Prevalence and risk surveillance of anti-mitochondrial antibody-positive myositis: Outcomes of a nationwide survey

IF 3.6 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences Pub Date : 2024-10-28 DOI:10.1016/j.jns.2024.123287

Meiko Maeda , Takuya Kawahara , Akatsuki Kubota , Jun Shimizu , Tatsushi Toda

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引用次数: 0

Abstract

Background

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.

Methods

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.

Results

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.

Conclusions

AMA-positive myositis is characterized by severe and urgent organ complications, and anticipatory management is critical for management of this disease.

查看原文本刊更多论文

抗线粒体抗体阳性肌炎的患病率和风险监测：全国性调查的结果。

背景：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障碍有关。本研究旨在确定抗线粒体抗体阳性肌炎的患病率、器官并发症发生率和预后风险因素：我们在 2011 年至 2021 年期间进行了一项横断面研究，在全国范围内进行了问卷调查，参与者来自日本神经病学学会认证的 811 家机构的神经病学部门：结果：共发现 380 名患者，患病率为每 10 万人中有 0.3 人。心脏并发症和限制性通气障碍的发生率分别为 53% 和 33%，而心脏装置和呼吸机的使用率分别为 32% 和 22%。复发、亚急性加重、肌力无改善、使用心脏装置、使用呼吸机和死亡的频率分别为 29%、25%、54%、32%、22% 和 12%。根据单变量分析，异常超声心动图（比值比 [OR]，5.43）、限制性通气功能障碍（OR，3.70）和肌肉活检显示的炎症变化（OR，0.34）与亚急性加重有关，而异常超声心动图（OR，8.00）和发病到入院和诊断的持续时间（OR，2.99）与心脏装置的引入有关。多变量分析显示，限制性通气功能障碍与复发有关（调整后OR值为3.01），与发病到入院和诊断的持续时间有关，与亚急性加重有关（调整后OR值为3.86），与异常超声心动图和炎症变化有关：结论：AMA阳性肌炎的特点是严重和紧急的器官并发症，预见性治疗对该病的治疗至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of the Neurological Sciences 医学-临床神经学

CiteScore

7.60

自引率

2.30%

发文量

313

审稿时长

22 days

期刊介绍： The Journal of the Neurological Sciences provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. JNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials). JNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism.