Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan.
Vita-Salute San Raffaele University, Milan.
Rheumatology (Oxford). 2020 Sep 1;59(9):2523-2533. doi: 10.1093/rheumatology/kez658.
To outline the clinical, histological and prognostic features of systemic sclerosis (SSc) endomyocardial biopsy-proven myocarditis with respect to those of diverse endomyocardial biopsy-proven virus-negative myocarditis (VNM).
We retrospectively analysed data from three cohorts of endomyocardial biopsy-proven myocarditis: SSc-related VNM (SSc-VNM); isolated VNM (i-VNM); and VNM related to other systemic autoimmune diseases (a-VNM). The degree of myocardial fibrosis was expressed as relative percentage and fibrotic score (0-3). Clinical data, cardiac enzymes, echocardiogram, 24 h ECG Holter and cardiac magnetic resonance were obtained at baseline and during follow-up. Non-parametric tests were used.
We enrolled 12 SSc-VNM [11 females, mean age 49.3 (14.2) years; seven diffuse-SSc, five early-SSc], 12 i-VNM [12 females, mean age 47.7 (10.8) years] and 10 a-VNM [four females, mean age 48.4 (16.3) years] patients. SSc patients had higher degrees of myocardial fibrosis as assessed by both percentage [SSc-VNM: 44.8 (18.8)%; a-VNM: 28.6 (16.5)%; i-VNM: 24.9 (10.3)%; P = 0.019] and score [SSc-VNM: 2.3 (0.8); a-VNM: 1.4 (1.1); i-VNM: 1.2 (0.7); P = 0.002]. Myocardial fibrosis directly correlated with skin score (r = 0.625, P = 0.03) and number of ventricular ectopic beats on 24 h ECG Holter in SSc patients (r = 0.756, P = 0.01). Dyspnoea class was higher at presentation in SSc-VNM patients (P = 0.041) and we found heart failure only in SSc patients (25%) (P = 0.05). At cardiac magnetic resonance, myocardial oedema was nearly undetectable in SSc-VNM patients compared with others (P = 0.02). All patients received immunosuppressive treatment. The number of patients who died during follow-up due to cardiac complications was significantly higher in SSc-VNM patients (50%), as compared with a-VNM (0%) and i-VNM (8.3%) patients (P = 0.006). Patients who died during follow-up had higher degrees of myocardial fibrosis [52.2 (11.6)% vs 27.5 (12.9)%, P = 0.024; fibrotic score: 2.83 (0.41) vs 1.4 (0.9), P < 0.001].
SSc has unique clinical and histological features, as it tends to present more frequently with heart failure and a higher dyspnoea class and to show higher degrees of myocardial fibrosis. These specific features are paralleled by a worse cardiac prognosis.
概述系统性硬化症（SSc）心肌活检证实心肌炎的临床、组织学和预后特征，以及不同心肌活检证实病毒阴性心肌炎（VNM）的特征。
我们回顾性分析了三个心肌活检证实心肌炎队列的数据：SSc 相关 VNM（SSc-VNM）；孤立性 VNM（i-VNM）；以及与其他系统性自身免疫性疾病相关的 VNM（a-VNM）。心肌纤维化程度用相对百分比和纤维化评分（0-3）表示。基线和随访时获取临床数据、心脏酶、超声心动图、24 小时心电图 Holter 和心脏磁共振。采用非参数检验。
我们纳入了 12 例 SSc-VNM [11 例女性，平均年龄 49.3（14.2）岁；7 例弥漫性 SSc，5 例早期 SSc]、12 例 i-VNM [12 例女性，平均年龄 47.7（10.8）岁]和 10 例 a-VNM [4 例女性，平均年龄 48.4（16.3）岁]患者。通过百分比和评分评估，SSc 患者的心肌纤维化程度更高[SSc-VNM：44.8（18.8）%；a-VNM：28.6（16.5）%；i-VNM：24.9（10.3）%；P=0.019]和评分[SSc-VNM：2.3（0.8）；a-VNM：1.4（1.1）；i-VNM：1.2（0.7）；P=0.002]。在 SSc 患者中，心肌纤维化与皮肤评分（r=0.625，P=0.03）和 24 小时心电图 Holter 室性异位搏动数（r=0.756，P=0.01）直接相关。在 SSc-VNM 患者中，呼吸困难的发生率更高（P=0.041），并且我们仅在 SSc 患者中发现心力衰竭（25%）（P=0.05）。与其他患者相比，在心脏磁共振上，SSc-VNM 患者的心肌水肿几乎不可检测（P=0.02）。所有患者均接受免疫抑制治疗。在随访期间，由于心脏并发症而死亡的患者中，SSc-VNM 患者（50%）明显高于 a-VNM 患者（0%）和 i-VNM 患者（8.3%）（P=0.006）。在随访期间死亡的患者的心肌纤维化程度更高[52.2（11.6）%比 27.5（12.9）%，P=0.024；纤维化评分：2.83（0.41）比 1.4（0.9），P<0.001]。
SSc 具有独特的临床和组织学特征，其心力衰竭发生率更高，呼吸困难程度更高，且心肌纤维化程度更高。这些特定特征与更差的心脏预后相关。
