groups (17.1% versus 13.2%, OR = 1.35, P = 0.51). After stratification for the effect of HLA-B*51, MICA*049 was still associated with BD in HLA-B*51 negative patients (OR = 40.61, P = 0.02). Our results indicate that MICA*049, not MICA*009, is a risk factor to BD, and that is independent from HLA-B*51 in the Han Chinese cohort.

Comparison of nitric oxide level in Behçet’s disease patients with or without psychiatric comorbidity

ABSTRACTBoth Behçet’s disease (BD) and major depressive disorder (MDD) are diseases associated with nitric oxide (NO). We hypothesized that the comorbidity of MDD affects the levels of NO in BD. In this study, we investigate whether there was a difference in NO levels among BD patients with accompanying MDD, BD patients with no depressive symptoms and healthy control group. There was a significant difference in NO levels among BD and control group (P < 0.05). Also, there was a significant difference between the BD group with MDD and BD group without psychiatric comorbidity in terms of NO levels (P < 0.05). This study is interesting as it demonstrates that accompanying psychiatric comorbidity puts an additional NO burden on the shoulders of BD patients.

Clinical and Demographical Characteristics of Familial Behçet’s Disease (Southeast Marmara Region)

Background: Familial aggregation in Behçet’s disease (BD) has been reported in Turkish and Japanese populations. While the frequency of familial cases has been reported to be 2–5% worldwide, this rate reaches up to 15% in the Middle East. Objective: This study aimed to determine the incidence of familial BD cases followed in the BD polyclinic and to compare their clinical and demographic characteristics to those observed in sporadic cases. Methods: Data related to BD patients who were followed between 1995 and 2014 were collected from computerized archive records and were assessed for detailed family histories. Only first-degree relatives (brother, sister, mother, father, children) were considered to be cases of familial BD. Clinical and demographic ­features were retrieved. Our BD polyclinic is located in the Southeast Marmara Region in Turkey. Results: BD was detected in 36 first-degree relatives of 33 patients out of 840 patients with BD. A total of 45 patients were diagnosed as familial BD;23 were female, and 22 were male. In our patients, the incidence of familial BD was determined to be 3.9%. The rates for HLA-B5 positivity, ocular involvement, genital ulcers, and erythema nodosum were determined to be 86.6% (26/30), 26.6%, 82.2%, and 60%, respectively. None of the patients had neurological involvement, but 2 had vascular involvement. Conclusion: This study may contribute to the epidemiological data of BD from Turkey.

Local Cytokine Expression Profiling in Patients with Specific Autoimmune Uveitic Entities

ABSTRACTPurpose: To evaluate expression of cytokines GM-CSF, IL-11, IL-12p40, IL-12p70, IL-27p28, IL-35, APRIL, BAFF, TWEAK, and LIGHT in uveitis.Methods: Aqueous humor samples from patients with active uveitis associated with Behçet’s disease (BD), sarcoidosis, HLA-B27-related inflammation, and Vogt-Koyanagi-Harada (VKH) disease and control patients were assayed with a multiplex assay.Results: Comparing all patients to controls, GM-CSF, IL-11, IL-12p40, APRIL, and BAFF were significantly increased, whereas LIGHT was significantly decreased. IL-11 and BAFF were the most strongly upregulated, being elevated 19.7-fold and 14.1-fold, respectively, compared with controls. IL-11 was significantly highest in HLA-B27 uveitis. GM-CSF, IL-11, and IL-12p40 were significantly higher in nongranulomatous uveitis (BD and HLA-B27) than in granulomatous uveitis (sarcoidosis and VKH), whereas APRIL and TWEAK were significantly higher in granulomatous uveitis.Conclusions: IL-11-driven immune responses might be more potent in nongranulomatous uveitis, particularly in HLA-B27 uveitis. BAFF and APRIL might contribute to B cell-driven autoimmune response in uveitis.

