Department of Infection and Immunology, Aichi Children's Health and Medical Center, Obu, Japan.
The Japan Pediatric Sjögren's Syndrome Study Group, Tokyo, Japan.
Pediatr Rheumatol Online J. 2020 Sep 17;18(1):73. doi: 10.1186/s12969-020-00458-1.
The European League Against Rheumatism (EULAR) Sjögren Syndrome Disease Activity Index (ESSDAI) has been utilized to assess Sjögren syndrome-related systemic involvement in adult patients. To date, however, the ESSDAI has not been validated in children with primary Sjögren's syndrome. This study evaluated the applicability of the ESSDAI to Japanese children with primary Sjögren's syndrome.
The medical records of children who had been diagnosed with Sjogren syndrome at age ≤ 16 years between June 2011 and October 2016 were collected, and their ESSDAIs at initial presentation were calculated. Clinical symptoms and treatment regimens were surveyed by questionnaire, and patients were divided into groups based on ESSDAI and glucocorticoid dosages. The associations of ESSDAI scores with treatment regimens were analyzed statistically.
The study subjects included 31 children (3 boys, 28 girls) with primary Sjögren's syndrome. Their median age at disease onset was 10 years (interquartile range [IQR], 8-13 years), and their median initial ESSDAI was 7.0 (IQR; 5.0-15.0). ESSDAI-determined disease activity was high in nine patients (29.0%), moderate in 15 (48.4%), and low in seven (22.6%). During the first year after their initial visit, 14 patients (45.2%) were treated with prednisolone (PSL) and six (19.4%) with immunosuppressants. Dose of PSL was significantly associated with ESSDAI score. Median ESSDAI score was significantly higher in patients treated with high/medium- than with no/low-dose PSL (16.5 [IQR 10.5-18.0] vs 5.0 [IQR 3.0-8.5]). Eight (66.7%) of 12 patients administered medium/high-dose PSL and one (5.3%) of 19 administered no/low-dose PSL had high disease activity on ESSDAI.
Disease activity assessed by ESSDAI tended to be consistent with disease activity assessed by pediatric rheumatologists in determining treatment regimens. ESSDAI is useful for assessing disease activity in Japanese children with primary Sjögren's syndrome.
欧洲抗风湿病联盟（EULAR）干燥综合征疾病活动指数（ESSDAI）已被用于评估成人干燥综合征相关的系统性受累。然而，迄今为止，该指数尚未在原发性干燥综合征的儿童中得到验证。本研究评估了 ESSDAI 对日本原发性干燥综合征儿童的适用性。
收集了 2011 年 6 月至 2016 年 10 月期间被诊断为干燥综合征且年龄≤16 岁的儿童的病历，并计算了他们初诊时的 ESSDAI。通过问卷调查调查了临床症状和治疗方案，并根据 ESSDAI 和糖皮质激素剂量将患者分为不同的组。对 ESSDAI 评分与治疗方案之间的相关性进行了统计学分析。
本研究共纳入 31 例原发性干燥综合征儿童（3 例男性，28 例女性）。疾病发病的中位年龄为 10 岁（四分位距[IQR]，8-13 岁），初诊时的中位 ESSDAI 为 7.0（IQR：5.0-15.0）。9 例（29.0%）患者 ESSDAI 确定的疾病活动度较高，15 例（48.4%）为中度，7 例（22.6%）为低度。在初诊后的第一年，14 例（45.2%）患者接受了泼尼松龙（PSL）治疗，6 例（19.4%）患者接受了免疫抑制剂治疗。PSL 剂量与 ESSDAI 评分显著相关。接受高/中剂量 PSL 治疗的患者的 ESSDAI 中位数显著高于接受低剂量 PSL 治疗的患者（16.5 [IQR 10.5-18.0] vs. 5.0 [IQR 3.0-8.5]）。12 例接受中/高剂量 PSL 治疗的患者中，有 8 例（66.7%）和 19 例接受低剂量 PSL 治疗的患者中，有 1 例（5.3%） ESSDAI 显示疾病活动度高。
ESSDAI 评估的疾病活动度与儿科风湿病学家评估的疾病活动度在确定治疗方案时趋于一致。ESSDAI 可用于评估日本原发性干燥综合征儿童的疾病活动度。
