Berger Simon Girmai, Berntsen Kristin Schjander, Risum Kristine, Marstein Henriette Schermacher, Sjaastad Ivar, Stokke Mathis Korseberg, Sanner Helga
Oslo University Hospital Institute for Experimental Medical Research, Oslo, Norway.
Department of Rheumatology, Oslo University Hospital Division of Surgery and Specialized Medicine, Oslo, Oslo, Norway.
RMD Open. 2025 Jun 5;11(2):e005598. doi: 10.1136/rmdopen-2025-005598.
To quantify longitudinal development of disease activity, organ damage, patient-reported outcomes, and myositis autoantibody profiles in patients with juvenile dermatomyositis (JDM) after long-term follow-up, and to identify predictors for persistent active disease.
Forty patients (65% female) diagnosed with JDM were clinically examined at two different time points (visits 1 and 2). We assessed clinically inactive/active disease by the updated PRINTO criteria and the Juvenile DermatoMyositis Activity Index (JDMAI). Organ damage was evaluated by Myositis Damage Index (MDI) and physical function by Childhood Health Assessment Questionnaire (CHAQ/HAQ). Myositis autoantibodies were measured by myositis line immunoassay.
Median disease duration from symptom onset was 15.1 (2.0-34.6) at visit 1 and 21.7 (7.6-42.7) years at visit 2. At visit 2, active disease (PRINTO) was found in 53%, impaired physical function (CHAQ/HAQ>0) in 40%, organ damage (MDI≥1) in 95% and myositis specific or associated antibodies in 33% of patients. Disease activity (JDMAI) was low in 24%, moderate in 8% and high in 3% of patients. There were no significant differences in organ damage and disease activity between visits. Higher disease activity and organ damage at visit 1 predicted persistent active disease at visit 2.
After a median of 21.7 years from symptom onset, the majority of JDM patients still had active disease. Higher organ damage and disease activity at the initial visit predicted persistent active disease at follow-up. These results underscore the chronic nature of JDM, emphasising the need for enhanced early and long-term management strategies to improve patient outcomes.
对青少年皮肌炎（JDM）患者进行长期随访后，量化疾病活动度、器官损害、患者报告结局及肌炎自身抗体谱的纵向发展情况，并确定持续性活动性疾病的预测因素。
40例诊断为JDM的患者（65%为女性）在两个不同时间点（访视1和访视2）接受临床检查。我们采用更新后的PRINTO标准和青少年皮肌炎活动指数（JDMAI）评估临床非活动性/活动性疾病。通过肌炎损害指数（MDI）评估器官损害，通过儿童健康评估问卷（CHAQ/HAQ）评估身体功能。通过肌炎线性免疫分析法检测肌炎自身抗体。
访视1时症状出现后的疾病持续时间中位数为15.1（2.0 - 34.6）年，访视2时为21.7（7.6 - 42.7）年。在访视2时，53%的患者存在活动性疾病（PRINTO标准），40%的患者身体功能受损（CHAQ/HAQ>0）；95%的患者存在器官损害（MDI≥1），33%的患者存在肌炎特异性或相关抗体。24%的患者疾病活动度（JDMAI）低，8%的患者为中度，3%的患者为高度。两次访视之间器官损害和疾病活动度无显著差异。访视1时较高的疾病活动度和器官损害可预测访视2时的持续性活动性疾病。
从症状出现起中位数21.7年后，大多数JDM患者仍患有活动性疾病。初次访视时较高的器官损害和疾病活动度可预测随访时的持续性活动性疾病。这些结果强调了JDM的慢性本质，突出了加强早期和长期管理策略以改善患者结局的必要性。
