Kawano Sayaka, Kato Johji, Kawano Noriaki, Yoshimura Yuki, Masuyama Hiroyuki, Fukunaga Takashi, Sato Yuichiro, Maruyama Haruhiko, Mihara Kenroh, Ueda Akira, Toyoda Kiyokazu, Imamura Takuroh, Kitamura Kazuo
Department of Internal Medicine, Circulatory and Body Fluid Regulation, Faculty of Medicine, University of Miyazaki, Japan.
Intern Med. 2011;50(9):975-81. doi: 10.2169/internalmedicine.50.4079. Epub 2011 May 1.
Eosinophilic myocarditis is a rare clinical entity characterized by eosinophilia and myocardial inflammation with infiltrating eosinophils. The prognosis of patients with eosinophilic myocarditis is difficult to determine due the disease's rarity and varied causes; consequently, standard treatment has not been established.
To elucidate the clinical characteristics and treatment outcome of eosinophilic myocarditis, we retrospectively studied 7 patients fulfilling the criteria of the Japanese Circulation Society for eosinophilic myocarditis from among 64 patients admitted to our institution with eosinophilia over a 27-year period.
The patients' ages at diagnosis ranged from 36 to 83 years (median: 52 years). The etiologies of the eosinophilic myocarditis were found to be idiopathic (3 patients), Churg-Strauss syndrome (2 patients), parasitic infection (1 patient) and chronic eosinophilic leukemia (CEL) (1 patient). In addition to treatment for the underlying disease, we also administered prednisolone at a dose appropriate to the disease severity (6 of 7 patients). The patient who was diagnosed with a parasitic infection was treated only with albendazole, because eosinophilic myocarditis was mild. The patient with CEL was positive for the FIP1 L1-PDGFRα fusion gene and was treated with imatinib. Eosinophilic cationic protein was a useful marker for assessing disease activity and treatment efficacy. At the end of the study, of the seven patients treated, six were alive (86%), giving a mean survival time of 37 ± 40 months (mean ± SD).
Because eosinophilic myocarditis has various etiologies, it is essential to identify the etiology of the underlying disease. In the majority of eosinophilic myocarditis patients, administration of prednisolone may be an effective therapeutic modality producing a good outcome.
嗜酸性粒细胞性心肌炎是一种罕见的临床病症,其特征为嗜酸性粒细胞增多以及伴有嗜酸性粒细胞浸润的心肌炎症。由于该疾病罕见且病因多样,嗜酸性粒细胞性心肌炎患者的预后难以确定;因此,尚未确立标准治疗方法。
为阐明嗜酸性粒细胞性心肌炎的临床特征及治疗结果,我们对在27年期间入住我院的64例嗜酸性粒细胞增多患者中符合日本循环学会嗜酸性粒细胞性心肌炎标准的7例患者进行了回顾性研究。
诊断时患者年龄为36至83岁(中位数:52岁)。发现嗜酸性粒细胞性心肌炎的病因包括特发性(3例患者)、变应性肉芽肿性血管炎(2例患者)、寄生虫感染(1例患者)和慢性嗜酸性粒细胞白血病(CEL)(1例患者)。除了针对基础疾病进行治疗外,我们还根据疾病严重程度给予了适当剂量的泼尼松龙(7例患者中的6例)。被诊断为寄生虫感染的患者仅接受阿苯达唑治疗,因为嗜酸性粒细胞性心肌炎较轻。患有CEL的患者FIP1 L1 - PDGFRα融合基因呈阳性,并接受了伊马替尼治疗。嗜酸性阳离子蛋白是评估疾病活动度和治疗效果的有用标志物。在研究结束时,接受治疗的7例患者中有6例存活(86%),平均生存时间为37±40个月(平均值±标准差)。
由于嗜酸性粒细胞性心肌炎病因多样,确定基础疾病的病因至关重要。在大多数嗜酸性粒细胞性心肌炎患者中,给予泼尼松龙可能是一种有效的治疗方式,可产生良好的治疗效果。