Pediatric Echocardiography Laboratory, Instituto da Criança e do Adolescente do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Clin Rheumatol. 2021 Apr;40(4):1497-1505. doi: 10.1007/s10067-020-05418-4. Epub 2020 Sep 28.
This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated.
Thirty-five consecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients' conditions.
Patients and controls had similar age (12.6 ± 0.7 vs.12.5 ± 0.6; p = 0.97) and gender (11F:24M vs.11F:24M; p = 1.0). Median of JDM duration was 4.6 (0.04-17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (≥ 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [-22(-17.2 to -27.9) % vs. -23(-20.8 to -27.4) %; p = 0.028)] and circumferential -23.9 ± 2.8% vs. -26.7 ± 2.9%; p = 0.0002) strain. Lower longitudinal strain was associated with DAS >3 -19.9(-17.2 to -26.5)% vs. -22.1-18.9 to -27.9)%; p = 0.046], MDI extent > 0 [-19(-17.2 to -22.5)% vs. -22.1-19.2 to -27.9)%; p = 0.0008], MDI severity > 0 [-19(-17.2 to -22.1)% vs. -22.3(-20.3 to -27.9)%; p = 0.0001] and calcinosis[-20.6(-17.2 to -23)% vs. -22.3(-20.3 to -27.9)%; p = 0.03]. Lower circumferential strain was associated with MDI extent > 0 (-22.1 ± 3.87% vs. -24.4 ± 2.3%; p = 0.039), MDI severity > 0 (-21.7 ± 3% vs. 24.7 ± 2.3%; p = 0.004) and calcinosis (-22.5 ± 3.3% vs. -24.8 ± 2.1%; p = 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r = -0.44; p = 0.009) and methotrexate (r = -0.33; p = 0.0008).
LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage.
• Serious cardiac involvement is rare but has been associated with death in juvenile dermatomyositis. • Two-dimensional speckle tracking stands out for the identification of subclinical myocardial compromise in juvenile dermatomyositis. • Longitudinal strain impairment is associated with disease activity and cumulative damage, whereas circumferential strain impairment is associated exclusively with cumulative damage.
本研究旨在使用二维斑点追踪超声心动图（2DST）研究青少年皮肌炎（JDM）患者的亚临床左心室（LV）收缩功能障碍。还评估了 LV 变形损伤与疾病活动/累积损伤之间的可能关联。
纳入了 35 名无心脏症状的连续 JDM 患者和 35 名健康志愿者。从病历中收集临床数据，并由一名儿科心脏病专家进行超声心动图检查，而该专家并不知道患者的情况。
患者和对照组的年龄（12.6 ± 0.7 岁比 12.5 ± 0.6 岁；p = 0.97）和性别（11 名女性：24 名男性比 11 名女性：24 名男性；p = 1.0）相似。JDM 病程的中位数为 4.6（0.04-17.6）年，只有 6/35（17%）患者存在活动期疾病（疾病活动评分（DAS）>3）。常规超声心动图显示所有个体的左心室射血分数（EF）均正常（≥55%）。在 JDM 中，2DST 发现 LV 纵向应变[-22（-17.2 至-27.9）%比-23（-20.8 至-27.4）%；p = 0.028]和圆周应变[-23.9 ± 2.8%比-26.7 ± 2.9%；p = 0.0002]减少。纵向应变降低与 DAS >3[19.9（-17.2 至-26.5）%比-22.1-18.9 至-27.9）%；p = 0.046]、MDI 程度>0[19（-17.2 至-22.5）%比-22.1-19.2 至-27.9）%；p = 0.0008]、MDI 严重程度>0[19（-17.2 至-22.1）%比-22.3（-20.3 至-27.9）%；p = 0.0001]和钙沉着[20.6（-17.2 至-23）%比-22.3（-20.3 至-27.9）%；p = 0.03]相关。圆周应变降低与 MDI 程度>0[-22.1 ± 3.87%比-24.4 ± 2.3%；p = 0.039]、MDI 严重程度>0[-21.7 ± 3%比 24.7 ± 2.3%；p = 0.004]和钙沉着[-22.5 ± 3.3%比-24.8 ± 2.1%；p = 0.02]相关。纵向应变与累积泼尼松剂量（r = -0.44；p = 0.009）和甲氨蝶呤（r = -0.33；p = 0.0008）呈负相关。
在无症状的儿科 JDM 患者中，2DST 检测到早期收缩性心肌损伤，EF 正常。纵向应变损伤与疾病活动和累积损伤相关，而圆周应变损伤仅与累积损伤相关。
• 严重的心脏受累虽少见，但与青少年皮肌炎的死亡有关。• 二维斑点追踪技术在青少年皮肌炎的亚临床心肌损伤的识别中表现出色。• 纵向应变损伤与疾病活动和累积损伤相关，而圆周应变损伤仅与累积损伤相关。
