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Objectives: Chronic diseases impact people's quality of life (QoL). Behçet's disease (BD) is a multisystemic chronic disease characterized by vasculitis of various vessels. We aimed to assess QoL in paediatric BD (PEDBD) patients and their parents. Methods: We conducted a cross-sectional study from June to December 2022, including PEDBD patients meeting classification criteria. We recorded clinical characteristics and assessed QoL using PedsQL for patients and WHOQOL-Bref for parents. Results: A study of 38 patients (60.5% girls, 39.5% boys), median age 15.5 years (range 10–17), with oral aphthae and various mucocutaneous symptoms: genital ulcers (78.9%), pseudofolliculitis (76.3%), erythema nodosum (23.6%), and some with uveitis (13.1%), vascular (13.1%), neurological (10.5%), and gastrointestinal (5.2%) involvement. All were in remission under treatment. Median PedsQL scores: total 74.5 (range 40–94.8), physical health 76.5 (43–100), psychosocial health 75 (25–92); 14 patients scored below cutoff. Girls had lower physical health scores (P = .004), and disease duration correlated negatively with PedsQL score (r = -0.648, P = .001). Median WHOQOL score for parents: 50 (25–100), with 20 scoring below cutoff. Conclusion: One-third of PEDBD children and over half of their parents had low QoL. Disease duration and gender, as with many chronic illnesses, correlated with QoL in PEDBD. [ABSTRACT FROM AUTHOR]

Objectives: Specific human leukocyte antigen (HLA) haplotypes are associated with Behçet's disease. Because the effects of HLA-A26 and its combination with HLA-B51 on organ involvement in Behçet's disease have not been well demonstrated, we aimed to examine them. Methods: This multicentre, cross-sectional, observational study enrolled patients with Behçet's disease who visited Kyoto University Hospital between 2018 and 2021 or Kurashiki Central Hospital between 2006 and 2016 (n = 200). Disease severity was evaluated using the Krause score. Results: Uveitis and gastrointestinal involvement were observed in 95/196 and 57/167 patients, respectively. The HLA alleles identified were HLA-B51 (n = 52/106), HLA-A26 (n = 25/88), and HLA-B51 and HLA-A26 (n = 6/88). In patients harbouring HLA-B51, the presence of HLA-A26 was associated with higher frequencies of uveitis (P = .03) and coexistence of uveitis and gastrointestinal involvement (P = .002) and higher Krause scores (P = .02). Furthermore, the presence of HLA-A26 was associated with a higher frequency of uveitis in patients with gastrointestinal involvement (P = .001) and gastrointestinal involvement in patients with uveitis (P = .001). Conclusions: Since specific HLA haplotypes and their combinations are associated with organ involvement, both HLA-A and HLA-B haplotypes should be confirmed when screening for affected organs. [ABSTRACT FROM AUTHOR]

Objectives: We investigated differential diagnoses that should be noted with familial Mediterranean fever (FMF) and useful variables for differentiation in a large Japanese cohort. Methods: Patients aged ≥13 years who were clinically suspected of having FMF by Livneh criteria were studied 1 year after MEFV genetic testing. Patients ultimately diagnosed with other diseases were studied, and the association among each disease, patient characteristics, and clinical variables were analysed using multiple correspondence analysis. Results: In total, 504 patients were included in this study; 34 (6.7%) were diagnosed with a disease other than FMF. The most common diagnosis was Behçet's disease, followed by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, inflammatory bowel disease, myelodysplastic syndromes (MDS), and infectious diseases. Although none of the non-FMF patients had exon 10 variants, some responded to colchicine treatment. Multiple correspondence analysis suggested that atypical symptoms such as stomatitis were associated with Behçet's disease and PFAPA syndrome, whereas characteristic situations such as disease onset ≥40 years were associated with MDS and infectious diseases. Conclusion: Careful follow-ups and reanalysis of the diagnosis should be performed for patients with atypical findings and no exon 10 variants, even if their symptoms meet the clinical criteria for FMF. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to develop clinical guidelines for the management of vascular Behçet's disease (BD) by the Behçet's Disease Research Committee of the Ministry of Health, Labour and Welfare of the Japanese Government. Methods: A task force proposed clinical questions (CQs) concerning vascular BD based on a literature search. After screening, draft recommendations were developed for each CQ and brushed up in three blinded Delphi rounds, leading to the final recommendations. Results: This study provides recommendations for 17 CQs concerning diagnosis and differential diagnoses, assessment of disease activity, and treatment. The guidelines recommend immunosuppressive treatments, for both arterial and venous involvement with active inflammation. Anticoagulation is also recommended for deep vein thrombosis except in high-risk patients. Surgical and endovascular therapies can be optional, particularly in patients with urgent arterial lesions undergoing immunosuppression. In addition, two sets of algorithms for diagnosis and treatment are shown for arterial and venous involvement. Conclusions: These recommendations are expected to serve as useful tools in the daily clinical practice of BD. This content has already been published in Japanese in the Guideline for the Management of Behçet's Disease 2020 and is submitted with permission from both the primary and secondary publishers. [ABSTRACT FROM AUTHOR]

Objectives: Behçet's disease (BD) is a multisystem disease and frequently occurs during the second–fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD. Materials and methods: This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13–17 years), adult onset (18–39 years), and late onset (>40 years). Results: The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group. Conclusion: There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic. [ABSTRACT FROM AUTHOR]

