Maeda Meiko, Kawahara Takuya, Kubota Akatsuki, Shimizu Jun, Toda Tatsushi
Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Clinical Research Promotion Center, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
J Neurol Sci. 2024 Dec 15;467:123287. doi: 10.1016/j.jns.2024.123287. Epub 2024 Oct 28.
Anti-mitochondrial antibody (AMA)-positive myositis is a chronic disease characterized by skeletal muscle atrophy and is associated with cardiac complications and restrictive ventilatory impairment. This study aimed to determine the prevalence, rate of organ complications, and prognostic risk factors of AMA-positive myositis.
We conducted a cross-sectional study using a nationwide questionnaire from 2011 to 2021, enrolling participants from neurology departments at 811 facilities certified by the Japanese Society of Neurology.
A total of 380 patients were identified, with a prevalence rate of 0.3 per 100,000 persons. The frequencies of cardiac complications and restrictive ventilatory impairment were 53 and 33 %, respectively; whereas, those of cardiac device and respirator introduction were 32 and 22 %, respectively. The frequencies of recurrence, subacute exacerbation, no muscle strength improvement, cardiac device introduction, respirator introduction, and death were 29, 25, 54, 32, 22, and 12 %, respectively. According to univariate analysis, abnormal echocardiograms (odds ratio [OR], 5.43), restrictive ventilatory impairment (OR, 3.70), and inflammatory changes revealed by muscle biopsy (OR, 0.34) were associated with subacute exacerbations, whereas abnormal echocardiograms (OR, 8.00) and durations from onset to admission and diagnosis (OR, 2.99) were associated with cardiac device introduction. Multivariable analysis showed that restrictive ventilatory impairment was associated with recurrence (adjusted OR, 3.01), adjusted for the duration from onset to admission and diagnosis, and with subacute exacerbations (adjusted OR, 3.86), adjusted for abnormal echocardiograms and inflammatory changes.
AMA-positive myositis is characterized by severe and urgent organ complications, and anticipatory management is critical for management of this disease.
抗线粒体抗体（AMA）阳性肌炎是一种以骨骼肌萎缩为特征的慢性疾病，与心脏并发症和限制性通气功能障碍有关。本研究旨在确定AMA阳性肌炎的患病率、器官并发症发生率及预后危险因素。
我们使用2011年至2021年的全国性调查问卷进行了一项横断面研究，纳入了日本神经学会认证的811家机构神经内科的参与者。
共识别出380例患者，患病率为每10万人0.3例。心脏并发症和限制性通气功能障碍的发生率分别为53%和33%；而心脏装置植入和使用呼吸机的发生率分别为32%和22%。复发、亚急性加重、肌力未改善、心脏装置植入、使用呼吸机和死亡的发生率分别为29%、25%、54%、32%、22%和12%。单因素分析显示，超声心动图异常（比值比[OR]，5.43）、限制性通气功能障碍（OR，3.70）以及肌肉活检显示的炎症改变（OR，0.34）与亚急性加重相关，而超声心动图异常（OR，8.00）以及起病至入院和诊断的时间（OR，2.99）与心脏装置植入相关。多变量分析显示，在调整起病至入院和诊断的时间后，限制性通气功能障碍与复发相关（调整后OR，3.01），在调整超声心动图异常和炎症改变后，与亚急性加重相关（调整后OR，3.86）。
AMA阳性肌炎以严重且紧急的器官并发症为特征，前瞻性管理对该疾病的治疗至关重要。
