Rheumatology and Immunology Service, Department of Paediatric Subspecialties, KK Women's and Children's Hospital, Singapore, Singapore.
Division of Nursing, KK Women's and Children's Hospital, Singapore, Singapore.
Clin Rheumatol. 2022 Oct;41(10):3027-3034. doi: 10.1007/s10067-022-06275-z. Epub 2022 Jul 6.
To assess short- and long-term outcomes of ERA in a large monocentric cohort in Singapore.
Children diagnosed with ERA according to ILAR criteria from 2002 to 2021 were recruited. Nonparametric statistics were used to describe the data. Outcomes were defined according to modified Wallace criteria, and probabilities and predictors were determined using Kaplan-Meier survival and logistic regression analyses.
One hundred fifty-one ERA patients (male 86%; Chinese 81%) were included. The median age at onset was 11.9 years (IQR: 9.4-13.9), and disease duration was 5.3 years (IQR: 2.9-8.4). At diagnosis, 39% of the patients had sacroiliitis. HLA-B27 was positive in 83%, and biologics were used in 72% of the patients. Clinical inactive disease (CID) was achieved in 92% of the patients, of which 27% achieved within 6 months. Sacroiliitis at diagnosis is an unfavorable predictor of early CID at 6 months. Medication was discontinued in one-third of the patients. Favorable predictor of medication withdrawal includes male gender, while unfavorable predictors include positive HLA-B27 and ANA. Two-thirds of the patients with CID had at least one disease flare. Sacroiliitis at diagnosis is a protective predictor of flare after stopping medication.
Despite a high proportion of ERA patients achieving CID, only one-third could stop medication with high rates of disease flare. Unfavorable predictors include older age at onset, HLA-B27, and ANA positivity. While sacroiliitis at diagnosis is a negative predictor of CID at 6 months, it is associated with less disease flare after discontinuing medication.
评估新加坡单中心大队列中 ERA 的短期和长期结局。
招募了 2002 年至 2021 年根据 ILAR 标准诊断为 ERA 的儿童。使用非参数统计描述数据。根据改良 Wallace 标准定义结局,并使用 Kaplan-Meier 生存和逻辑回归分析确定概率和预测因素。
共纳入 151 例 ERA 患者(男性 86%;中国人 81%)。发病年龄中位数为 11.9 岁(IQR:9.4-13.9),病程为 5.3 年(IQR:2.9-8.4)。诊断时,39%的患者存在骶髂关节炎。83%的患者 HLA-B27 阳性,72%的患者使用生物制剂。92%的患者达到临床无活动疾病(CID),其中 27%在 6 个月内达到。诊断时的骶髂关节炎是 6 个月内早期 CID 的不利预测因素。三分之一的患者停止用药。停药的有利预测因素包括男性,而不利预测因素包括 HLA-B27 和 ANA 阳性。达到 CID 的患者中有三分之二至少有一次疾病发作。诊断时的骶髂关节炎是停止用药后疾病发作的保护预测因素。
尽管 ERA 患者达到 CID 的比例较高,但只有三分之一的患者能够停药且疾病发作率较高。不利的预测因素包括发病年龄较大、HLA-B27 和 ANA 阳性。虽然诊断时的骶髂关节炎是 6 个月时 CID 的负性预测因素,但与停药后疾病发作较少相关。