Abstract
BackgroundThis 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.MethodsA 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.ResultsBetween 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).ConclusionsThe 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.
Highlights
This study reports on the analysis of the application and diagnostic predictability of the revised 2014 International Criteria for Behçet’s disease (ICBD) criteria in an unselected cohort of UK patients, and the ensuing organ associations and patterns of disease
ICBD 2014 demonstrated an estimated sensitivity of 97.89%, compared to 94.8% quoted in the original validation set, considerably higher than that of the International Study Group (ISG) 1990 criteria (77.89%)
The proposed ICBD 2014 criteria were more sensitive at picking up cases than ISG 1990 using the multidisciplinary clinical assessment process as the gold standard
Summary
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. BD has a very heterogeneous and unpredictable phenotype with the potential to involve the cardiovascular, renal, gastrointestinal, pulmonary, vascular, musculoskeletal, urological and central nervous systems to varying degrees [2,3,4]. Behçet’s disease is more common, and often more severe, along the ancient Silk Road, which extended from eastern Asia to the Mediterranean [5, 6]. It is most common in Turkey (80 to 370 cases per 100,000) [6], while the prevalence is much lower in Northern European and North American populations (1 per 15,000 to 1 per 500,000) [7]. Familial clustering has been reported, most cases of Behçet’s are thought to be sporadic in onset [8, 9]
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