Lack of association between serum IL-9 levels and Behçet’s disease

IntroductionConsidering the role of interleukin (IL)-9 and IL-9-producing Th9 cells in pathogenesis of autoimmune diseases, this study aimed to evaluate serum IL-9 levels in patients with Behçet’s disease (BD) compared to healthy subjects and to assess whether there is an association between serum IL-9 levels and disease characteristics in BD. MethodologyIn this cross-sectional study, 32 BD patients according to the International Criteria for BD (ICBD) and 56 age-matched healthy controls were included. In patients, clinical examination was performed and Behcet’s Disease Current Activity Form (BDCAF), Iranian Behcet’s Disease Dynamic Activity Measure (IBDDAM) and Total Inflammatory Activity Index (TIAI) were assessed. Serum IL-9 level was measured using ELISA kit. ResultsThe mean ± SD age of patients and controls was 39.06 ± 9.86 and 38.64 ± 8.40 years, respectively and 41% of patients and 66% of controls were males. The most common clinical symptoms in BD patients were oral aphthous ulcers, ocular involvement, and genital ulcers, respectively. The median (Min–Max) of BDCAF, IBDDAM and TIAI in patients were 2 (0–4), 1.3 (0–7), and 2 (0–22), respectively. There was no significant difference in serum IL-9 levels between BD patients (47.12 ± 7.34 mg/dL) and healthy controls (48.61 ± 7.76 mg/dL) (P > 0.05). There were no significant correlations between serum IL-9 levels with BD clinical characteristics as well as with disease severity (P > 0.05). ConclusionOur study revealed no significant difference in serum IL-9 between BD patients and healthy controls as well as no significant correlation between serum IL-9 with clinical characteristics and disease severity. Further studies are certainly needed, but on a wider cohort of BD patients to identify IL-9 involvement in BD pathogenesis.

Ischemia modified albumin: a useful marker for increased oxidative stress in Behçet’s disease

Background Increased oxidant stress play an important role in pathogenesis of Behçet’s disease (BD). It needs to be clearly defined by using a sensitive marker.Objective We sought to investigate usefulness of ischemia modified albumin (IMA) to show increased oxidative stress in patients with BD and its value considering the disease activity.Methods The sera from BD patients (n=57) and healthy individuals (n=45) were collected. IMA, serum total antioxidative capacity (TAC) and total oxidant status (TOS) were measured using Erel’s automated method, and the percentage ratio of total peroksit level to TAC level was considered the oxidative stress index (OSI). Receiver operating characteristic (ROC) curves were constructed for all markers.Results IMA, TAC, TOS, OSI, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels were found to be significantly higher in patients with BD than those in controls. IMA was the only marker which showed difference between active and inactive periods, and it had higher area under curve (AUC) value than those for other markers in ROC analysis (p=0.004). IMA also showed significant correlations with CRP, both in all BD patients and those in active period (r=0.50, p&amp;lt;0.01; r =0.54, p&amp;lt;0.005, respectively).Conclusions IMA showed superiority to other markers such as TAC, TOS or OSI to evaluate oxidative stress in BD patients as well as in considering disease activity. The higher serum level of IMA and its relationship with CRP observed in active period of BD indicate that IMA may be a useful marker for monitoring disease activity.

The molecular and clinical evidence of vitamin D signaling as a modulator of the immune system: Role in Behçet’s disease

Various tissues and cell types are the targets of vitamin D. However, the major targets of vitamin D in the immune system are monocytes/macrophages, dendritic cells (DCs), as well as B and T cells. Vitamin D plays an important role in the immune system modulation via regulating the expression of genes that generate pro-inflammatory mediators and inhibiting the proliferation of pro-inflammatory cells, both of which have been implicated in the pathophysiology of the inflammatory diseases. Recent studies have revealed the important relations between vitamin D and Behçet’s disease (BD). Vitamin D function and its deficiency have been linked to a wide range of metabolic disorders including malignant, cardiovascular, infectious, neuromuscular, and autoimmune diseases. Here, we provide a brief analysis of the recent literature regarding immune-regulatory effects as well as clinical evidence of vitamin D influence on the molecular level in BD.

