title: Birmingham Behçet's service: classification of disease and application of the 2014 International Criteria for Behçet's Disease (ICBD) to a UK cohort.
abstract: This study reports on the analysis of the application and diagnostic predictability of the revised 2014 ICBD criteria in an unselected cohort of UK patients, and the ensuing organ associations and patterns of disease.
  
  
  
  A retrospective cohort study was conducted using a database of electronic medical records. Three categories were recognised: clinically defined BD, incomplete BD and rejected diagnoses of BD. We applied the ISG 1990 and ICBD 2014 classification criteria to these subgroups to validate diagnostic accuracy against the multidisciplinary assessment.
  
  
  
  Between 2012 and 2015, 281 patients underwent initial assessment at an urban tertiary care centre: 190 patients with a confirmed diagnosis of BD, 7 with an incomplete diagnosis, and 84 with a rejected diagnosis. ICBD 2014 demonstrated an estimated sensitivity of 97.89% (95% CI: 94.70 to 99.42) and positive likelihood ratio of 1.21 (1.10 to 1.28). The strongest independent predictors were: Central nervous lesions (OR = 10.57, 95% CI: 1.34 to 83.30); Genital ulceration (OR = 9.05, 95% CI: 3.35 to 24.47); Erythema nodosum (OR = 6.59, 95% CI: 2.35 to 18.51); Retinal vasculitis (OR = 6.25, 95% CI: 1.47 to 26.60); Anterior uveitis (OR = 6.16, 95% CI: 2.37 to 16.02); Posterior uveitis (OR = 4.82, 95% CI: 1.25 to 18.59).
  
  
  
  The ICBD 2014 criteria were more sensitive at picking up cases than ISG 1990 using the multidisciplinary assessment as the gold standard. ICBD may over-diagnose BD in a UK population. Patients who have an incomplete form of BD represent a distinct group that should not be given an early diagnostic label. Behçet's disease is a complex disease that is best diagnosed by multidisciplinary clinical assessment. Patients in the UK differ in their clinical presentation and genetic susceptibility from the original descriptions. This study also highlights an incomplete group of Behçet's patients that are less well defined by their clinical presentation.
authors.affiliation: Rheumatology Department, Birmingham City Hospital, Sandwell and West Birmingham Hospitals NHS Trust, Dudley Rd, Birmingham, B18 7QH, UK. timblakewarwick@gmail.com.
authors.forename: Tim
authors.initials: T
authors.lastname: Blake
authors.affiliation: Institute of Inflammation and Ageing, University of Birmingham College of Medical and Dental Sciences, Birmingham, UK.
authors.forename: Luke
authors.initials: L
authors.lastname: Pickup
authors.affiliation: Rheumatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: David
authors.initials: D
authors.lastname: Carruthers
authors.affiliation: Ophthalmology, Birmingham and Midland Eye Centre, Birmingham, UK.
authors.forename: Erika Marie
authors.initials: EM
authors.lastname: Damato
authors.affiliation: Institute of Inflammation and Ageing, University of Birmingham College of Medical and Dental Sciences, Birmingham, UK.|Ophthalmology, Birmingham and Midland Eye Centre, Birmingham, UK.
authors.forename: Alastair
authors.initials: A
authors.lastname: Denniston
authors.affiliation: Oral Medicine, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: John
authors.initials: J
authors.lastname: Hamburger
authors.affiliation: Rheumatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: Claire
authors.initials: C
authors.lastname: Maxton
authors.affiliation: Rheumatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: Debbie
authors.initials: D
authors.lastname: Mitton
authors.affiliation: Institute of Inflammation and Ageing, University of Birmingham College of Medical and Dental Sciences, Birmingham, UK.|Ophthalmology, Birmingham and Midland Eye Centre, Birmingham, UK.
authors.forename: Philip I
authors.initials: PI
authors.lastname: Murray
authors.affiliation: Wolfson Computer Laboratory, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK.
authors.forename: Peter
authors.initials: P
authors.lastname: Nightingale
authors.affiliation: School of Dentistry, University of Birmingham, Birmingham, UK.
authors.forename: Ana
authors.initials: A
authors.lastname: Poveda-Gallego
authors.affiliation: Oral Medicine, Birmingham Community Healthcare NHS Trust, Birmingham, UK.
authors.forename: Andrea
authors.initials: A
authors.lastname: Richards
authors.affiliation: Rheumatology, Dudley Group of Hospitals NHS Foundation Trust, Birmingham, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Whallett
authors.affiliation: Rheumatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: Deva
authors.initials: D
authors.lastname: Situnayake
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001528:Behcet Syndrome / Q000145:classification* / Q000175:diagnosis*; D016208:Databases, Factual; D057286:Electronic Health Records; D005260:Female; D006801:Humans; D033183:Interdisciplinary Communication; D008297:Male; D008403:Mass Screening / Q000379:methods*; D008875:Middle Aged; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D062606:Tertiary Care Centers; D006113:United Kingdom; D055815:Young Adult
keywords: Auto-inflammatory disorders; Behçet’s disease; Epidemiology; History of medicine; Inflammation