目的:起始轴肌受累,定义为跌落头综合征(DHS)和/或喜忧乐(CC),在炎性肌病(IM)中描述不佳。这项研究旨在进一步表征患有DHS/CC的IM患者,他们的结果和护理管理。
方法:这项回顾性研究包括2000年至2021年诊断的IM患者。主要纳入标准是轴向肌缺陷(DHS/CC)显示的IM。
结果:纳入27例患者;首发症状时的中位年龄(IQR)为66.0岁(55.5-75.0);21例为女性(77.8%)。有9例包涵体肌炎(IBM,33.3%),9重叠肌炎(OM,33.3%),5皮肌炎(DM,18.5%),2免疫检查点抑制剂相关性肌炎(7.4%),1例局灶性肌炎(3.7%)和1例抗Hu抗体的肌炎(3.7%)。16例患者首次出现症状的年龄≤70岁(59.3%),包括所有DM患者和8/9OM患者(88.9%)。在这个群体中,9/16(56.3%)患者部分缓解,1/16患者完全缓解(6.3%);3/16患者(18.8%)实现了DHS/CC的消退。相反,在11名年龄>70岁的患者组中,有8个IBM(72.7%)。5/11患者部分缓解(45.5%),6/11例患者(54.5%)病情稳定,未完全缓解,DHS/CC也未消退.
结论:对患有首次DHS/CC的IM患者的分析根据临床和结果特异性的首发症状年龄划分了两组患者。并提出了一种适应的诊断和护理管理方法,以防止长期并发症。
OBJECTIVE: Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management.
METHODS: This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC).
RESULTS: Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5-75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC.
CONCLUSIONS: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.