The association analysis between HLA-A*26 and Beh\xe7et\u2019s disease

The strongest genetic risk factor of Behçet’s disease (BD) is HLA-B*51. Our group previously reported that HLA-A*26 is independently associated with the risk of the onset of BD apart from HLA-B*51. Here, we re-evaluated the association between HLA-A*26 and BD in the Japanese population. We also performed a comprehensive literature search and meta-analyzed the extracted published data concerning the relationship between HLA-A*26 and BD to estimate the odds ratio (OR) of HLA-A*26 to BD. In this study, we genotyped 611 Japanese BD patients and 2,955 unrelated ethnically matched healthy controls. Genotyping results showed that the phenotype frequency of HLA-A*26 was higher in BD patients than in controls (OR = 2.12, 95% CI: 1.75–2.56). Furthermore, within the HLA-B*51-negative populations, the phenotype frequency of HLA-A*26 was significantly higher in BD patients than in controls (OR = 3.10, 95% CI: 2.43–3.95). Results obtained from meta-analysis combined with our data showed that the modified OR of HLA-A*26 became 1.80 (95% CI:1.58–2.06), whereas within the HLA-B*51-negative population, the modified OR became 4.02 (95% CI: 2.29–7.05). A subgroup analysis arranged by the geographical regions showed HLA-A*26 is in fact associated with the onset of BD in Northeast Asia (OR = 2.11, 95% CI: 1.75–2.56), but not in the Middle East or in Europe.

Epigenome-wide association study identifies Behçet's disease-associated methylation loci in Han Chinese.

The aetiology of Behçet's disease (BD), known as a systemic vasculitis, is not completely understood. Increasing evidence suggests that aberrant DNA methylation may contribute to the pathogenesis of BD. The aim of this epigenome-wide association study was to identify BD-associated methylation loci in Han Chinese. Genome-wide DNA methylation profiles were compared between 60 BD patients and 60 healthy controls using the Infinium Human Methylation 450 K Beadchip. BD-associated methylation loci were validated in 100 BD patients and 100 healthy controls by pyrosequencing. Gene expression and cytokine production was quantified by real-time PCR and ELISA. A total of 4332 differentially methylated CpG sites were associated with BD. Five differentially methylated CpG sites (cg03546163, cg25114611, cg20228731, cg23261343 and cg14290576) revealed a significant hypomethylation status across four different genes (FKBP5, FLJ43663, RUNX2 and NFIL3) and were validated by pyrosequencing. Validation results showed that the most significant locus was located in the 5'UTR of FKBP5 (cg03546163, P = 3.81E-13). Four CpG sites with an aberrant methylation status, including cg03546163, cg25114611, cg23261343 and cg14290576, may serve as a diagnostic marker for BD (area under the receiver operating curve curve = 83.95%, 95% CI 78.20, 89.70%). A significantly inverse correlation was found between the degree of methylation at cg03546163 as well as cg25114611 and FKBP5 mRNA expression. Treatment with a demethylation agent, 5-Aza-2'-deoxycytidine resulted in an increase of FKBP5 mRNA expression and a stimulated IL-1β production. Our findings suggest that aberrant DNA methylation, independently of previously known genetic variants, plays a vital role in the pathogenesis of BD. Chinese Clinical Trial Registry, chictr.org.cn, ChiCTR-CCC-12002184.

Comparisons of Clinical Features in Japanese Patients with Behçet’s Uveitis Treated in the 1990s and the 2000s

ABSTRACTPurpose: We investigated clinical characteristics of ocular Behçet’s disease (BD) patients treated in the 1990s and the 2000s.Methods: We retrospectively examined records of 68 newly arrived patients with ocular BD followed for more than 4 months during the 2000s and compared to those of 107 patients during the 1990s. Patient profiles, ocular and systemic symptoms, frequency of ocular attacks, BD ocular attack score 24–6 months (BOS24-6M), best-corrected visual acuity (BCVA), and immunomodulatory treatment were noted.Results: Clinical characteristics in the 2000s showed increases in iridocyclitis type, intestinal-, vasculo-, and neuro-BD cases, oral corticosteroid, methotrexate, and infliximab therapy usage, cataract and glaucoma surgery, and pseudophakia, and decreases in BOS24-6M and cyclophosphamide usage. BCVA of 20/30 or better at the final visit was slightly increased in the 2000s.Conclusions: Milder ocular BD tendency was seen in cases in the 2000s, whereas the incidence of special type of BD might be increasing.

