Clinical and Genetic Aspects of Behçet's Disease in Japan

Kirino, Yohei; Nakajima, Hitoshi

doi:10.2169/internalmedicine.2035-18

Cited by 33 publications

(28 citation statements)

References 70 publications

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“…As shown in Tables 2 and 3 , HLA-B51 positivity was significantly less prevalent in cluster 2 (YCU) and cluster A (MHLW) than in other clusters, suggesting that the GI cluster is associated with a distinct genetic background [ 50 ]. Identification of the diverse genetic characteristics of the intestinal variant will facilitate its management as a separate disease entity [ 51 ]. Moreover, it was possible that autoinflammatory disease (AID) associated with single gene mutation had been diagnosed as BD.…”

Section: Discussionmentioning

confidence: 99%

Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet’s disease in Japan

et al. 2021

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Background We hypothesized that Behçet’s disease (BD) consists of several clinical subtypes with different severity, resulting in heterogeneity of the disease. Here, we conducted a study to identify clinical clusters of BD. Methods A total of 657 patients registered in the Yokohama City University (YCU) regional BD registry between 1990 and 2018, as well as 6754 patients who were initially registered in the Japanese Ministry of Health, Labour and Welfare (MHLW) database between 2003 and 2014, were investigated. The YCU registry data regarding the clinical manifestations of BD, human leukocyte antigen (HLA) status, treatments, and hospitalizations were analyzed first, followed by similar analyses of the MHLW for validation. A hierarchical cluster analysis was independently performed in both patient groups. Results A hierarchical cluster analysis determined five independent clinical clusters in the YCU cohort. Individual counterparts of the YCU clusters were confirmed in the MHLW registry. Recent phenotypical evolutions of BD in Japan, such as increased gastrointestinal (GI) involvement, reduced complete type according to the Japan Criteria, and reduced HLA-B51 positivity were associated with chronologically changing proportions of the clinical clusters. Conclusions In this study, we identified independent clinical clusters among BD patients in Japan and found that the proportion of each cluster varied over time. We propose five independent clusters namely “mucocutaneous”, “mucocutaneous with arthritis”, “neuro”, “GI”, and “eye.”

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Section: Discussionmentioning

confidence: 99%

Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet’s disease in Japan

et al. 2021

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“…Thus, HLA-B51 may not always be useful in the diagnosis of BD. 17 In several studies, it was reported that men with HLA-B51 were more at risk for BD and it was associated with early onset age. 18 However, in our study, no correlation was found between HLA-B51 positivity and age at gender and onset.…”

Section: Resultsmentioning

confidence: 99%

Frequency of HLA-B51 in Behçet's Patients and Relationship with Clinical Findings

Akyürek¹,

Koçak²

2019

Turkiye Klinikleri J Dermatol

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The most important genetic factors known in the pathogenesis of Behçet's disease (BD) is HLA-B51. It has been shown that HLA-B51 positivity is high in countries on the 'Silk Road' where BD is frequent. HLA-B51 is thought to be a prognostic marker that is related to severe clinical forms of BD. In this study, we aimed to investigate the prevalence of HLA-B51 positivity in patients diagnosed with BD and the relationship between the demographic and clinical findings of the patients. M Ma at te er ri ia al l a an nd d M Me et th ho od ds s: : Between March 2013 and 2018, the files of the patients who were diagnosed with BD according to the criteria of the International Study Group for Behçet Disease in dermatology polyclinic of Selçuk University Medical Faculty were reviewed retrospectively. Forty-seven patients (22 women and 25 men) with HLA-B51 were included in the study. R Re es su ul lt ts s: : In our study, no correlation was found between HLA-B51 positivity and gender, age at onset, family history, clinical findings (such as oral and genital ulcer, papulopustular eruption, erythema nodosum, pathergy test positivity, thrombophlebitis, ocular involvement, arthritis) and systemic manifestations. C Co on nc cl lu us si io on n: : In previous studies, conflicting results have been reported on the relationship between HLA-B51 and BD manifestations in different ethnic groups. In our study, no statistically significant correlation was found between HLA-B51 positivity and demographic and clinical findings of Behçet patients. We believe that there is a need for larger comparative prospective studies investigating the relationship between HLA-B51 and BD in different ethnic groups. K Ke ey yw wo or rd ds s: : Behçet's disease; HLA-B51 Ö ÖZ ZE ET T A Am ma aç ç: : Behçet hastalığının (BH) patogenezindeki bilinen genetik faktörlerin en önemlisi HLA-B51'dir. Behçet hastalığının sık görüldüğü "İpek yolu" üzerindeki ülkelerde HLA-B51 pozitifliğinin yüksek olduğu gösterilmiştir. HLAB-51'in, BH'nin şiddetli klinik formları ile ilişkili prognostik bir marker olabileceği düşünülmüştür. Bu çalışmada, BH tanısı ile takip edilen hastalarda, HLA-B51 pozitifliğinin sıklığı ve hastaların demografik ve klinik bulguları ile ilişkisini araştırmayı amaçladık. G Ge er re eç ç v ve e Y Yö ön nt te em ml le er r: : Mart 2013-2018 tarihleri arasında Selçuk Üniversitesi Tıp Fakültesi dermatoloji polikliniğinde takip edilen, Uluslararası Behçet Hastalığı Çalışma Grubu kriterlerinine göre BH tanısı olan hastaların dosyaları retrospektif olarak incelendi. HLA-B51 bakılan 47 hasta (22 kadın ve 25 erkek) çalışmaya dahil edildi. B Bu ul lg gu ul la ar r: : Çalışmamızda HLA-B51 pozitifliği ile cinsiyet, başlangıç yaşı, aile öyküsü, klinik bulgular (oral ve genital ülser, papülopüstüler erüpsiyon, eritema nodozum, paterji testi pozitifliği, tromboflebit, oküler tutulum, artrit gibi) ve sistemik bulgular arasında bir ilişki bulunmadı. S So on nu uç ç: : Daha önceki çalışmalarda, farklı etnik gruplarda, HLA-B51'in BH'nin klinik bulguları arasındaki ilişkiy...

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“…There are at least 17 diagnostic/classification criteria for BD which have various diagnostic sensitivities and specificities 7. His presentations met some of these criteria including Japanese one, and not others including International Study Group diagnostic criteria unless we regard the heel lesion as consistent with BD 8. It is still important to accumulate atypical dermatological findings of BD for further better diagnostic procedure, even though many decades have passed since the name was established.…”

Section: Descriptionmentioning

confidence: 99%