Objectives: Behçet’s disease (BD) is a systemic vasculitis affecting many organ systems, with the involvement of all-sized arteries and veins. The study aims to determine the main characteristics of paediatric BD patients and also analyse the clustering phenotypes. Methods: Demographic data, clinical manifestations, laboratory features, treatment schedules, and disease outcomes were achieved from patients’ charts retrospectively. A cluster analysis was performed according to the phenotype. Results: A total of 225 (109 male/116 female) patients with BD were enrolled in the study. The median ages of disease onset and diagnosis were 131 (36-151) and 156 (36-192) months, respectively. According to cluster analysis, 132 (58.6%) patients belonged to the mucocutaneous-only cluster (C1), while 35 (15.6%) patients fitted to articular type (C2), 25 (11.1%) were in the ocular cluster (C3), 26 (11.6%) were in the vascular cluster (C4), and 7(3.1%) belonged to the gastrointestinal cluster (C5). Ocular and vascular clusters were more common in boys (푝 < .001), while girls usually presented with the mucocutaneous-only cluster. The disease activity at the diagnosis and the last control was higher in ocular, vascular, and gastrointestinal clusters. Conclusions: These identified juvenile BD clusters express different phenotypes with different outcomes Our analysis may help clinicians to identify the disease subtypes accurately and to arrange personalized treatment. [ABSTRACT FROM AUTHOR]

Objectives: To describe the long-term clinical course of each manifestation of Behçet’s disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients. Methods: We retrospectively studied 155 BD patients visiting our hospital (1989–2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant [OU prognosis score (OuP score)]. Risk stratification was examined by dividing the OuP scores into four stages. Results: OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678. Conclusions: Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment. [ABSTRACT FROM AUTHOR]

Objectives: This retrospective cohort study aimed to find out predictors and early biomarkers of Infliximab (IFX) refractory intestinal Behçet’s syndrome (intestinal BS). Methods: We collected the baseline clinical characteristics, laboratory parameters, and concomitant therapies of intestinal BS patients treated by IFX from the Shanghai Behçet’s syndrome database. After 1 year IFX therapy, intestinal BS patients with non-mucosal healing (NMH, intestinal ulcers detected by colonoscopy) and/or no clinical remission [NCR, scores of the disease activity index for intestinal Behçet’s disease (DAIBD) ≥20] were defined as IFX refractory intestinal BS. Multivariate logistic regression analysis was performed to evaluate the predictors for NMH and NCR in IFX refractory intestinal BS. Results: In 85 intestinal BS patients, NMH was identified in 29 (34.12%) patients, and NCR was confirmed in 20 (23.53%) patients. Erythrocyte sedimentation rate (ESR; ≥24 mm/h) and free triiodothyronine (fT3; ≤3.3pmol/L) were the independent risk factors of NMH in IFX refractory intestinal BS. Drinking alcohol and the fT3/free thyroxine ratio (fT3/fT4; ≤0.24) were independent risk factors, and thalidomide was an independent protective factor, for NCR in intestinal BS patients treated by IFX. Conclusion: This study may be applicable for adjusting the therapeutic strategy and sidestepping unnecessary exposure to IFX in intestinal BS patients. Routine assessments of ESR, fT3, and fT3/fT4 ratio are helpful to identify high-risk individuals of IFX refractory intestinal BS. Thalidomide is suggested to be a concomitant therapy with IFX for intestinal BS patients. [ABSTRACT FROM AUTHOR]

Objectives: Approximately 30-60% of Behçet's disease patients exhibit joint symptoms. The aim of this study was to determine the clinical characteristics of such patients in Japan. Methods: This study retrospectively analyzed 151 Behçet's disease patients with joint symptoms who had been treated at seven cooperative medical institutions from 2007 to 2017. We investigated their clinical characteristics and treatments. Results: The most commonly affected joints were the knee, ankle, and proximal interphalangeal joints. Of the cases with pain and swelling, 18 of 293 joints (11 cases) displayed narrowing of the cleft or deformity by X-ray analysis. Improvement in their arthritis was observed in 80% of the patients who received steroids as initial treatment; however, the rate of improvement was lower in patients who had received prednisolone (PSL) at<10 mg/day. The recurrence of joint symptoms was significantly less common in the colchicine group than in the PSL group. Conclusions: These results suggest that PSL is effective for remission induction for the treatment of joint symptoms of Behçet's disease. Additionally, colchicine is effective in preventing the recurrence of joint symptoms in Behçet's disease. Furthermore, joint damages like joint space narrowing or with any deformity can often be observed in Behçet's disease. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to determine the clinical efficacy of apremilast for oral ulcers (OUs), extra-oral manifestations, and overall disease activity in patients with Behçet's disease (BD). Methods: A systematic literature search was performed in PubMed, Embase, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom-free for individual manifestations and mean difference (MD) of Behçet's Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis. Results: Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: OUs, 45.76 (95% CI, 13.23-158.31); genital ulcers, 4.56 (95% CI, 2.47-8.44); erythema nodosum, 3.59 (95% CI, 1.11-11.61); pseudofolliculitis, 2.81 95% CI, 1.29-6.15); and arthritis, 3.55 (95% CI, 1.71-7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=-1.38; -1.78 to -0.99). However, the proportion of oral-ulcer-free patients increased at 24 weeks (OR=14.88; 4.81 to 46.07). Conclusions: The currently accumulated data indicate an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study is to investigate the outcomes of coronavirus disease 2019 (COVID-19) in our cohort of Behçet's disease (BD) patients and to reveal the rate of BD exacerbations due to COVID-19. Methods: Patients who have been followed with a diagnosis of BD were retrospectively investigated for a positive severe acute respiratory syndrome-coronavirus 2 polymerized chain reaction (PCR) test. Data regarding demographics, clinical features and COVID-19 outcomes were collected from medical records for patients with a positive PCR. PCR-positive patients were reached via phone numbers, and 'Behçet's Disease Current Activity Form' (BDCAF) scores for pre- and post-COVID-19 BD symptoms were calculated. Results: Out of a total 648 BD patients, 59 were detected to have a positive PCR test. Three of the 59 patients (5.0%) were found to be hospitalized, none of them was admitted to the ICU or died. An increasing trend in the frequency of comorbid diseases and older age was observed in hospitalized patients. 32.2% of BD patients suffered from exacerbation of at least one symptom related to BD. Conclusions: We observed no ICU admission or mortality with COVID-19 in our BD patient cohort. A substantial number of patients suffered from exacerbation of BD symptoms. [ABSTRACT FROM AUTHOR]

