Background/Purpose: Behçet disease (BD) is characterized by painful recurrent oral aphthosis genital ulcers and skin lesions. Nevertheless, the major causes of morbidity result from ocular, vascular and neurological involvement. Diagnosis of BD is usually performed according to the International Study Group (ISG) (Lancet 1990; 335:1078-80). Recently, criteria proposed by the International Team for the Revision of the International Criteria for BD (ITR-ICBD) have demonstrated a higher sensitivity (J Eur Acad Dermatology Venereol 2014;28:338-47).

The aim of the present study was to assess a) the concordance and differences between ISG and ICDB criteria b) sensitivity in diagnosing severe manifestations (ocular, vascular and neurological).

Methods: The study included 120 patients diagnosed with definitive or possible BD by expert rheumatologists. They were diagnosed at a well-defined population in Northern Spain between January 1980 and December 2019. The ISG and ICBD diagnostic criteria for BD were applied to all patients and compared among them.

Results: 120 patients (62 men/ 58 women) were studied. Mean age at diagnosis was 37.6±13.8 years. 59 (49.2%) patients fulfilled ISG criteria and 96 (80%) ICBD criteria. Concordance between both criteria was moderate (Kappa 0.41). ICBD criteria diagnosed more patients with neurological (χ²=49.1, p< 0.01), vascular (χ²= 56.7, p< 0.01) and ocular manifestations (χ²=84.4 p< 0.01) (Figure).