title: Behçet disease: evolution of clinical manifestations.
abstract: Clinical phenotypes of Behçet disease (BD) vary among ethnic groups. We chronologically analyzed the clinical manifestations of BD in 412 patients meeting the Japanese criteria for BD seen at 2 Yokohama City University hospitals from July 1991 to December 2007. We examined the onset of individual symptoms in each patient. A single initial symptom appeared earlier than any other manifestation in 78% of the patients. Time from the initial symptom to diagnosis was 8.6 ± 10.1 years. Oral ulcer, the most common initial manifestation, preceded the diagnosis by 7.5 ± 10.2 years. Genital ulcer and eye and skin involvement appeared 1 or 2 years before diagnosis, whereas gastrointestinal, central nervous system, or vascular involvement developed later. The frequency of eye involvement was significantly higher in patients with neurologic lesions, but significantly lower in those with gastrointestinal or vascular involvement. However, no particular combination of major symptoms predicted the development of organ involvement. There has been a recent decrease in the rate of "complete" BD (patients having all 4 of the major symptoms of oral ulcers, genital ulcers, and eye and skin lesions), whereas the frequencies of arthritis, gastrointestinal, and vascular involvement have been increasing. Further assessment may allow the detection of early predictors of the more aggressive disease, which requires more intensive treatment.
authors.affiliation: From Center for Rheumatic Diseases (HI, SO), Yokohama City University Medical Center, Yokohama; Department of Internal Medicine and Clinical Immunology (AS, MT, AU, YI), Yokohama City University Graduate School of Medicine, Yokohama; and Department of Rheumatology (AS), Yokohama Minami Kyosai Hospital, Yokohama, Japan.
authors.forename: Haruko
authors.initials: H
authors.lastname: Ideguchi
authors.forename: Akiko
authors.initials: A
authors.lastname: Suda
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Takeno
authors.forename: Atsuhisa
authors.initials: A
authors.lastname: Ueda
authors.forename: Shigeru
authors.initials: S
authors.lastname: Ohno
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ishigatsubo
mesh_terms: D000328:Adult; D000367:Age Factors; D001528:Behcet Syndrome / Q000208:ethnology* / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D015235:HLA-B Antigens / Q000097:blood; D059949:HLA-B51 Antigen; D015395:Histocompatibility Antigens Class I / Q000097:blood*; D006785:Hospitals, University; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D015995:Prevalence; D012737:Sex Factors; D013997:Time Factors
chemical_list: D015235:HLA-B Antigens; D059949:HLA-B51 Antigen; D015395:Histocompatibility Antigens Class I