Mavrogeni Sophie, Sfikakis Petros P, Dimitroulas Theodoros, Koutsogeorgopoulou Loukia, Karabela Georgia, Katsifis Gikas, Stavropoulos Efthymios, Gialafos Elias, Spiliotis George, Kolovou Genovefa, Kitas George D
Onassis Cardiac Surgery Center, 50 Esperou Street, 175 61 P. Faliro, Athens, Greece.
Inflamm Allergy Drug Targets. 2015;14(2):111-6. doi: 10.2174/1871528114666160105112758.
To clarify the imaging patterns of cardiovascular lesions in patients with mixed connective tissue disease (MCTD) and cardiovascular symptoms with or/ without abnormal routine non-invasive evaluation.
PATIENTS-METHODS: Twenty-two MCTD patients (19F/3M), aged 38±4 yrs with cardiovascular symptoms were evaluated using a 1.5 T scanner. Of them, 8/22 had systemic lupus erythematosus (SLE), 5/22 rheumatoid arthritis (RA), 5/22 scleroderma (SSc) and 4/22 myositis (MY) overlap syndromes; 10/22 patients with MCTD presented with Raynaud phenomenon (RP) and all were positive for Anti-RNP antibodies. The cardiovascular magnetic resonance study (CMR) included evaluation of function, inflammation and fibrosis. Myocardial stress perfusion-fibrosis evaluation was performed only in MCTD patients with RP.
A positive CMR study was identified in 4/8 with SLE, 1/5 with RA, 4/5 with SSc and in 1/4 with MY like MCTD. The CMR lesions were subendocardial or transmural LGE following the distribution of coronary arteries, intramyocardial LGE and diffuse subendocardial LGE in SLE-RA, MY and SSc like MCTD, respectively. Although no evidence of fibrosis was identified in patients with RP, adenosine stress myocardial perfusion revealed diffuse subendocardial perfusion defects. No correlation between disease duration and/or inflammatory indices and cardiac lesions was identified.
CMR can reveal myocardial lesions in MCTD patients with cardiac symptoms including myocardial infarction, inflammation, diffuse subendocardial fibrosis and diffuse perfusion defects, necessitating further cardiac investigation and/or treatment.
明确混合性结缔组织病（MCTD）合并心血管症状患者的心血管病变影像学表现，无论常规非侵入性评估是否异常。
对22例年龄38±4岁、有心血管症状的MCTD患者（19例女性/3例男性）使用1.5T扫描仪进行评估。其中，8/22有系统性红斑狼疮（SLE）、5/22有类风湿关节炎（RA）、5/22有硬皮病（SSc）和4/22有肌炎（MY）重叠综合征；10/22例MCTD患者出现雷诺现象（RP），且抗RNP抗体均为阳性。心血管磁共振研究（CMR）包括功能、炎症和纤维化评估。仅对有RP的MCTD患者进行心肌应力灌注-纤维化评估。
4/8例SLE、1/5例RA、4/5例SSc和1/4例类似MCTD的MY患者CMR检查呈阳性。CMR病变在类似MCTD的SLE-RA、MY和SSc中分别为心内膜下或透壁性延迟强化（LGE），沿冠状动脉分布、心肌内LGE和弥漫性心内膜下LGE。尽管有RP的患者未发现纤维化证据，但腺苷负荷心肌灌注显示弥漫性心内膜下灌注缺损。未发现病程和/或炎症指标与心脏病变之间存在相关性。
CMR可揭示有心脏症状的MCTD患者的心肌病变，包括心肌梗死、炎症、弥漫性心内膜下纤维化和弥漫性灌注缺损，需要进一步的心脏检查和/或治疗。
