Tsuchiya Hiroki, Kashimura Takeshi, Washiyama Yuzo, Kumaki Takayuki, Watanabe Mitsuhiro, Kase Mayumi, Ishizuka Mitsuo, Sakai Ryohei, Fujiki Shinya, Takayama Tsugumi, Ishihara Shiro, Inomata Takayuki
Department of Cardiovascular Biology and Medicine, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
J Cardiol Cases. 2023 Feb 17;27(6):258-261. doi: 10.1016/j.jccase.2023.01.009. eCollection 2023 Jun.
A 45-year-old woman with no medical history underwent pacemaker implantation for a symptomatic complete atrioventricular block. On day 6, she noticed diplopia and then fever, general malaise, and elevation of serum creatinine kinase (CK). She was transferred to our hospital on day 21. Serum CK was elevated to 4543 IU/L, and echocardiography revealed a left ventricular ejection fraction of 43 %. We diagnosed her with giant cell myocarditis (GCM) via an emergent myocardial biopsy that revealed a proliferation of lymphocytes, eosinophils, and giant cells without granulomas. Initial treatment with high doses of intravenous methylprednisolone and immunoglobulin improved her symptoms in a few days, and prednisolone was given as follow-up treatment. CK was normalized in a week and a thinning of the interventricular septum mimicking cardiac sarcoidosis (CS) occurred. On day 38, we added a calcineurin inhibitor, tacrolimus, and maintained her with a combination of prednisolone and tacrolimus at a target dose of 10-15 ng/mL. Six months after the onset, there were no signs of relapse despite the persistent mild elevation of troponin I levels. We present a case of GCM mimicking CS successfully maintained by a combination of two immunosuppressive agents.
Recommended treatment for giant cell myocarditis (GCM), a potentially fatal disease, is a combination of three immunosuppressive agents. However, GCM shares many characteristics with cardiac sarcoidosis (CS), which is treated using prednisolone alone in many cases. Recent studies on GCM and CS suggest they are different spectrums of a common entity. Although they can clinically overlap, they have different progressive speeds and severities. We present a case of GCM mimicking CS successfully treated with a combination of two immunosuppressive agents.
一名无病史的45岁女性因有症状的完全性房室传导阻滞接受了起搏器植入术。在第6天,她出现复视,随后出现发热、全身不适以及血清肌酸激酶(CK)升高。她于第21天被转至我院。血清CK升高至4543 IU/L,超声心动图显示左心室射血分数为43%。通过紧急心肌活检,我们诊断她为巨细胞性心肌炎(GCM),活检显示淋巴细胞、嗜酸性粒细胞和巨细胞增生,无肉芽肿形成。最初使用大剂量静脉注射甲泼尼龙和免疫球蛋白治疗,几天后症状改善,随后给予泼尼松龙进行后续治疗。CK在一周内恢复正常,出现了类似于心脏结节病(CS)的室间隔变薄。在第38天,我们加用了钙调神经磷酸酶抑制剂他克莫司,并以泼尼松龙和他克莫司联合维持治疗,目标剂量为10 - 15 ng/mL。发病6个月后,尽管肌钙蛋白I水平持续轻度升高,但无复发迹象。我们报告一例通过两种免疫抑制剂联合成功维持治疗的酷似CS的GCM病例。
对于潜在致命性疾病巨细胞性心肌炎(GCM),推荐的治疗方法是三种免疫抑制剂联合使用。然而,GCM与心脏结节病(CS)有许多共同特征,在许多情况下CS仅用泼尼松龙治疗。最近关于GCM和CS的研究表明它们是同一疾病的不同谱系。尽管它们在临床上可能重叠,但它们的进展速度和严重程度不同。我们报告一例通过两种免疫抑制剂联合成功治疗的酷似CS的GCM病例。