Panopoulos Stylianos, Mavrogeni Sophie, Vlachopoulos Charalambos, Sfikakis Petros P
First Department of Propaedeutic and Internal Medicine and Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Department of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece.
Rheumatology (Oxford). 2023 Apr 3;62(4):1535-1542. doi: 10.1093/rheumatology/keac504.
Cardiac magnetic resonance imaging (CMRI) is increasingly used to evaluate cardiac involvement in SSc. We assessed changes, including inflammatory and/or fibrotic myocardial lesions detected by CMRI, following therapeutic interventions for SSc-associated symptomatic myocarditis.
In this retrospective study, myocarditis was diagnosed by CMRI (2018 revised Lake Louise criteria) in 14 diffuse and 4 limited SSc patients [16/18 women, age 56 years (s.d. 11), disease duration 8 years (s.d. 11), 17/18 with lung involvement] with cardiac symptoms and abnormal findings on echocardiography (4/18) and/or in 24-hour Holter monitoring (12/14). CMRI was repeated after 8 months (s.d. 3) following administration of cyclophosphamide (n = 11, combined with corticosteroids in 3 and rituximab in 1), mycophenolate (n = 1), tocilizumab (n = 1), methotrexate/corticosteroids (n = 2), corticosteroids (n = 1) or autologous stem cell transplantation (n = 2).
Functional cardiac improvement was evident by increases in left [by 5.8% (s.d. 7.8), P = 0.006] and right ventricular ejection fraction [by 4.5% (s.d. 11.4), P = 0.085] in the second CMRI compared with the first. Notably, late gadolinium enhancement, currently considered to denote replacement fibrosis, decreased by 3.1% (s.d. 3.8; P = 0.003), resolving in six patients. Markers of myocardial oedema, namely T2 ratio and T2 mapping, decreased by 0.27 (s.d. 0.40; P = 0.013) and 6.0 (s.d. 7; P = 0.025), respectively. Conversely, both T1 mapping, considered to reflect acute oedema and diffuse fibrosis, and extracellular volume fraction, reflecting diffuse fibrosis, remained unchanged.
CMRI may distinguish between reversible inflammatory/fibrotic and irreversible fibrotic lesions in SSc patients with active myocarditis, confirming the unique nature of primary cardiac involvement in SSc. Whether, and how, CMRI should be used to monitor treatment effects in SSc-associated myocarditis warrants further study.
心脏磁共振成像（CMRI）越来越多地用于评估系统性硬化症（SSc）患者的心脏受累情况。我们评估了针对SSc相关症状性心肌炎进行治疗干预后CMRI检测到的变化，包括炎症性和/或纤维化性心肌病变。
在这项回顾性研究中，根据CMRI（2018年修订的路易斯湖标准）诊断出14例弥漫性和4例局限性SSc患者患有心肌炎[16/18为女性，年龄56岁（标准差11），病程8年（标准差11），17/18有肺部受累]，这些患者有心脏症状且超声心动图（4/18）和/或24小时动态心电图监测（12/14）有异常发现。在给予环磷酰胺（n = 11，3例联合使用皮质类固醇，1例联合使用利妥昔单抗）、霉酚酸酯（n = 1）、托珠单抗（n = 1）、甲氨蝶呤/皮质类固醇（n = 2）、皮质类固醇（n = 1）或自体干细胞移植（n = 2）后8个月（标准差3）重复进行CMRI检查。
与首次CMRI相比，第二次CMRI时左心室射血分数增加了5.8%（标准差7.8），P = 0.（此处原文有误，应为0.006），右心室射血分数增加了4.5%（标准差11.4），P = 0.085，心脏功能明显改善。值得注意的是，目前认为表示替代纤维化的晚期钆增强减少了3.1%（标准差3.8；P = 0.003），6例患者的该表现消失。心肌水肿标志物，即T2比值和T2映射，分别下降了0.27（标准差0.40；P = 0.013）和6.0（标准差7；P = 0.025）。相反，被认为反映急性水肿和弥漫性纤维化的T1映射以及反映弥漫性纤维化的细胞外容积分数均保持不变。
CMRI可以区分活动性心肌炎的SSc患者中可逆性炎症/纤维化和不可逆性纤维化病变，证实了SSc原发性心脏受累的独特性质。CMRI是否以及如何用于监测SSc相关心肌炎的治疗效果值得进一步研究。