Beh\xe7et\u2019s disease and genetic interactions between HLA-B*51 and variants in genes of autoinflammatory syndromes

Behçet’s disease (BD) is an immune-mediated systemic disorder with a well-established genetic base. In a previous study, using a next generation sequencing approach, we found many rare variants and some functional polymorphisms in genes related to autoinflammatory syndromes (AID): CECR1, MEFV, MVK, NLRP3, NOD2, PSTPIP1 and TNFRSF1A in our BD cohort. Our strategy did not allow us to establish either number of patients with variants, proportion of individuals accumulating them or relationship with other genetic factors. With the goal to answer these questions, the individual samples were sequenced. Additionally, three functional polymorphisms: NLRP3 p.Gln703Lys, NOD2 p.Arg702Trp and p.Val955Ile were genotyped using TaqMan assays. A total of 98 patients (27.6%) carried at least one rare variant and 13 of them (3.7%) accumulated two or three. Functional regression model analysis suggests epistatic interaction between B51 and MEFV (P = 0.003). A suggestive protective association of the minor allele of NOD2 p.Arg702Trp (P = 0.01) was found in both, B51 positive and negative individuals. Therefore, a high percentage of patients with BD have rare variants in AID genes. Our results suggest that the association of MEFV with BD could be modulated by the HLA molecules; whereas the protective effect of NOD2 p.Arg702Trp would be independent of HLA.

Arterial aneurysms in Behçet’s disease patients: Frequency, clinical characteristics and long-term outcome

IntroductionVasculitis in Behçet’s disease (BD) is common and unique as it can involve blood vessels of any type and any size. Literature on the characteristics of arterial aneurysms (AA) is scanty. Aim of the workTo present the clinical characteristics and long-term outcome of BD patients with AA compared to those without. Patients and methodsThe medical records of 146 BD patients admitted to the Rheumatology Department, Cairo University Hospitals (2004–2017) were retrospectively revised. The frequency and anatomical location of arterial aneurysms were recorded and the associated clinical characteristics analyzed. ResultsThe mean age of the BD patients was 34.3 ± 8.02 years and they were 127 males and 19 females (6.7:1) with disease duration of 6.81 ± 3.67 years. Apart from oro-genital ulcerations; deep venous thrombosis was the most common associated manifestation while the musculoskeletal manifestations were the least. Twenty-seven (18.4%) patients had arterial aneurysms. All of them were males. Pulmonary artery was the most common artery involved (44.4%) followed by abdominal aorta. Surgical intervention was done for 40.7% patients. The mortality rate was 14.8% (4 patients) in those with AA while it was 2.5% (3 patients) in those without. ConclusionArterial aneurysms are common in Egyptian BD patients mostly males. Increased awareness of the rheumatologists and including appropriate imaging techniques for early detection of AA in BD are mandatory. Early detection and prompt treatment with immunosuppressives may improve the disease outcome.

Comparison of Spectral Domain Optical Coherence Tomography (SD OCT) Findings with Laser Flare Photometry (LFP) Measurements in Behçet’s Uveitis

ABSTRACTPurpose: This study aims to compare the spectral domain optical coherence tomography (SD OCT) findings and laser flare photometry (LFP) measurements in Behçet’s disease (BD).Methods: This study was composed of 4 groups as follows: “Group 1: Healthy volunteers, Group 2: Volunteers with BD and no eye involvement, Group 3: Patients with active uveitis with BD, Group 4: Patients with BD in quiescent phase of uveitis”. Laser Flare Photometry (Kowa FM-600, Kowa Acculas, San Jose, CA, USA), SD-OCT and Enhanced Depth Imaging protocol (EDI) (Spectralis®, Heidelberg Engineering Inc., Heidelberg, Germany) and Fundus Florescein Angiography (FFA) (Heidelberg Retina Angiograph 2®; Heidelberg Engineering, Heidelberg, Germany) were performed for all patients.Results: Twenty-four eyes of 12 healthy volunteers (Group 1); 61 eyes without ocular involvement of 31 volunteers with BD (Group 2); 29 active uveitic eyes of 17 patients with BD (Group 3); 35 eyes with quiescent uveitis in 18 patients with BD (Group 4) participated in the evaluation. There was a significant difference between visual acuity, submacular choroidal thickness (SCT), and flare values comparison among the groups(p<0.001,p<0.002, and p<0.001). When compared considering the SCT, choroidal thickness in Group 3 was higher than in the eyes of Group 1 and Group 2 (p:0.005, p:0.006). Central macular thickness (CMT) and disease duration were negatively correlated (rho:−0.453, p<0.001), while CMT and flare values were positively correlated (rho:0.267,p:0.006).Conclusion: Flare is a reliable indicator of the inflammation in Behçet’s uveitis. Its concurrent use with OCT to evaluate choroid and macula enables the assessment of inflammation.

