Objectives: To present and analyze the literature sources regarding the management of Behçet’s disease (BD) identified during the systematic literature research, which formed the basis for the EULAR evidence based recommendations for the management of BD.
Methods: Problem areas and related keywords regarding the management of BD were determined by the multidisciplinary expert committee commissioned by EULAR for developing the recommendations. Systematic literature research was performed using MedLine and Cochrane library through December 2006. Meta analyses, systematic reviews, randomized controlled trials (RCT), open studies, observational studies, case control studies, case series involving ≥5 patients were included. For each intervention the effect size and number needed to treat were calculated for efficacy. Odds ratios and number needed to harm were calculated for safety issues of different treatment modalities where possible.
Results: The literature research yielded 137 articles, which met the inclusion criteria. Twenty of these were randomized contrrolled trials (RCTs). There was good evidence supporting the use of azathioprine and cyclosporine A in eye involvement and interferon-α in mucocutaneous involvement. There were no RCTs with interferon-α or TNF-α antagonists in eye involvement. Similarly controlled data for the management of vascular, gastrointestinal and neurological involvement is lacking.
Conclusion: Properly designed controlled studies, both new and confirmatory, are still needed to guide us in managing BD.