Kazama Shingo, Kondo Toru, Ito Ryota, Kimura Yuki, Kuwayama Tasuku, Hiraiwa Hiroaki, Morimoto Ryota, Okumura Takahiro, Murohara Toyoaki
Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
J Cardiol Cases. 2023 Mar 7;28(1):11-15. doi: 10.1016/j.jccase.2023.02.021. eCollection 2023 Jul.
Patients with dilated cardiomyopathy (DCM) sometimes show anti-mitochondrial M2 antibody (AMA-M2) positivity. We aimed to compare the characteristics of DCM cases with and without AMA-M2, and to describe cases of DCM with AMA-M2 positivity.A total of 84 patients with DCM were analyzed. Six patients (7.1 %) were positive for AMA-M2. Of these six patients, five (83.3 %) had primary biliary cirrhosis (PBC) and four (66.7 %) had myositis. Patients with AMA-M2 positivity had more atrial fibrillation and more premature ventricular contractions than those without. Left and right atrial longitudinal dimensions were larger in patients with AMA positivity (left atrium, 65.9 mm vs. 54.7 mm, p = 0.02; right atrium, 57.0 mm vs. 46.1 mm, p = 0.02). Of the six patients with AMA-M2 positivity, three underwent cardiac resynchronization therapy with defibrillator implantation and three required catheter ablation treatment. Steroids were used in three patients. One patient died of unresolved lethal arrhythmia and another required re-hospitalization for heart failure; the remaining four patients did not have adverse events.Patients with DCM with AMA-M2 positivity had a higher affinity for PBC and myositis than those without, and are characterized by atrial enlargement and arrhythmias.
Patients with dilated cardiomyopathy sometimes exhibit anti-mitochondrial M2 antibody positivity. These patients are at higher risk for primary biliary cirrhosis and inflammatory myositis, and their cardiac disorders are characterized by atrial enlargement and various arrhythmias. The course of the disease up to the time of diagnosis and after steroid use varies, and the prognosis is poor in advanced cases.
扩张型心肌病（DCM）患者有时会出现抗线粒体M2抗体（AMA-M2）阳性。我们旨在比较有和没有AMA-M2的DCM病例的特征，并描述AMA-M2阳性的DCM病例。共分析了84例DCM患者。6例患者（7.1%）AMA-M2阳性。在这6例患者中，5例（83.3%）患有原发性胆汁性肝硬化（PBC），4例（66.7%）患有肌炎。AMA-M2阳性的患者比阴性患者有更多的心房颤动和室性早搏。AMA阳性患者的左、右心房纵向直径更大（左心房，65.9mm对54.7mm，p = 0.02；右心房，57.0mm对46.1mm，p = 0.02）。在6例AMA-M2阳性患者中，3例接受了心脏再同步化治疗并植入除颤器，3例需要导管消融治疗。3例患者使用了类固醇。1例患者死于无法解决的致命心律失常，另1例因心力衰竭需要再次住院；其余4例患者没有不良事件。AMA-M2阳性的DCM患者比阴性患者对PBC和肌炎的亲和力更高，其特征为心房扩大和心律失常。
扩张型心肌病患者有时会出现抗线粒体M2抗体阳性。这些患者患原发性胆汁性肝硬化和炎性肌炎的风险更高，其心脏疾病的特征为心房扩大和各种心律失常。直至诊断时及使用类固醇后的疾病进程各不相同，晚期病例预后较差。
