Objectives The aim of this systematic literature review was to inform the task force for updating the European League Against Rheumatism recommendations for the management of Behçet's Syndrome (BS), about the evidence for treatment of skin, mucosa and joint involvement of BS.
Methods A systematic literature search, data extraction and statistical analyses according to pre-specified and protocolised eligibility criteria were performed using the GRADE approach. The protocol for the review was registered and is available at PROSPERO (CRD42015027033). The Cochrane Library, including the Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews (CDSR), Database of Abstracts of Reviews of Effects (DARE), Health Technology Assessments (HTA), MEDLINE (from 1950), EMBASE (from 1980) and International Pharmaceutical Abstracts Database (IPAD) were systematically searched. Randomised controlled trials (RCT), non-randomised controlled clinical trials and open label trials (OLT) on BS comparing an active intervention (alone or in combination) with any control or placebo were eligible. If controlled trials were not available for answering a specific research question, uncontrolled evidence from cohort studies or case series involving ≥5 patients were considered. The quality of evidence was assessed by using the GRADE approach. Risk ratios were calculated for the binary outcomes whereas for the continuous outcomes we calculated the standardized mean difference (SMD).
Results Among the 3927 references that we have screened, 22 studies satisfied the inclusion criteria for mucocutaneous involvement and 15 studies for joint involvement. Seventeen of these studies were RCTs assessing mucocutaneous and/or joint involvement. RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed different levels of beneficial results on different types of skin and mucosa lesions and arthritis. Differences in the outcome measures that were used across the included studies made it difficult to compare the results. These agents were generally well tolerated with few adverse events causing withdrawal from the study in BS patients.
Conclusions It was gratifying to see that randomised trials formed the majority (17/22, 77%) of the sources forming the basis for the recommendations related to skin mucosa and joint involvement in the updated EULAR recommendations for the management of BS.
Disclosure of Interest None declared