Objectives: We investigated differential diagnoses that should be noted with familial Mediterranean fever (FMF) and useful variables for differentiation in a large Japanese cohort., Methods: Patients aged ≥13 years who were clinically suspected of having FMF by Livneh criteria were studied 1 year after MEFV genetic testing. Patients ultimately diagnosed with other diseases were studied, and the association among each disease, patient characteristics, and clinical variables were analysed using multiple correspondence analysis., Results: In total, 504 patients were included in this study; 34 (6.7%) were diagnosed with a disease other than FMF. The most common diagnosis was Behçet's disease, followed by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, inflammatory bowel disease, myelodysplastic syndromes (MDS), and infectious diseases. Although none of the non-FMF patients had exon 10 variants, some responded to colchicine treatment. Multiple correspondence analysis suggested that atypical symptoms such as stomatitis were associated with Behçet's disease and PFAPA syndrome, whereas characteristic situations such as disease onset ≥40 years were associated with MDS and infectious diseases., Conclusion: Careful follow-ups and reanalysis of the diagnosis should be performed for patients with atypical findings and no exon 10 variants, even if their symptoms meet the clinical criteria for FMF., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: To describe the clinical features and patterns of phenotype aggregation in pediatric Behçet's syndrome (BS) in a tertiary center in China. Methods: This was a cross-sectional study of consecutive BS patients in Huadong Hospital, Fudan University between September 2012 and January 2020. Pediatric BS was defined as diagnosed before 16 years old. We compared clinical variables between pediatric and adult patients. We calculate relative risks (RRs) of clinical variables according to sex. Moreover, a hierarchical cluster analysis was undertaken according to 29 clinical variables to determine homogeneous subgroups. Results: From 1596 consecutive BS cases, we identified 69 pediatric BS. Compared with adult-diagnosed BS, pediatric-diagnosed patients had a higher frequency of folliculitis [RR 1.57 (95% CI 1.12, 2.20)], a lower frequency of arthralgia [RR 0.15 (95% CI 0.02, 1.07)] and panuveitis [RR 0.43 (95% CI 0.18, 1.03)], no cardiac lesion. There was an association between male and arterial thrombosis or aneurysms (p=.006). A cluster analysis stratified three sub-clusters (C1-C3): C1 (n=30) showed a disease type merely affecting skin and mucosa. C2 (n=20) represented the gastrointestinal type; most patients presented with intestinal involvement, and two cases with esophageal ulcers. In C3 (n=19), showing a mixture of uveitis, vascular and central nervous system (CNS) involvement, six patients presented with uveitis and nine had vascular lesions, and three cases had CNS lesions. Conclusion: We conducted a comprehensive statistical analysis in a cohort of pediatric patients with BS in China. Less ocular involvement and no cardiac lesions were observed in childhood-diagnosed patients. For the first time, three distinct phenotype subgroups in pediatric-diagnosed patients were identified by cluster analysis. [ABSTRACT FROM AUTHOR]

Background: Many factors can influence the response to treatment and prognosis of Behcçet's disease (BD). Identifying the predictors of response to treatment can improve the quality and decrease the cost of medical care. This analytical study was performed to identify factors affecting the remission and outcome in BD patients with long-term follow-up. Methods: A total of 245 BD patients aged over 16 years were followed for at least 12 months and visited at least three times a year were included. The outcome was assessed by the number of patients who were in sustained and long-term remission, had lost the primary criteria of BD for at least 12 months, were asymptomatic, and developed the sequela of disease or deceased. Sustained remission was defined as being in remission for at least six months. Long-term remission was defined as remission for ≥ 5 years. Results: Mean age and mean duration of follow-up were 35.1 ± 10.7 years and 92.3 months, respectively. At the end of follow-up, 63.2% of the patients lost the criteria of BD, 51.8% of the cases were in sustained remission, and 36.2% of them were asymptomatic. Predictors of sustained remission were adherence to therapy and treatment for more than six years. Having genital ulcers and treatment with methotrexate were associated with non-remission. Predictor of long-term remission was remission induction in the first two years of the treatment. Treatment with methotrexate was associated with non-remission. Poor outcome was observed in 31.8% of patients. Male sex, obesity, and having severe disease were the risk factors of poor outcome. Conclusion: Achieving remission in BD is not inaccessible. Treatment with conventional and biologic disease-modifying antirheumatic drugs may cause sustained and long-term remission. Adherence to treatment, remission induction during the two years after the diagnosis and treatment for at least six years have significant role. [ABSTRACT FROM AUTHOR]

