Development and validation of novel diagnostic criteria for intestinal Behçet's disease in Korean patients with ileocolonic ulcers

Abstract

Objectives: It is difficult to diagnose intestinal Behçet's disease (BD) due to various extraintestinal manifestations emerging at different time points in the disease course and a lack of reliable diagnostic criteria. We conducted this study to develop and validate novel diagnostic criteria for intestinal BD.

Methods: Experts from three universities generated the preliminary diagnostic criteria for intestinal BD, and a consensus was reached using a modified Delphi method with 13 gastroenterologists participating. To validate the criteria, we recruited 12,850 consecutive patients who underwent colonoscopic examinations between January 2000 and December 2006 at Severance Hospital, Yonsei University, Seoul, Korea.

Results: The novel diagnostic criteria were developed on the basis of two aspects: colonoscopic findings and extraintestinal manifestations. Of the 12,850 patients, 280 with ileocolonic ulcers were enrolled for validation. At the time of initial colonoscopic examinations, patients were categorized for BD status into 4 groups: definite (84 patients), probable (67), suspected (15), and nondiagnostic (114). At the end of the follow-up period (mean, 50.9+/-25.7 months), intestinal BD was confirmed in 145 patients (51.8%)-84 (100%) from the definite group, 49 (73.1%) from the probable group, 10 (66.7%) from the suspected group, and 2 (1.8%) from the nondiagnostic group. The overall sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the diagnosis probability of these criteria were 98.6, 83.0, 86.1, 98.2, and 91.1%, respectively.

Conclusions: These newly proposed, simple criteria might be useful in diagnosing intestinal BD, especially in patients with ileocolonic ulcers who do not fully satisfy the diagnostic criteria of systemic BD.