Mavrogeni Sophie, Karabela Georgia, Gialafos Elias, Stavropoulos Efthymios, Spiliotis George, Katsifis Gikas, Kolovou Genovefa
50 Esperou Street, 175-61 P. Faliro, Athens, Greece.
Inflamm Allergy Drug Targets. 2013 Oct;12(5):322-7. doi: 10.2174/18715281113129990054.
The cardiovascular magnetic resonance (CMR) pattern of Churg-Strauss syndrome (CSS) includes myopericarditis, diffuse subendocardial vasculitis or myocardial infarction with or without cardiac symptoms and is usually associated with lack of antineutrophil cytoplasmic antibodies (ANCA).
To correlate the CMR pattern with ANCA in CSS, compare it with healthy controls and systemic lupus erythematosus (SLE) patients and re-evaluate 2 yrs after the first CMR.
PATIENTS-METHODS: 28 consecutive CSS, aged 42±7 yrs, were referred for CMR and 2 yrs re-evaluation. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) imaging. Their results were compared with 28 systemic lupus erythematosus (SLE) under remission and 28 controls with normal myocardial perfusion, assessed by scintigraphy.
CMR revealed acute cardiac lesions in all ANCA (-) CSS with active disease and acute cardiac symptoms and only in one asymptomatic ANCA (+) CSS, with active disease. Diffuse subendocardial fibrosis (DSF) or past myocarditis was identified in both ANCA(+) and ANCA (-) CSS, but with higher incidence and fibrosis amount in ANCA (-) CSS (p<0.05). In comparison to SLE, both ANCA (+) and ANCA (-) CSS had higher incidence of DSF, lower incidence of myocarditis and no evidence of myocardial infarction, due to coronary artery disease (p<0.05). In 2 yrs CMR follow up, 1/3 of CSS with DSF presented LV function deterioration and one died, although immunosuppressive treatment was given early after CSS diagnosis.
Cardiac involvement either as DSF or myocarditis, can be detected in both ANCA (+) and ANCA (-) CSS, although more clinically overt in ANCA (-). DSF carries an ominous prognosis for LV function. CMR, due to its capability to detect disease severity, before cardiac dysfunction takes place, is an excellent tool for CSS risk stratification and treatment individualization.
变应性肉芽肿性血管炎（CSS）的心血管磁共振（CMR）表现包括心肌心包炎、弥漫性心内膜下血管炎或心肌梗死，伴有或不伴有心脏症状，且通常与抗中性粒细胞胞浆抗体（ANCA）缺乏相关。
将CSS患者的CMR表现与ANCA进行相关性分析，与健康对照者和系统性红斑狼疮（SLE）患者进行比较，并在首次CMR检查后2年进行重新评估。
连续纳入28例年龄为42±7岁的CSS患者，进行CMR检查及2年的随访评估。CMR检查包括左心室射血分数（LVEF）、T2加权成像（T2-W）、早期钆增强成像（EGE）和延迟钆增强成像（LGE）。将他们的结果与28例病情缓解的系统性红斑狼疮（SLE）患者以及28例心肌灌注正常的对照者（通过闪烁扫描评估）进行比较。
CMR显示，所有患有活动性疾病且有急性心脏症状的ANCA阴性CSS患者均有急性心脏病变，仅有1例无症状的ANCA阳性CSS患者（患有活动性疾病）出现急性心脏病变。在ANCA阳性和阴性的CSS患者中均发现了弥漫性心内膜下纤维化（DSF）或既往心肌炎，但ANCA阴性CSS患者的发生率和纤维化程度更高（p<0.05）。与SLE相比，ANCA阳性和阴性的CSS患者DSF发生率均较高，心肌炎发生率较低，且无冠状动脉疾病导致的心肌梗死证据（p<0.05）。在2年的CMR随访中，尽管在CSS诊断后早期给予了免疫抑制治疗，但1/3患有DSF的CSS患者出现了左心室功能恶化，1例患者死亡。
在ANCA阳性和阴性的CSS患者中均能检测到心脏受累，表现为DSF或心肌炎，尽管ANCA阴性患者的临床表现更为明显。DSF对左心室功能预后不佳。由于CMR能够在心脏功能障碍发生之前检测到疾病的严重程度，因此是CSS风险分层和个体化治疗的优秀工具。