Objective: To determine the real-world short-term efficacy and safety of apremilast for Behçet's disease (BD). Methods: The study included patients who received apremilast for refractory oral ulcers in addition to meeting International Study Group criteria for BD or the revised International Criteria for Behçet's Disease. To assess the efficacy of apremilast, Behçet's disease current activity form (BDCAF) and patients' self-perception of their disease activity were monitored for three months. The disease phenotypes, laboratory data, concomitant medication use, and adverse events were also investigated. Results: Fourteen BD patients were included in the study. Concomitant drug use were as follows: colchicine 92.9%, prednisolone 21.4%, immunosuppressants 28.6%, and tumor-necrosis inhibitor 14.3%. Oral ulcers and BDCAF scores at 3 months showed significant improvement compared to baseline. Adverse events during the study were diarrhea (n=3, 21.4%), nausea (n=3, 21.4%), music hallucination (n=1, 7.1%), and branch retinal vein occlusion (n=1, 7.1%). Apremilast was discontinued in 1 patient (7.1%) due to nausea. Conclusion: Significant improvement in oral ulcer and BDCAF with apremilast was confirmed in realworld BD patients after 3 months. The combination of colchicine and apremilast appears to be well tolerated in BD in the short-term. [ABSTRACT FROM AUTHOR]

Objectives: In this study, we aimed to evaluate the level of central sensitization (CS) and its relationship with health profile, including neuropathic pain and sleep quality in Behcet's disease (BD). Method: Eighty-eight patients with BD and 60 healthy controls (HCs) were included in the study between May 2018 and February 2019. Nottingham health profile (NHP), pain DETECT, Pittsburgh sleep quality index (PSQI) questionnaires and the central sensitization inventory (CSI) were administered to all participants. To evaluate the correlations of the NHP, PSQI, and PDQ scores with the CSI score, the CSI was modified for each questionnaire. The activity of BD was determined by the Behcet's disease current activity form (BDCAF). Results: CSI scores were significantly higher in patients with BD than HCs (BD: 41.2 ±21.1 vs HCs: 20.4 ±16.9, p < .001). Clinical CS was detected in 69.3% of patients with BD and 28.3% of HCs (p < .001). Severe or extreme CS (CSI score ≥ 50) was present in 37.5% of patients with BD and 5.0% of HCs (p < .001). There were high correlations between the modified CSI scores and the NHP, PDQ and PSQI scores in patients with BD (R = 0.804; p < .001, Rho = 0.698; p < .001, and Rho = 0.734; p < .001, respectively). There was significant correlation between CSI and BDCAF scores (Rho= 0.470, p < .001). Conclusion: CS is more frequent and more severe in patients with BD than in HCs. There is a strong correlation between the severity of CS and poor health profile in patients with BD. [ABSTRACT FROM AUTHOR]

Background How HLA-A26 modulates Behçet's disease (BD) ocular lesions such as iridocyclitis and retinochorioiditis has not been scrutinized. Methods Ministry of Health, Labour and Welfare of Japan provided us a database of BD patients who were registered from 2003 to 2014. We selected patients who satisfied International Criteria for BD and whose data for HLA-A26 was available. Results Eligible 557 patients consisting of 238 men (42.7%) and 319 women (57.3%), whose median age was 38 years old (interquartile range 29-47) were analyzed. Prevalence of general ocular lesions, iridocyclitis, retinochorioiditis, and chronic lesions were 43.1%, 30.7%, 34.1%, and 17.4%, respectively. The prevalence of ocular lesions was higher among HLA-A26 carriers compared to that among HLA-A26 non-carriers with odds ratio (OR) of 2.5 (95% confidence interval (95% CI) 1.8-3.5, p < .001) for general ocular lesions, OR of 2.5 (95% CI 1.7-3.6, p < .001) for iridocyclitis, OR of 2.8 (95% CI 1.9-4.0, p < .001) for retinochorioiditis, and OR of 2.7 (95% CI 1.7-4.3, p < .001) for 'chronic ocular lesion following iridocyclitis or retinochorioiditis'. The HLA-A26 had a similar impact on ocular lesions between HLA-B51 positive and negative cases (Breslow-Day test, p > .05). However, the HLA-A26 had a larger impact on iridocyclitis for men compared to women (Breslow-Day test, p = .040). The male HLA-A26 carriers had higher risk of iridocyclitis with OR of 3.4 (95% CI 2.0-5.9, p < .001), while the OR for women was 1.5 (95% CI 0.9-2.6, p = .146). Conclusion HLA-A26 carriers had higher risk for iridocyclitis and retinochorioiditis. However, the impact was more prominent for men. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Behçet's disease is a chronic relapsing inflammatory disorder characterized by oral and genital ulcers, skin lesions, and uveitis. Although the involvement of the gastrointestinal, large vascular, and central nervous systems is less common, it can be fatal at times. To suppress inflammatory exacerbations and recurrences which can cause irreversible organ damage, treatment should be individualized according to the disease phenotype and severity. Apremilast, an oral phosphodiesterase 4 inhibitor, is used to treat psoriasis. The enzyme specific inhibitor increases intracellular cAMP levels, which modulate multiple signaling pathways in both innate and adaptive immunocompetent cells, leading to attenuation of inflammatory responses through the suppression of the functions of various types of immunocompetent cells including Th1 cells, Th17 cells and M1 macrophages. Current evidence shows that apremilast is effective for oral ulcer, the most common symptom of Behçet's disease. An oral ulcer is considered to represent the basic immunopathology of Behçet's disease, because the disease susceptibility genes are shared between Behçet's disease and recurrent aphthous stomatitis. The favorable effects have been documented in other inflammatory diseases with symptoms similar to those of Behçet's disease. The further studies would shed the light on the potential of apremilast for the treatment of Behçet's disease. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to develop clinical guidelines for the management of vascular Behçet's disease (BD) by the Behçet's Disease Research Committee of the Ministry of Health, Labour and Welfare of the Japanese Government., Methods: A task force proposed clinical questions (CQs) concerning vascular BD based on a literature search. After screening, draft recommendations were developed for each CQ and brushed up in three blinded Delphi rounds, leading to the final recommendations., Results: This study provides recommendations for 17 CQs concerning diagnosis and differential diagnoses, assessment of disease activity, and treatment. The guidelines recommend immunosuppressive treatments, for both arterial and venous involvement with active inflammation. Anticoagulation is also recommended for deep vein thrombosis except in high-risk patients. Surgical and endovascular therapies can be optional, particularly in patients with urgent arterial lesions undergoing immunosuppression. In addition, two sets of algorithms for diagnosis and treatment are shown for arterial and venous involvement., Conclusions: These recommendations are expected to serve as useful tools in the daily clinical practice of BD. This content has already been published in Japanese in the Guideline for the Management of Behçet's Disease 2020 and is submitted with permission from both the primary and secondary publishers., (© Japan College of Rheumatology 2023. Published by Oxford University Press.)

Objectives: Behçet's disease (BD) is a multisystem disease and frequently occurs during the second-fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD., Materials and Methods: This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13-17 years), adult onset (18-39 years), and late onset (>40 years)., Results: The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group., Conclusion: There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries. Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals. Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7-56.9), Females 44.2% (43.1-45.3), Oral Aphthosis (OA) 97.5% (97.1-97.9), genital aphthosis (GA) 64.4% (63.3-65.5), skin lesions 62.2% (61.1-63.3), ocular lesions 55.6% (54.5-56.7), Joint Manifestations 38.1% (37.0-39.2), Gastrointestinal 6.8% (6.2-7.4), Vascular 8.9% (8.3-9.5), neurological (central-peripheral) 3.9% (3.5-4.3), epididymitis 4.6% (4.1-5.1). Lab tests were positive pathergy test 50.4% (49.3-51.5), elevated ESR 51.1% (50.0-52.2), abnormal urinalysis 13.4% (12.6-14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet's Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2-77.2) and 96.6% (96.2-97.0). The specificity was 99.3% (99.1-99.5) and 97.3% (96.9-97.7). The accuracy was 86.4% (85.8-87.0) and 96.9% (96.6-97.2). Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: Behcet's disease (BD) is an inflammatory disease and smoking may have a role in its triggering. This case-control study was conducted to investigate the association between smoking and the risk of BD. Methods: We included 192 patients with BD and 822 healthy siblings of patient with BD and 373 healthy unrelated persons as control groups. Demographic data and smoking history of patients and their siblings were obtained by direct and in some cases by telephone interview with the participants. Demographic data and smoking history of healthy controls were obtained by direct interview. Propensity score matching (PSM) analyses for reducing the heterogeneity between studied groups and calculating the actual effect of smoking in BD was performed. Matching was performed based on demographic characteristics (age, gender, educational status and marital status). After PSM, we carried out multivariate analyses with BD as the main outcome variable and smoking history as the main predictor variable to calculate odds ratios with 95% confidence intervals. Results: Ever smoking was not significantly associated with an increased risk of BD compared with never smoking. In comparison with healthy siblings and healthy unrelated persons, the relative risk of developing BD was 0.8-2.6. No significant differences were observed in the clinical manifestations of BD patients in ever smokers and never smokers. However, disease activity in ever smokers at disease presentation was significantly more than never smokers. Conclusion: Smoking is not a significant risk factor for BD. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to identify patients with high-probability of ocular involvement of Behçet's disease (BD). Methods: The Japanese Ministry of Health, Labour and Welfare provided dataset of ongoing nationwide BD registration project. A patient who had confirmed BD and who was suspected to have BD was registered. We mainly analyzed newly registered patients who had the data for all demographic and diagnostic parameters regardless of fulfilment of any diagnostic criteria. Results: Among 3213 patients with confirmed or possible BD, 1382 (43.0%) were men and 1831 (57.0%) were women with a median age of 38 years (interquartile range (IQR) 30-49 years). The median duration between onset and registration was 0 year (IQR 0-3). A binomial multivariable logistic regression analysis revealed that being female (odds ratio (OR) 0.63, 95% confidence interval (CI) 0.53-0.75, p<.001), duration since onset (OR 1.33 per 10 years, 95% CI 1.18-1.51, p<.001), genital ulceration (OR 0.28, 95% CI 0.23-0.34, p<.001), and gastrointestinal symptoms (OR 0.36, 95% CI 0.30-0.44, p<.001) were related to the ocular lesion. Analyses based on data of 2800 patients who satisfied International criteria of BD, age-, sex-, duration-based subgroup analyses, analyses targeting iridocyclitis and retino-uveitis, and analysis including patients with missing data confirmed that the four factors were associated with the probability of eye involvement. Conclusion: The ocular involvement did not accompany with genital ulcer or gastrointestinal symptoms at the early stage of BD. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of the present study was to describe our experience with the recombinant Fab' antibody fragment against TNF-a Certolizumab Pegol (CZP) in patients with Behc,et's disease (BD) refractory to standardized therapies and previous biologic agents. Methods: Retrieved data including demographic characteristics, clinical manifestations, and previous treatments were collected in three different specialized Rheumatologic Units in Italy. In order to evaluate disease activity, the BD current activity form (BDCAF) has been used before starting CZP therapy and at each visit during treatment. Results: Thirteen BD patients (mean age 42.6 ± 8.8 years) with a disease duration of 8.80 ± 6.9 years, underwent CZP treatment for 6.92 ± 3.52 months. Six patients (46.15%) experienced a worsening of symptoms after 4.16 ± 1.21 months, whereas a satisfactory response was achieved in seven patients (53.84%) who were still on CZP therapy at the last follow-up visit (after 9.28 ± 3.03 months of treatment). The mean decrease of BDCAF between the first and last visit was 0.308 ± 1.84 without reaching significant difference (mean 8.3 ± 1.3 and 8 ± 2.08, respectively; p= .51). During the whole study period, CZP was well tolerated in all patients except one who developed a generalized cutaneous reaction after the third administration. Conclusions: These results suggest that despite an improvement of clinical manifestations has been observed in more than half of the patients, it is not possible to draw firm conclusions about the effectiveness of CZP in BD and further studies with larger cohorts of patients are warranted. Whether the increase of CZP dosage may ensure a better clinical response remains an unsolved issue that needs to be considered. [ABSTRACT FROM AUTHOR]