Gastric Focal Neutrophil Infiltration and Wide Duodenal Gastric Foveolar Metaplasia Are Histologic Discriminative Markers for Crohn’s Disease and Behçet’s Disease

Background/Aims: Behçet’s disease (BD) with intestinal lesions and Crohn’s disease (CD) share clinical features. However, no report has compared the 2 diseases with regard to lesions of the upper gastrointestinal tract (UGT). We aimed to evaluate endoscopic and histologic findings of UGT in CD and BD. Methods: We retrospectively assessed the endoscopic records and biopsy samples of 84 Helicobacter pylori-negative patients (50 CD, 34 BD). In duodenal samples, MUC5AC immunohistochemical analysis was performed to identify gastric foveolar metaplasia. Results: In endoscopic findings, bamboo joint-like appearance (17/50 CD, 0/34 BD) and erosions (14/50 CD, 2/34 BD) were significantly more frequent in CD gastric lesions (p < 0.001, and p = 0.012). In histologic findings of stomach, focal neutrophil infiltration in lamina propria (15/48 CD, 1/34 BD) was significantly more frequent in CD (p < 0.001). In that of duodenum, wide gastric foveolar metaplasia (19/49 CD, 1/34 BD) was significantly more frequent in CD duodenal lesions (p = 0.013). The mean maximum width of the gastric foveolar metaplasia was 114.0 ± 10.6 and 29.5 ± 4.5 μm for CD and BD respectively (p = 0.003). Conclusions: In H. pylori-negative patients, gastric focal neutrophil infiltration and wide duodenal gastric foveolar metaplasia were important for distinguishing CD from BD.

Genetics and Epigenetics Mechanism in the Pathogenesis of Behçet's Disease.

Behçet's Disease (BD) is characterized by numerous systemic manifestations and is known for its ability to affect both, arteries and the veins. However, the etiology of BD is only partially understood, and previous studies have demonstrated a role for genetic and epigenetic factors that contribute to disease pathophysiology. Several studies have implicated T cells and monocytes in the pathogenesis of BD especially when these cells are stimulated by heat shock proteins and streptococcal antigen. Furthermore, during disease exacerbations adenosine deaminase has an important role in activating lymphocyte proliferation, maturation, and differentiation in BD. This article presents a review of the published literature mainly from the last 20 years. The topics of main concern were the role of genetic and epigenetic factors as contributing factors in disease pathophysiology. The authors used MeSH terms "Behçet's disease" with "pathophysiology," "pathogenesis," "genetic" or "epigenetic" to search the PubMed database. All the relevant studies identified were included and are described according to the aforementioned subheadings.

Epistatic Interaction of ERAP1 and HLA-B*51 in Iranian Patients with Beh\xe7et\u2019s Disease

Behçet’s Disease (BD) pathogenesis remains unclear, but some genetic loci and environmental factors are proposed to play a role. Here, we investigate the association of the endoplasmic reticulum aminopeptidase-1 (ERAP1) gene variants and HLA-B*51 with BD susceptibility and clinical manifestations in Iranian patients. In the study, 748 BD patients and 776 healthy individuals were included. The MGB-TaqMan Allelic Discrimination method was used to genotype 10 common missense single nucleotide polymorphisms (SNPs) and one intronic SNP in the ERAP1 gene region. We found no significant association between the 11 SNPs and BD in allelic and genotypic association tests. However, rs30187 showed the strongest association with BD in the recessive genotype model of the risk T allele in HLA-B*51 carriers. Although this became insignificant after correcting for multiple comparisons, the homozygous rs30187 risk allele genotype (TT) increased disease susceptibility in HLA-B*51 carriers in epistasis analysis, and the rs30187 TT recessive genotype showed a significant association with risk of cardiac involvement in the all patients and articular involvements in HLA-B*51 positive patients. Our findings suggest that gene-gene interactions between HLA-B*51 and ERAP1 variants is important for BD development, however, ERAP1 variants which interact with HLA-B*51 may differ among disease phenotypes or populations.

