Takahashi T, Suzuki T, Suzuki A, Yamada T, Koido N, Akizuki S, Matsuoka Y, Irimajiri S, Fukuda J
Department of Internal Medicine, Kawasaki Municipal Hospital, Kanagawa.
Ryumachi. 1999 Jun;39(3):573-9.
A 29-year-old female with systemic lupus erythematosus (SLE) was admitted because of exertional dyspnea in January, 1996. The diagnosis of SLE was made on the basis of malar rash, discoid rash, polyarthritis, leukopenia, positive antinuclear antibody and focal glomerulonephritis in 1991. She did not have any cardiac symptoms in 1994, when the electrocardiogram (ECG) abnormalities, such as poor R wave progression and right axis deviation, were present. On admission, she developed congestive heart failure without any signs of active SLE. Laboratory findings were unremarkable. There were new ECG abnormalities, such as left atrial overload and low voltage in limb leads. Chest roentgenogram showed mild pulmonary congestion and marked cardiomegaly. Echocardiography showed enlargement and diffuse hypokinesis of the left ventricle. Cardiac catheterization confirmed that the coronary arteries were normal and that the left ventricular function was poor (ejection fraction, 21%). Myocardial biopsy obtained from left ventricle revealed interstitial fibrosis. After furosemide, digoxin and captril were administered with predonisolone (PSL), her symptoms gradually improved. Since the Holter monitoring showed nonsustained ventricular tachycardia, the doses of PSL and mexiletine were increased up to 20 mg and 300 mg daily, respectively. Unexpectedly, she was found dead in her hospital room in May, 1996. The postmortem findings of the heart revealed mild infiltration of inflammatory cells, predominantly lymphocytes, and plasma cells, and interstitial fibrosis, which were consistent with interstitial myocarditis. In this case ECG abnormalities preceded cardiac symptoms, which may suggest that myocarditis subclinically developed. Serum creatinine kinase levels had not been elevated throughout the entire course. While several cases of acute myocarditis associated with a flare of SLE have been reported, there were few cases regarding interstitial myocarditis that chronically progress and can be fatal. This case is thought to be suggestive of elucidating the pathogenesis of lupus myocarditis.
一名29岁的系统性红斑狼疮（SLE）女性患者于1996年1月因劳力性呼吸困难入院。该患者于1991年根据颧部红斑、盘状红斑、多关节炎、白细胞减少、抗核抗体阳性及局灶性肾小球肾炎确诊为SLE。1994年时她没有任何心脏症状，但当时心电图（ECG）存在异常，如R波递增不良和电轴右偏。入院时，她出现充血性心力衰竭，且无任何活动性SLE的体征。实验室检查结果无明显异常。出现了新的心电图异常，如左心房负荷过重和肢体导联低电压。胸部X线片显示轻度肺淤血和明显心脏增大。超声心动图显示左心室扩大和弥漫性运动减弱。心导管检查证实冠状动脉正常，左心室功能较差（射血分数为21%）。从左心室获取的心肌活检显示间质纤维化。给予速尿、地高辛和卡托普利并联合泼尼松龙（PSL）治疗后，她的症状逐渐改善。由于动态心电图监测显示非持续性室性心动过速，PSL和美西律的剂量分别增加至每日20毫克和300毫克。出乎意料的是，她于1996年5月在医院病房死亡。心脏的尸检结果显示有轻度炎症细胞浸润，主要为淋巴细胞和浆细胞，以及间质纤维化，这与间质性心肌炎相符。在该病例中，心电图异常先于心脏症状出现，这可能提示心肌炎是亚临床发生的。在整个病程中血清肌酐激酶水平均未升高。虽然已有几例与SLE病情活动相关的急性心肌炎的报道，但关于慢性进展且可能致命的间质性心肌炎的病例却很少。该病例被认为有助于阐明狼疮性心肌炎的发病机制。