Objectives: To describe the long-term clinical course of each manifestation of Behçet's disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients., Methods: We retrospectively studied 155 BD patients visiting our hospital (1989-2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant [OU prognosis score (OuP score)]. Risk stratification was examined by dividing the OuP scores into four stages., Results: OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678., Conclusions: Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives. To identify the specific characteristics of patients with refractory intestinal Behçet's disease (BD) who required more than glucocorticoid (GC) and/or 5-aminosalicylic acid (5-ASA) treatment. Methods. A retrospective review of the patient records in a university hospital identified 34 patients with intestinal BD. The patients treated only with glucocorticoid and/or 5-ASA (n = 8) were compared with refractory cases which required additional immunosuppressants, anti-TNFα antibodies, or surgery (n = 12). Results. In the refractory group, ulcers were found outside the ileocecal region more often, and more active intestinal bleeding or melena was observed, than in the GC/5ASA-controlled group (75% vs 0%, p = 0.001), (58% vs 0%, p = 0.015). The refractory group also showed higher positivity for HLA-B51 (45% vs 0%, p = 0.044), higher blood C-reactive protein (CRP) levels (14.7 ± 8.74 vs 3.93 ± 6.33 mg/dL, p = 0.046), and a higher white blood cell or WBC count (14750 ± 6760 vs 7210 ± 1830/ml, p = 0.025) at onset. The existence of either HLA-B51, melena, or elevated CRP of more than 4 mg/dL predicted the refractory form of BD with 100% sensitivity and 85.7% specificity. Conclusions. Refractory intestinal BD was distinguished from the non-refractory form by distinct clinical and laboratory findings. These findings will be useful in identifying patients who require intensive therapy (e.g., anti-TNFα antibodies) in addition to GC/5ASA. [ABSTRACT FROM AUTHOR]

Behçet's disease is a multisystem chronic autoimmune disease of unknown etiology with a wide spectrum of symptoms and organ system involvement. Arterial manifestations, particularly of the extracranial vertebral arteries, are rare. We report an unusual case of an African American patient with Behçet's disease, who presented with bilateral spontaneous vertebral artery pseudoaneurysms. The patient underwent successful endovascular parent vessel occlusion with detachable coils and on follow-up had complete clinical and angiographic signs of recovery. [ABSTRACT FROM AUTHOR]

Pulmonary vascular involvement in Behçet's disease is a rare complication with a poor prognosis. We present an autopsy case of vasculo-Behçet's disease complicated by pulmonary hemorrhage, possibly caused by rupture of pulmonary artery aneurysms. The patient was treated with a combination of high-dose steroids and pulse cyclophosphamide, but he died from massive hemoptysis. This case highlights the need for potent new therapies for patients with vasculo-Behçet's disease refractory to conventional immunosuppressive therapy, such as a combination of steroids and cyclophosphamide. [ABSTRACT FROM AUTHOR]

