Rheumatology Institute of Lucania (IRel) and the Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza and Matera, Italy.
Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul 34098, Turkey.
Semin Arthritis Rheum. 2019 Feb;48(4):752-762. doi: 10.1016/j.semarthrit.2018.05.008. Epub 2018 May 19.
The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet's syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.
A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.
Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study.
RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results.
本系统评价旨在为欧洲抗风湿病联盟（EULAR）更新关于白塞病（BS）管理的建议提供信息，重点是 BS 的皮肤、黏膜和关节受累的治疗证据。
根据 PRISMA 指南的预定义方案，进行系统文献检索、数据提取、统计分析和证据质量评估。评估干预措施与阳性对照或安慰剂相比在治疗口腔溃疡、生殖器溃疡、丘疹脓疱性病变、结节性病变或关节炎方面疗效的研究被纳入。在可能的情况下，对二项结局计算风险比，对连续结局计算均数差。
在筛选的 3927 篇参考文献中，有 37 篇被纳入分析。其中 27 篇评估黏膜皮肤受累，17 篇评估关节受累。这些研究中有 21 项为随机对照试验（RCT）。秋水仙碱、硫唑嘌呤、干扰素-α、沙利度胺、依那西普和阿普米司特的 RCT 显示出有益的结果，但根据病变类型和性别存在一些差异。这些药物通常具有良好的耐受性，很少有不良反应导致退出研究。
在与皮肤、黏膜和关节受累相关的 EULAR 推荐治疗 BS 的证据综合中，RCT 占纳入研究来源的一半以上（21/37，57%）。纳入研究中使用的结局测量指标的差异常常使得难以合并和比较结果。
