Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune systemic necrotizing vasculitis of blood vessels that often presents with hypereosinophilia. Cardiac involvement in EGPA directly correlates with the mortality of patients with the disease and is a central part of the disease process. The evaluation and treatment of cardiac anomalies are vital in patients with EGPA. The frequency with which cardiac involvement is seen in the disease process makes early diagnosis crucial in all patients with EGPA. Early treatment has been proven to reverse or cause the disease to go into remission. Several studies have shown that cardiac magnetic resonance (CMR) imaging is the most sensitive and best early indicator of cardiovascular involvement in EGPA. CMR routinely outperforms other diagnostic techniques such as ECG (echocardiography) and CTA (computed tomography angiography) in the detection of cardiac anomalies and should be a part of the standardized assessment of all patients with EGPA. CMR is also a non-invasive diagnostic tool that can also outperform biopsy in the detection of EGPA cardiac involvement. CMR is also a valuable technique that can be used to monitor disease progression while treatment is being performed. Although long-term research studies have yet to show these benefits, the studies that are available today provide ample evidence that shows CMR imaging could ultimately help bring down mortality rates currently seen in EGPA patients if it is used as an evaluation tool from initial diagnosis and throughout the entire course of disease management.

Highlights

  • BackgroundEosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Straus syndrome, is an autoimmune systemic necrotizing vasculitis of small- to medium-sized blood vessels that presents with hypereosinophilia [1,2,3]

  • Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune systemic necrotizing vasculitis of blood vessels that often presents with hypereosinophilia

  • This study aims to demonstrate the importance of extensive cardiac imaging, primarily cardiac magnetic resonance (CMR), as a central part of the routine diagnostic workup and evaluation of the progression of cardiac anomalies in EGPA patients

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Summary

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Straus syndrome, is an autoimmune systemic necrotizing vasculitis of small- to medium-sized blood vessels that presents with hypereosinophilia [1,2,3]. Evaluating patients with ECG helps systematically screen for heart failure, neglecting the early involvement of the cardiovascular system, which is often detected by CMR. The evaluation of the cardiovascular system in EGPA patients involves the use of many imaging techniques including ECG, computed tomography angiography (CTA), and CMR [23]. The use of CMR has proven to be the most effective imaging technique and diagnostic tool for evaluating cardiovascular disease in EGPA patients. While CMR has established itself as a reliable diagnostic indicator of cardiac involvement, it can be used to provide snapshots over a period of time to help monitor cardiovascular disease progression or remission. A study by Fijolek et al showed that 10 of 12 patients in clinical remission after undergoing treatment for EGPA still presented with signs of change on CMR imaging [14]. The use of CMR for detection and evaluation throughout the various stages of disease has shown the versatility and reliability of CMR as a diagnostic tool

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Knockaert DC
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