Matsumoto Kenji, Ehara Shoichi, Sakaguchi Mikumo, Otsuka Kenichiro, Hasegawa Takao, Shimada Kenei, Yoshiyama Minoru
Osaka City Med J. 2015 Jun;61(1):9-17.
The presence of cardiac involvement in sarcoidosis, a multisystem granulomatous disease of unknown etiology, is associated with very poor prognosis. Therefore, early detection of cardiac sarcoidosis (C-sar) is very important for effective treatment. Recently, the value of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR), which allows visualization of even minute amounts of myocardial damage, has been emphasized in diagnosing C-sar. Although the presence of LGE has been added as a minor criterion for diagnosing C-sar in the most recent Japan Ministry of Health and Welfare (JMHW) guidelines, its clinical utility remains unknown.
A total of 17 patients with biopsy-proven extracardiac sarcoidosis, who underwent CMR and examinations using a sufficient number of modalities to diagnose or exclude C-sar in accordance with the JMHW diagnostic criteria, were retrospectively enrolled in this study. Among these 17 patients, 7 patients were diagnosed with C-sar and 10 with non-C-sar. We investigated the clinical performance of LGE in the detection of cardiac involvement, compared the distribution of LGE with perfusion defects of iodine-123-labeled 15-(p-iodophenyl)-3R,S-methyl pentadecanoic acid (BMIPP) or thallium-201 (201Tl) scintigraphy, and evaluated the prognostic value of LGE on CMR in identifying adverse clinical events.
All patients diagnosed with C-sar were positive for LGE and all diagnosed with non-C-sar patients were negative. Evaluation of LGE revealed a broader range of abnormalities than the evaluation of the defects shown by either BMIPP or 201Tl scintigraphy. All adverse events occurred in the C-sar patients with LGE.
This study showed that LGE is useful for the detection of cardiac involvement, and it might be a promising tool for determining the prognosis of patients with biopsy-proven extracardiac sarcoidosis.
结节病是一种病因不明的多系统肉芽肿性疾病，心脏受累与预后极差相关。因此，早期发现心脏结节病（C-sar）对有效治疗非常重要。近年来，心脏磁共振成像（CMR）中的延迟钆增强（LGE）在诊断C-sar方面的价值得到了重视，它能够显示即使是微量的心肌损伤。尽管在日本厚生劳动省（JMHW）最新指南中，LGE的存在已被列为诊断C-sar的次要标准，但其临床实用性仍不明确。
本研究回顾性纳入了17例经活检证实有心脏外结节病的患者，这些患者接受了CMR检查，并根据JMHW诊断标准使用了足够数量的检查方法来诊断或排除C-sar。在这17例患者中，7例被诊断为C-sar，10例为非C-sar。我们研究了LGE在检测心脏受累方面的临床表现，比较了LGE的分布与碘-123标记的15-（对碘苯基）-3R，S-甲基十五烷酸（BMIPP）或铊-201（201Tl）闪烁显像的灌注缺损情况，并评估了CMR上LGE对识别不良临床事件的预后价值。
所有诊断为C-sar的患者LGE均为阳性，所有诊断为非C-sar的患者LGE均为阴性。与BMIPP或201Tl闪烁显像显示的缺损评估相比，LGE评估显示出更广泛的异常。所有不良事件均发生在有LGE的C-sar患者中。
本研究表明，LGE对检测心脏受累有用，它可能是确定经活检证实有心脏外结节病患者预后的一个有前景的工具。
