Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States; Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States.
Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States; Hacettepe University Vasculitis Research Centre, Ankara, Turkey.
Semin Arthritis Rheum. 2022 Feb;52:151924. doi: 10.1016/j.semarthrit.2021.10.007. Epub 2021 Nov 14.
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To date, no studies have defined the clinical spectrum of disease in a cohort of patients with MAGIC syndrome.
Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome by searching four databases: PubMed (US National Library of Medicine), Embase (Elsevier), Scopus (Elsevier) and Web of Science: Core Collection (Clarivate Analytics). The inclusion criteria used were: [1] patients of any age or gender who were diagnosed with MAGIC syndrome, or both RP and BD; [2] case report or case series study; [3] published from 1985 - July 2020; and [4] in English language. Risk of bias was assessed using a checklist developed by the authors and based on the Consensus-based Clinical Case Reporting (CARE) Guidelines. Search results screening, article inclusion, data extraction and risk of bais assessment was performed independently by two investigators. Clinical characteristics, particularly BD-related features, were compared between patients with MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of different criteria to classify MAGIC syndrome were also evaluated.
Out of 96 patients with RP, 13 (14%) patients were diagnosed with MAGIC syndrome. For the systematic review, 380 articles were retrieved of which 90 were screened at title and abstract levels. Of these screened, 60 were excluded and 30 proceeded to full text review where an additional 8 were excluded. Twenty-two articles were included in our review and from which 27 additional cases of MAGIC syndrome were identified. Pooling all 40 cases together and comparing them with non-MAGIC RP, there was a significantly higher prevalence of ocular involvement (28% vs 4%, p<0.01), cutaneous involvement (35% vs 1%, p<0.01), GI involvement (23% vs 4%, p<0.01), and CNS involvement (8% vs 0, p = 0.04) in MAGIC syndrome. A higher prevalence of aortitis (23% vs 1%, p<0.01), Raynaud's phenomenon (54% vs 11%, p<0.01), and elevated anti-collagen II antibodies (50% vs 9%, p = 0.04) were observed in MAGIC syndrome. Fulfillment of either McAdam's or Damiani's Criteria for RP plus the International Criteria for Behçet's Disease had excellent sensitivity (98%) to classify cases of MAGIC syndrome.
A substantial proportion of patients with RP can be clinically diagnosed with MAGIC syndrome. These patients have features of RP, BD, and other unique features including aortitis, Raynaud's phenomenon and elevated anti-collagen II antibodies.
口和生殖器溃疡伴软骨炎（MAGIC）综合征的特征是重叠的复发性多软骨炎（RP）和白塞病（BD）的特征。迄今为止，尚无研究在 MAGIC 综合征患者的队列中定义疾病的临床谱。
正在进行的前瞻性、观察性 RP 队列研究中的成年患者接受 MAGIC 综合征的临床评估。通过在四个数据库（美国国立医学图书馆的 PubMed、爱思唯尔的 Embase、爱思唯尔的 Scopus 和 Clarivate Analytics 的 Web of Science：核心合集）中进行系统检索，以识别 MAGIC 综合征的其他病例。使用的纳入标准为：[1] 任何年龄或性别的患者，诊断为 MAGIC 综合征或 RP 和 BD 均确诊；[2] 病例报告或病例系列研究；[3] 发表于 1985 年至 2020 年 7 月；[4] 英文。使用作者制定的基于共识的临床病例报告（CARE）指南的检查表评估偏倚风险。两名研究者独立进行了研究结果筛选、文章纳入、数据提取和偏倚风险评估。比较 MAGIC 综合征患者和非 MAGIC RP 病例的临床特征，特别是 BD 相关特征。还评估了不同标准分类 MAGIC 综合征的性能特征。
在 96 例 RP 患者中，有 13 例（14%）患者被诊断为 MAGIC 综合征。对于系统评价，检索到 380 篇文章，其中 90 篇进行了标题和摘要筛选。在筛选的文章中，有 60 篇被排除，30 篇进入全文审查，其中另外 8 篇被排除。22 篇文章被纳入我们的综述，从中确定了 27 例额外的 MAGIC 综合征病例。将所有 40 例病例汇总，并与非 MAGIC RP 病例进行比较，MAGIC 综合征患者中眼受累（28% vs 4%，p<0.01）、皮肤受累（35% vs 1%，p<0.01）、胃肠道受累（23% vs 4%，p<0.01）和中枢神经系统受累（8% vs 0，p=0.04）的发生率明显更高。MAGIC 综合征患者中主动脉炎（23% vs 1%，p<0.01）、雷诺现象（54% vs 11%，p<0.01）和抗胶原 II 抗体升高（50% vs 9%，p=0.04）的发生率更高。
相当一部分 RP 患者可临床诊断为 MAGIC 综合征。这些患者具有 RP、BD 和其他独特特征的特征，包括主动脉炎、雷诺现象和抗胶原 II 抗体升高。
