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Cardiac sarcoidosis: state-of-the-art review
Heart ( IF 5.1 ) Pub Date : 2021-10-01 , DOI: 10.1136/heartjnl-2019-316442
Vasileios Kouranos 1, 2 , Rakesh Sharma 3, 4
Affiliation  

### Learning objectives Sarcoidosis is a systemic inflammatory disease characterised histologically by the formation of non-caseating granuloma in multiple organs.1 The cause of the disease remains unknown. It is hypothesised that exposure to an antigen in patients with a genetic predisposition results in an exaggerated immune response leading to granuloma formation. Clinicians face several challenges including achieving a confident diagnosis and providing patients with a long-term management plan. These challenges largely relate to the heterogeneity of the original presentation, disease evolution and outcome. Sarcoidosis may be diagnosed incidentally in patients who have no symptoms, those with non-specific respiratory symptoms such as a persistent cough or those with prominent systemic features (fever, weight loss and fatigue). Moreover, in many patients, the condition will remit spontaneously, whereas in others long-term immunomodulation is required to prevent disease progression. While the lungs and intrathoracic lymph nodes are involved in 90%–95% of cases, the exact prevalence of cardiac sarcoidosis (CS) remains largely unknown.2 Clinically overt CS has been reported in 5%–10% of cases with systemic sarcoidosis.2 However, the true prevalence is likely to be higher based on postmortem analyses and registries using advanced imaging modalities.3–12 Patients with CS may present with arrhythmias (such as advanced atrioventricular block or ventricular tachycardia) and/or unexplained new onset heart failure without a history of systemic sarcoidosis.13 14 The initial investigation of such cases should focus on identifying extracardiac disease to improve the certainty of the diagnosis. Histologically confirmed isolated CS has also been reported.7 Therefore, the true burden of CS …

中文翻译:

心脏结节病:最先进的审查

### 学习目标 结节病是一种全身性炎症性疾病,其组织学特征是在多个器官中形成非干酪性肉芽肿。1 该病的病因尚不清楚。假设具有遗传易感性的患者暴露于抗原会导致过度的免疫反应,从而导致肉芽肿的形成。临床医生面临着几个挑战,包括获得自信的诊断和为患者提供长期管理计划。这些挑战主要与原始表现、疾病演变和结果的异质性有关。结节病可能在没有症状的患者、有非特异性呼吸道症状(如持续咳嗽)或有明显全身特征(发烧、体重减轻和疲劳)的患者中被偶然诊断出来。而且,在许多患者中,病情会自发缓解,而在其他患者中,需要长期免疫调节以防止疾病进展。虽然肺和胸内淋巴结在 90%–95% 的病例中受累,但心脏结节病 (CS) 的确切患病率在很大程度上仍然未知。2 据报道,5%–10% 的系统性结节病病例有临床明显的 CS。 2 然而,根据尸检分析和使用先进成像方式的登记,真实患病率可能更高。3-12 CS 患者可能出现心律失常(如晚期房室传导阻滞或室性心动过速)和/或不明原因的新发心力衰竭没有系统性结节病病史。13 14 对此类病例的初步调查应侧重于识别心外疾病,以提高诊断的确定性。
更新日期:2021-09-14
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