Multicenter inception cohort of enthesitis-related arthritis: variation in disease characteristics and treatment approaches.
Multicenter inception cohort of enthesitis-related arthritis: variation in disease characteristics and treatment approaches.
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DOI：
10.1186/s13075-017-1297-x
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发表时间：
2017-05-02
影响因子：
4.9
通讯作者：
Weiss PF
中科院分区：
文献类型：
--
作者：
Gmuca S;Xiao R;Brandon TG;Pagnini I;Wright TB;Beukelman T;Morgan EM;Weiss PF
关节炎相关关节炎（ERA）是根据国际风湿病协会联盟（ILAR）标准定义的青少年特发性关节炎（JIA）的一种特定亚型。我们的目的是描述ERA患儿的临床特征和治疗方案。我们进行了一项回顾性、横断面、多中心队列研究，包括1989年至2012年间诊断为ERA的受试者。所有患者在首次到风湿病诊所就诊后的3个月内均符合ERA的ILAR标准。临床标准在不同研究地点和人类白细胞抗原(HLA)-B27状态下的流行程度差异，酌情使用Wilcoxon秩和或卡方检验进行评估。234名儿童符合纳入标准。确诊时的中位年龄为11.6岁，59%为hla - b27阳性。69%的人在诊断时患有鼻炎和关节炎。78%的患者患有关节功能不全。除关节炎和急性前葡萄膜炎外，所有ILAR诊断标准的患病率在不同部位差异显著（均p < 0.01）。周围性关节炎患儿不同部位的药物使用差异显著（p < 0.001），但骶髂炎或阑尾炎患儿无明显差异。非甾体类抗炎药和改善疾病的抗风湿药是最常用的治疗方法，抗肿瘤坏死因子药物主要用于骶髂炎的起始治疗。HLA-B27阳性与男性、较高的活动关节计数、骶髂炎和发病时较高的疾病活动性相关。大多数儿童有关节缺失的发病，并且基于HLA-B27状态确定了几个具有统计学意义的临床差异。观察到的不同地区临床表现的异质性反映了患者群体的真实差异或ILAR标准应用方式的差异。
Enthesitis-related arthritis (ERA) is a specific subtype of juvenile idiopathic arthritis (JIA) defined according to the International League of Associations for Rheumatology (ILAR) criteria. We aimed to characterize the clinical features and treatment regimens in an inception cohort of children with ERA. We performed a retrospective, cross-sectional, multicenter cohort study including subjects diagnosed with ERA between 1989 and 2012. Patients all fulfilled the ILAR criteria for ERA within 3 months of initial presentation to the rheumatology clinic. Differences in the prevalence of clinical criteria across study sites and by human leukocyte antigen (HLA)-B27 status were assessed using the Wilcoxon rank-sum or chi-square test, as appropriate. Two hundred thirty-four children met the inclusion criteria. Their median age at diagnosis was 11.6 years, and 59% were HLA-B27-positive. Sixty-nine percent had enthesitis and arthritis at the time of diagnosis. Seventy-eight percent had a pauciarticular onset. The prevalence of all ILAR criteria at diagnosis, except arthritis and acute anterior uveitis, differed significantly across sites (all p < 0.01). Medication use varied significantly across sites for children with peripheral arthritis (p < 0.001), but not for sacroiliitis or enthesitis only. Nonsteroidal anti-inflammatory drugs and disease-modifying antirheumatic drugs were the most commonly prescribed treatments, with anti-TNF agents primarily being initiation for sacroiliitis. HLA-B27 positivity was associated with male sex, higher active joint count, sacroiliitis, and higher disease activity at disease onset. The majority of children had a pauciarticular onset, and several statistically significant clinical differences based on HLA-B27 status were identified. The observed heterogeneity in clinical presentation across sites reflects either true differences in patient populations or differences in how the ILAR criteria are being applied.