title: Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet's disease: a retrospective observational study.
abstract: It has been suggested that the phenotypes of Behçet's disease (BD) in Japan are changing. To ask whether the evolution of BD holds true in recent-onset cases in Japan, we performed a retrospective study.
  
  
  
  We reviewed the records of 578 patients with BD who met the 1987 revised diagnostic criteria of the Behçet's disease research committee of Japan. The patients were divided into three groups based on the date of disease onset. We compared the demography, clinical features, and treatments among them with or without adjustment for the observation period. Patients having oral ulcers, genital ulcers, regional skin involvement, and uveitis are categorized as having complete-type BD, and the associated factors were determined by univariate and multivariate logistic regression analyses.
  
  
  
  Male patients had a higher propensity for uveitis and central nervous system (CNS) involvement, whereas female patients had higher rates of genital ulcers and arthritis. We found a significant trend in reduction of complete-type, genital ulcer, HLA-B51 carriers, and increment of gastrointestinal BD over time. Multiple regression analysis identified HLA-B51 positivity, earlier date of disease onset, and younger age of onset as independently associated with complete-type BD. Although treatments had been also chronologically changed, the causative relationship between therapeutic agents and phenotypical changes was not determined from the study.
  
  
  
  The present study revealed that phenotypical evolution was characterized by decreased incidence of the complete type and increment of gastrointestinal involvement in Japanese patients with BD during the last 30 years.
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan. Kirino@yokohama-cu.ac.jp.|Y-CURD study group, Yokohama, Japan. Kirino@yokohama-cu.ac.jp.
authors.forename: Yohei
authors.identifier: 0000-0002-9488-661X
authors.initials: Y
authors.lastname: Kirino
authors.affiliation: Department of Rheumatology, Yokohama Minami Kyosai Hospital, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Haruko
authors.initials: H
authors.lastname: Ideguchi
authors.affiliation: Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Takeno
authors.affiliation: Yokosuka Center for Rheumatic Diseases, Yokosuka City Hospital, Yokosuka, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Suda
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.
authors.forename: Kana
authors.initials: K
authors.lastname: Higashitani
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Kunishita
authors.affiliation: Center for Rheumatic Diseases, Yokohama City University Medical Center, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Kaoru
authors.initials: K
authors.lastname: Takase-Minegishi
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Maasa
authors.initials: M
authors.lastname: Tamura
authors.affiliation: Department of Rheumatology, National Hospital Organization Yokohama Medical Center, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Asami
authors.affiliation: Department of Rheumatology, Chigasaki City Hospital, Chigasaki, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Takeaki
authors.initials: T
authors.lastname: Uehara
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Ryusuke
authors.initials: R
authors.lastname: Yoshimi
authors.affiliation: Department of Rheumatology, Yamato City Hospital, Yamato, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Tetsu
authors.initials: T
authors.lastname: Yamazaki
authors.affiliation: Department of Hematology and Rheumatology, Fujisawa City Hospital, Fujisawa, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Sekiguchi
authors.affiliation: Department of Rheumatology, National Hospital Organization Yokohama Medical Center, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Ihata
authors.affiliation: Center for Rheumatic Diseases, Yokohama City University Medical Center, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Shigeru
authors.initials: S
authors.lastname: Ohno
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Atsuhisa
authors.initials: A
authors.lastname: Ueda
authors.affiliation: Department of Rheumatology, Yamato City Hospital, Yamato, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Toshihisa
authors.initials: T
authors.lastname: Igarashi
authors.affiliation: Department of Rheumatology, Yokohama Minami Kyosai Hospital, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Shohei
authors.initials: S
authors.lastname: Nagaoka
authors.affiliation: Yokohama City University, Yokohama, Japan.|Y-CURD study group, Yokohama, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ishigatsubo
authors.affiliation: Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Nakajima
mesh_terms: D000328:Adult; D044466:Asian People; D001528:Behcet Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies
keywords: Behçet’s disease; Complete type; Evolution; HLA-B51