title: Behçet syndrome manifestations and activity in the United States versus Turkey -- a cross-sectional cohort comparison.
abstract: OBJECTIVE
  To compare clinical manifestations and activity of Behçet syndrome (BS) in the United States versus Turkey using validated outcome measures.
  
  
  METHODS
  Consecutive patients with BS from the US National Institutes of Health (NIH), New York University, and the University of Istanbul were evaluated. Disease activity was measured using the Behçet's Syndrome Activity Scale (BSAS) and the Behçet's Disease Current Activity Form (BDCAF) with quality of life measured by the Behçet Disease Quality of Life (BDQOL) form. One-way ANOVA, t-tests, and multivariate regression analyses were performed.
  
  
  RESULTS
  Mean age did not differ between sites; however, more women were seen in the United States versus in Turkey (p < 0.001), and disease duration was longer in the United States (p = 0.02). Organ manifestations were similar for oral and genital ulcers, skin disease, arthralgia, eye disease, and thrombosis. However, more gastrointestinal (p < 0.001) and neurologic disease (p = 0.003) was seen in the United States. BSAS and BDCAF scores were worse in the United States compared to Turkey (p = 0.013 and < 0.001, respectively). Worse mean BDQOL scores were observed at the NIH compared to Istanbul (not significant). Multivariable regression models showed worse scores in ethnically atypical patients for BSAS and BDCAF (p = 0.04 and p = 0.001), American patients for BDCAF (p = 0.01), older age for BDCAF (p = 0.005), and women for BDQOL (p = 0.01).
  
  
  CONCLUSIONS
  Demographic and clinical manifestations of BS differ between sites with higher disease activity in the United States compared to Turkey. Referral patterns, age, sex, ethnicity, and country of origin may be important in these differences. These observations raise the question of whether pathogenic mechanisms differ in Turkish and American patients.
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa. sibleych@mail.nih.gov.
authors.forename: Cailin
authors.initials: C
authors.lastname: Sibley
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Yazici
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Koray
authors.initials: K
authors.lastname: Tascilar
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Nafiz
authors.initials: N
authors.lastname: Khan
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Yasmin
authors.initials: Y
authors.lastname: Bata
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Hasan
authors.initials: H
authors.lastname: Yazici
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Raphaela
authors.initials: R
authors.lastname: Goldbach-Mansky
authors.affiliation: From the Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health, Bethesda, Maryland; Department of Rheumatology, Oregon Health and Science University, Portland, Oregon; Department of Rheumatology, New York University, New York, New York, USA; Department of Rheumatology, University of Istanbul Cerrahpasa, Istanbul, Turkey.C. Sibley, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; and Department of Rheumatology, Oregon Health and Science University; Y. Yazici, MD, Department of Rheumatology, New York University; K. Tascilar, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; N. Khan, BS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; Y. Bata, BA, Department of Rheumatology, New York University; H. Yazici, MD, Department of Rheumatology, University of Istanbul Cerrahpasa; R. Goldbach-Mansky, MD, MHS, Translational Autoinflammatory Disease Section, NIAMS at the NIH; G. Hatemi, MD, Department of Rheumatology, University of Istanbul Cerrahpasa.
authors.forename: Gulen
authors.initials: G
authors.lastname: Hatemi
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000175:diagnosis*; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012720:Severity of Illness Index; D014421:Turkey; D014481:United States; D055815:Young Adult
keywords: BEHÇET SYNDROME; DISEASE MANIFESTATIONS; TURKEY; UNITED STATES