Srikantharajah Mukunthan, Gopalan Deepa, Wilson-Morkeh Harold, Siddiqui Salman, McAdoo Stephen P, Youngstein Taryn
Vasculitis Centre, Department of Immunology and Inflammation, Imperial College London, London, UK.
Department of Renal Medicine, Hammersmith Hospital, London, UK.
Curr Cardiol Rep. 2025 Jul 9;27(1):109. doi: 10.1007/s11886-025-02258-z.
This review highlights recent advances in the pathophysiology, diagnosis, and treatment of cardiac disease in patients with Eosinophilic granulomatosis with polyangiitis (EGPA). We outline a diagnostic approach to facilitate early identification of affected patients.
Recent advancements in diagnostic techniques including cardiac magnetic resonance (CMR) have improved recognition of cardiac disease in patients with EGPA. CMR has demonstrated a high prevalence of cardiac abnormalities, even in the absence of clinical symptoms, electrocardiographic or echocardiographic findings. Cardiac disease is a major cause of mortality in patients with EGPA, accounting for around 50% of disease-related deaths. However, due to the lack of standardised screening and diagnostic criteria, the true incidence remains unknown. Systemic immunosuppressive therapy is warranted to prevent acute complications as well as mitigate the long-term impact of chronic inflammation and tissue damage. Given the challenges in early detection and the prognostic significance of cardiac involvement, we recommend including CMR in the baseline evaluation of all EGPA patients at diagnosis.
本综述重点介绍嗜酸性肉芽肿性多血管炎(EGPA)患者心脏病的病理生理学、诊断和治疗方面的最新进展。我们概述了一种诊断方法,以促进对受影响患者的早期识别。
包括心脏磁共振成像(CMR)在内的诊断技术的最新进展提高了对EGPA患者心脏病的识别。CMR已显示即使在没有临床症状、心电图或超声心动图表现的情况下,心脏异常的发生率也很高。心脏病是EGPA患者死亡的主要原因,约占疾病相关死亡的50%。然而,由于缺乏标准化的筛查和诊断标准,真实发病率仍然未知。全身性免疫抑制治疗对于预防急性并发症以及减轻慢性炎症和组织损伤的长期影响是必要的。鉴于早期检测存在挑战以及心脏受累的预后意义,我们建议在所有EGPA患者诊断时的基线评估中纳入CMR。