Sato Hideki, Iwasaki Eisuke, Nogawa Shigeru, Suzuki Shigeaki, Amano Takahiro, Fukuuchi Yasuo, Shimoda Masayuki, Okada Yasunori
Department of Neurology, School of Medicine, Keio University.
Rinsho Shinkeigaku. 2003 Aug;43(8):496-9.
We report a 62-year-old man with giant cell myocarditis and myositis associated with thymoma and myasthenia gravis (MG). He was diagnosed as having MG and invasive thymoma at the age of 45. After he had a myasthenic crisis at the age of 61, tacrolimus was indicated in order to improve his neurological symptoms, in addition to glucocorticoid. Three months later, he was readmitted to our hospital complaining of general fatigue and dyspnea. Serum level of creatine phosphokinase (9,835IU/L) and myocardium-derived troponin T (3.24 ng/mL) were elevated, and the ECG showed atrioventricular dissociation and accelerated idioventricular rhythm. In spite of glucocorticoid pulse therapies and high-dose immunoglobulin therapies, he died of cardiac failure within a few days. Autopsy was done, and histological examination of the myocardium and the skeletal muscle showed massive necrosis with infiltration of inflammatory cells including giant cells. These findings constituted giant cell myocarditis and myositis. Although it has been suggested that myocardial disorders can often occur in patients with thymoma and/or MG, the mechanism involved is still unknown. This report may provide new knowledge about the pathophysiology of giant cell myocarditis and myositis associated with thymoma and MG.
我们报告了一名62岁男性，患有与胸腺瘤和重症肌无力（MG）相关的巨细胞性心肌炎和肌炎。他在45岁时被诊断为重症肌无力和侵袭性胸腺瘤。61岁时发生重症肌无力危象后，除糖皮质激素外，开始使用他克莫司以改善其神经症状。三个月后，他因全身乏力和呼吸困难再次入院。血清肌酸磷酸激酶水平（9,835IU/L）和心肌肌钙蛋白T（3.24 ng/mL）升高，心电图显示房室分离和加速性室性自主心律。尽管进行了糖皮质激素冲击治疗和大剂量免疫球蛋白治疗，但他在数天内死于心力衰竭。进行了尸检，心肌和骨骼肌的组织学检查显示大量坏死，并伴有包括巨细胞在内的炎性细胞浸润。这些发现构成了巨细胞性心肌炎和肌炎。虽然已有研究提示胸腺瘤和/或重症肌无力患者常可发生心肌病变，但其相关机制仍不清楚。本报告可能为与胸腺瘤和重症肌无力相关的巨细胞性心肌炎和肌炎的病理生理学提供新的认识。