Objectives. To investigate the influences of various factors on the relapse of acute neurological attacks in patients with Behçet's disease (BD). Methods. Sixty-one patients, who met the international classification criteria for BD, had attacks of acute neuro-Behçet's disease (NBD) and could be followed up at least for 2 months, including 60 patients in our multicenter retrospective survey on BD patients in 2011. The factors associated with relapse of acute neurological attacks were assessed. Results. Twenty-one of 61 patients had been taking cyclosporine A (CyA) at the onset of acute NBD. All the 21 patients with CyA and 33 of the 40 patients without CyA had eye involvement. There were no significant differences in demographic features, clinical symptoms, MRI findings, the need for, and responses to corticosteroid therapy including pulse therapy between patients with CyA and those without CyA. CyA was withdrawn in 19 of 21 patients with CyA. Of note, patients with CyA showed significantly lower relapse rates than those without CyA (HR 0.1283, 95% CI: 0.0788-0.7836, p = 0.0186 as calculated by log-rank test). Moreover, colchicine was found to reduce the relapse rates in patients with acute NBD without CyA (HR 0.2771, 95% CI: 0.0827-0.9422, p = 0.0450 [log-rank test]). Conclusion. These results indicate that CyA-related acute neurological manifestations are almost identical with CyA-unrelated acute events of NBD, except for the paucity of relapse on withdrawal of CyA. The data also demonstrate that colchicine is effective to prevent relapses of acute neurological attacks especially in patients with CyA-unrelated acute NBD. [ABSTRACT FROM AUTHOR]

Objective. Behçet's disease (BD) is a systemic inflammatory disease with unknown etiology. Studies have shown that some T helper (Th) 1-associated cytokines have role in the inflammation of BD. The CD4+ Th cells can be differentiated into Th1, Th2, Th17 and Th22 secrete different cytokines to regulate immune system. In this study, cytokine secretion of Th subsets in BD was investigated. Methods. The study group consisted of 26 BD patients with mucocutaneous involvement and 12 healthy subjects. Lymphocyte subpopulations, IL-5, IL-10, IL-17, IL-22 and IFN-γ secretion of CD4+ T and Foxp3+ Treg cells were determined by flow cytometry. Results. Compared with healthy subjects, Th1 (IL-17A−IL-22−IFN-γ+), Th22 (IL-17A−IL-22+IFN-γ) and IL-17A+IFN-γ+-secreting cells were significantly increased, and the percentage of Treg cells were dramatically reduced in BD patients. The frequency of recurrent oral ulcers was associated with increased Th22 cells. Conclusions. Our study describes an association between Th22 cell subset and IL-17A+ IFNγ+-secreting cells with mucocutaneous BD. These findings revealed that reduced levels of Tregs and increased levels of Th1 and Th22 cells as well as Th17/Th1 cells might be associated with the pathogenesis of BD. [ABSTRACT FROM AUTHOR]

Objectives. To examine the association between the Intercellular adhesion molecule-1 (ICAM1) Polymorphisms and Behçet's disease. Methods. MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials for original studies up to July 31, 2012 were searched for relevant studies. All pooled odds ratios (ORs) were derived from either fixed or random-effects model with its 95% confidence intervals (CI). Results. Five studies met the inclusion criteria. Overall, ICAM1 E469 (OR = 1.45, 95% CI = 1.06-1.97), genotype ICAM1 469 E/E (OR = 1.45, 95% CI = 1.09-1.94), ICAM1 241 G/R (OR = 3.65, 95% CI = 1.69-7.89), had significant associations with Behçet's disease. A significant association was found between the presence of skin lesions and genotype ICAM1 469 E/E (OR = 3.52, 95% CI = 1.62-7.66). Conclusions. Behçet's disease was associated with the ICAM1 E469, genotype ICAM1 469 E/E, ICAM1 241 G/R polymorphisms in different ethnic groups. Among patients, genotype ICAM1 469 E/E had a significant association with skin lesion. [ABSTRACT FROM AUTHOR]

Behçet's disease is a multisystemic, chronic inflammatory disorder with diffuse clinical manifestations including the cardiovascular system. Endomyocardial fibrosis is a rarely seen complication of Behçet's disease leading to progressive heart failure. We report a case of right ventricular endomyocardial fibrosis mimicking Ebstein anomaly in a 26-year-old male Turkish patient with Behçet's disease, who had heart failure symptoms. In addition, the previously reported cases of endomyocardial fibrosis complicating Behçet's disease are reviewed in this article. [ABSTRACT FROM AUTHOR]

Objectives. Polymorphisms in the Interleukin (IL)-1-related genes at the locations -889, -511, + 3962 and mspa1l 1100 have been investigated for possible association with Behçet's disease (BD). Methods. A literature-based search was conducted to identify all relevant studies. Five independent studies from Turkish population met the included criteria. Results. IL-1α -889 CT [odds ratio (OR) = 0.72, 95% confidence interval (CI) = 0.55-0.95], IL-1α -889 TT (OR = 0.61, 95% CI = 0.40-0.93), IL-1β + 3962 C (OR = 1.41, 95% CI = 1.07-1.88), IL-1β + 3962 T (OR = 0.71, 95% CI = 0.53-0.94) IL-1β + 3962 CC (OR = 2.08, 95% CI = 1.08-3.99), IL-1β + 3962 CT (OR = 0.58, 95% CI = 0.38-0.88), IL-1 receptor antagonist (IL-1 Ra) mspa1l 1100 CT (OR = 0.69, 95% CI = 0.49-0.96), IL-1 Ra mspa1l 1100 TT (OR = 1.50, 95% CI = 1.08-2.08) had a significant association with BD. The pooled estimates for IL-1α -889 C, IL-1α -889 CC, IL-1α -889 T had a non-significant association with BD. Conclusions. IL-1α -889 CT, IL-1α -889 TT, IL-1β + 3962 C, IL-1β + 3962 T, IL-1β + 3962 CC, IL-1β + 3962 CT, IL-1 Ra mspa1l 1100 CT, IL-1Ra mspa1l 1100 TT promoter polymorphisms may confer susceptibility to BD in Turkish population. [ABSTRACT FROM AUTHOR]

Aim. The aim of our study was to determine the prevalence of anti-C1q antibodies and their possible association with clinical presentation in Behcet's disease (BD) patients with special emphasis for patients with vascular involvement. Methods. Plasma anti-C1q Abs levels were measured using an enzyme-linked immunosorbent assay in 51 BD patients and 25 age- and gender-matched healthy controls. Results. We found elevated concentrations of anti-C1q more frequently in patients with BD (18 %) than in healthy controls (8 %). The highest prevalence was found in patients with vascular BD (42 %) which was significantly higher than patients without vascular BD and healthy controls ( p = 0.025). Furthermore, patients with vascular BD had the highest mean anti-C1q levels when compared to BD patients without vascular involvement or healthy control subjects ( p = 0.015). We did not find significant differences in the prevalence of any other organ involvement between BD patients with elevated vs. normal anti-C1q ab levels. Anti-C1q ab levels positively correlated with ESR ( r = 0.383, p = 0.006) and negatively with C4 ( r = −0.304, p = 0 .030). Conclusion. In conclusion, we found an increased prevalence of anti-C1q autoantibodies in BD patients with vascular involvement. Further large scale longitudinal studies are required to assess and clarify the significance and the pathogenic role of anti-C1q antibodies in BD and other autoimmune diseases in which vasculitis is a component. [ABSTRACT FROM AUTHOR]

Arthritis associated with Behçet's disease is typically nonerosive and nondeforming, and most patients respond to colchicine treatment. However, destructive arthritis and refractory arthritis have also been reported on occasion. Elevated white blood cell counts may occur in synovial fluid in BD, but pseudoseptic arthritis is a very rare event in this disease. We report a patient with BD and rapidly progressive deforming pseudoseptic arthritis refractory to colchicine, corticosteroids, and methotrexate, who entered remission after infliximab treatment. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to determine the clinical efficacy of apremilast for oral ulcers (OUs), extra-oral manifestations, and overall disease activity in patients with Behçet's disease (BD)., Methods: A systematic literature search was performed in PubMed, Embase, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom-free for individual manifestations and mean difference (MD) of Behçet's Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis., Results: Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: OUs, 45.76 (95% CI, 13.23-158.31); genital ulcers, 4.56 (95% CI, 2.47-8.44); erythema nodosum, 3.59 (95% CI, 1.11-11.61); pseudofolliculitis, 2.81 (95% CI, 1.29-6.15); and arthritis, 3.55 (95% CI, 1.71-7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=-1.38; -1.78 to -0.99). However, the proportion of oral-ulcer-free patients increased at 24 weeks (OR = 14.88; 4.81 to 46.07)., Conclusions: The currently accumulated data indicate an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

We report a case of a 73-year-old Japanese male patient who developed Behçet’s disease (BD) after poststreptococcal acute glomerulonephritis. Three months after the initial presentation, acneiform eruption and oral and genital ulcers appeared. Treatment with oral prednisolone (20 mg/day) resulted in the remarkable disappearance of these symptoms. These findings support the hypothesis that Streptococcus pyogenes may be an etiologic factor of BD. [ABSTRACT FROM AUTHOR]

Here, we present two Behçet's disease (BD) patients with an uncommon complication: inflammation surrounding the spinous processes revealed by positron emission tomography/computed tomography (PET/CT). Both patients were treated successfully with 20 mg of prednisolone daily. Whether BD should be considered a form of spondylarthritis remains an open question. However, inflammation around the spinous processes should be regarded as a possible uncommon finding of seronegative spondyloarthropathy (SpA) in BD patients. [ABSTRACT FROM AUTHOR]

Objective: To compare Behçet's syndrome (BS) cohorts from the US and Japan in terms of rates of concordance with the International Study Group (ISG) criteria and Japanese criteria, disease manifestations, and treatment. Methods: All BS patients seen at the NYU Hospital for Joint Diseases in the US and the Kameda Medical Center and St. Luke's International Hospital in Japan between 2003 and 2010 were included. Diagnosis of BS was made on the basis of clinical manifestations and the clinical decisions of experienced specialists familiar with BS. We classified the patients into complete and incomplete types based on their symptoms; both complete or incomplete types were assumed to fulfil the Japanese criteria. Results: A total of 769 patients (US n = 634, Japan n = 135) were reviewed. 61.5 % in the US and 63.7 % in Japan fulfilled the ISG criteria. Similarly, there was no difference in the proportions of US and Japanese patients who fulfilled the Japanese criteria. Japanese patients were less likely to be female and to have genital ulcers, but were more likely to have epididymitis and pulmonary disease. Significantly more patients were treated with colchicine, sulfasalazine/mesalazine, and NSAIDs in Japan, while significantly more patients in the US received first-line immunosuppressants. Conclusions: The concordance rates for ISG and Japanese criteria fulfillment in the US and Japan were not significantly different. These findings could help to clarify regional differences in the diagnostic and clinical features of BS. [ABSTRACT FROM AUTHOR]