Clinical features of early-stage possible Behçet’s disease patients with a variant-type major organ involvement in Japan

Background: Clinical data of patients with entro-, vasculo-, and neuro-variant possible Behçet’s disease (BD) based on Japanese criteria has not yet comprehensively reported.Methods: This ongoing nation-wide registration has been carried out by the Japanese Ministry of Health, Labour and Welfare. The Ministry asked physicians who diagnosed a patient with confirmed or possible BD to register the patient data by filling out a registration form. The Ministry provided us with the dataset after unlinkable anonymization. We analyzed 2003–2014 database generated from the early stage new cases.Results: Among the 7950 analyzable cases, 694 (8.7%) had variant-type possible BD without satisfying complete/incomplete criteria. Of the 694 patients, 479, 46, and 169 had entero-, vasculo-, and neuro-variant possible BD, respectively. Out of these 694 patients, 35 (5.0%) and 154 (22.2%) satisfied the International Study Group criteria and the International Criteria of BD, respectively. Entero-variant possible patients rarely (1.8%) had ocular lesions. Patients with vasculo-variant possible BD were featured by low genital ulceration risk (6.8%) and frequent positive HLA-B51 (60.0%). Neuro-variant possible BD was featured by high median age at registration (48 year). Vasculo- (69.6%) and neuro-variant (68.6%) BD patients showed clear male dominance. Epididymitis was very rare among variant-type possible BD men.Conclusion: We analyzed 694 early-stage variant-type possible BD cases. We believe the data from our study will contribute to further international discussion regarding BD diagnostic criteria and clarification of the clinical presentations of the Japanese variant-type possible BD patients.

The assessment of contributing factors to oral ulcer presence in Behçet's disease: Dietary and non-dietary factors.

ObjectiveThe aim of this study was to assess the contributing factors for oral ulcer activity in Behçet’s disease (BD).MethodsNinety-two patients with BD (F/M: 42/50, mean age: 38.7±10.02 years) participated in this cross-sectional study. Data regarding disease-related factors, smoking patterns, and self-reported dietary/non-dietary triggering factors for oral ulcer activity were collected by a questionnaire. Treatment protocol was categorized as mild and intensive groups associated with organ involvements.ResultsA mild treatment protocol was more common in females (52.4% vs 20%) than in males (p=0.002). During the last three months, the number of oral ulcers in female patients was higher in the mild treatment group (6.4±6.5) than in the intensive treatment group (3.3±4.9) (p=0.045). In patients with active oral ulcers (n=63), rate of being a non-smoker was also higher in females (86.7% vs 63.6%) than in males. Daily frequency of tooth brushing was 1.2±0.8 in patients with BD, and was higher in females (1.5±0.9 vs 0.9±0.6) (p=0.001). Stress and fatigue (78.3%) were reported as the most frequent triggering factors for oral ulcer presence in patients with BD. A total of 148 different dietary factors associated with oral ulcer presence were reported in the study.ConclusionA mild treatment protocol and being a non-smoker were found to be the contributing factors associated with oral ulcer activity in patients with BD. Being motivated for oral hygiene and being non-smokers were positive health behaviors observed in females. Irrespective of gender, stress and fatigue were defined as the most common self-reported triggering factors for oral ulcer presence in BD. In addition, the roles of some dietary factors were also reported.

Olfactory functions in Behçet’s disease: A review

Abstract OBJECTIVES. We reviewed the relationship between olfactory functions and Behçet’s disease (BD). MATERIAL AND METHODS. We searched Pubmed, Google, Google Scholar and Proquest Cebtral Database with the key words of “olfactory”, “functions”, “smell”, “nasal” and “Behçet’s disease”. RESULTS. Behçet’s disease influences the nasal mucosa. Nasal mucosal inclusion causes mucosal ulcers, pain, burning, nasal obstruction, epistaxis, nasal itching and dysosmia. Nasal cartilage deformity is also reported. The higher rate of comorbid chronic rhinosinusitis (CRS) in BD patients may likewise be because of the complex mechanism of the disease inclining the host tissues to bacterial infections. Olfactory functions may decrease in BD. Odor identification may be lower in patients BD. CONCLUSION. An olfactory dysfunction may be seen in patients with BD. BD patients should be evaluated for the involvement of the olfactory function and may require treatment because of a malfunction of the olfactory system that influences the quality of life. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement.