Mavrogeni Sophie, Koutsogeorgopoulou Loukia, Karabela Georgia, Stavropoulos Efthymios, Katsifis Gikas, Raftakis John, Plastiras Sotiris, Noutsias Michel, Markousis-Mavrogenis George, Kolovou Genovefa
Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61 P.Faliro, Athens, Greece.
Pathophysiology Department, Laikon Hospital, Athens, Greece.
BMC Cardiovasc Disord. 2017 Jul 17;17(1):187. doi: 10.1186/s12872-017-0619-x.
Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis. We hypothesized that myocarditis may be diagnosed in asymptomatic SSc, undergoing routine cardio-vascular magnetic resonance (CMR) for fibrosis assessment, using the Lake Louise criteria: T2 ratio, early (EGE) and late gadolinium enhanced (LGE) images.
Eighty-two asymptomatic SSc, diagnosed according to American College of Rheumatology criteria, aged 43 ± 5 yrs., 62 with diffuse (dSSc) and 20 with localized (lSSc) systemic sclerosis were evaluated by CMR, performed at 1.5 T scanner, according to Lake Louise criteria.
CMR documented normal biventricular function in all SSc. However, 7/62 (11.2%) with dSSc and 2/20 (10%) with lSSc, had CMR signs of myocarditis according to Lake Louise criteria, without any clinical cardiac symptom. In these 9 patients, T2 ratio, EGE ratio and LGE (positive in all 9 SSc) were 2.8 ± 0.5%, 8 ± 3% and 5 ± 3% of LV mass, respectively. No correlation between CMR and blood inflammatory indices (C-reactive protein and erythrocyte sedimentation rate), cardiac troponin T, disease characteristics or type of SSc was identified. A repeat CMR at 6 months, after treatment with prednisone and azathioprine, showed normalisation of the acute inflammation CMR indices.
Silent myocarditis may be diagnosed using the Lake Louise paper criteria in SSc patients without cardiac symptoms, has no correlation with blood inflammatory indices, cardiac troponin or disease characteristics. CMR is a promising tool to diagnose silent myocarditis in SSc and monitor the response to immunosuppressive treatment.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为微血管异常、炎症和纤维化。我们推测，对于无症状的SSc患者，在进行常规心血管磁共振成像（CMR）以评估纤维化时，可能会根据路易斯湖标准（T2比值、早期钆增强[EGE]和晚期钆增强[LGE]图像）诊断出心肌炎。
根据美国风湿病学会标准诊断的82例无症状SSc患者，年龄43±5岁，其中62例为弥漫性（dSSc）系统性硬化症，20例为局限性（lSSc）系统性硬化症，在1.5T扫描仪上按照路易斯湖标准进行CMR评估。
CMR显示所有SSc患者的双心室功能正常。然而，根据路易斯湖标准，62例dSSc患者中有7例（11.2%）、20例lSSc患者中有2例（10%）有CMR心肌炎征象，但无任何临床心脏症状。在这9例患者中，T2比值、EGE比值和LGE（所有9例SSc患者均为阳性）分别占左心室质量的2.8±0.5%、8±3%和5±3%。未发现CMR与血液炎症指标（C反应蛋白和红细胞沉降率）、心肌肌钙蛋白T、疾病特征或SSc类型之间存在相关性。在用泼尼松和硫唑嘌呤治疗6个月后重复进行CMR检查，结果显示急性炎症CMR指标恢复正常。
在无心脏症状的SSc患者中，可使用路易斯湖标准诊断隐匿性心肌炎，其与血液炎症指标、心肌肌钙蛋白或疾病特征无关。CMR是诊断SSc隐匿性心肌炎并监测免疫抑制治疗反应的一种有前景的工具。
