Eosinophilic Myocarditis Demonstrated Using Cardiac Magnetic Resonance Imaging in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease)

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), historically known as the Churg-Strauss disease, is a small- to medium-sized vessel multi-organ vasculitis with a propensity to involve the heart. EGPA is a rare condition with an estimated annual incidence of one to 4.2 people per million. The cardiac involvement causes significant morbidity and mortality in EGPA patients. Approximately 50% of the deaths in EGPA are related to cardiac disease and occur within the first few months since diagnosis. The current recommendations support evaluation of cardiac involvement by using history, physical exam and multimodality imaging including echocardiogram and cardiac magnetic resonance imaging (CMR). Here, we report a rare case of eosinophilic myocarditis in a 19-year-old patient with EGPA seen on CMR. Pertinent literature is also reviewed. We highlighted the importance of CMR in diagnosing and follow up of EGPA patients.

The Critical Role of Cardiac Magnetic Resonance Imaging in Evaluating Patients With Eosinophilic Granulomatosis With Polyangiitis

“…The study conclusively demonstrated the strengths of CMR use as part of a new strategy to treat these patients before the progression of damage becomes insurmountable. Also, the patient in that study did not show any areas of stenosis, calcified plaque, or soft plaque upon evaluation with CTA [26]. The use of CMR has proven to be the most effective imaging technique and diagnostic tool for evaluating cardiovascular disease in EGPA patients.…”

“…The same study showed that CMR detected cardiac abnormalities with higher sensitivity than ECG (88% versus 82%) [25]. A study by Dalia et al also indicated CMR's capability when a patient with cardiovascular involvement in EGPA exhibited apical hypokinesis with an EF of 55% [26]. However, upon evaluation with CMR, there were several areas of delayed enhancement in the LV myocardium, and decreased perfusion in mid to apical septal and inferior segments through the cardiac apex [26].…”

“…A study by Dalia et al also indicated CMR's capability when a patient with cardiovascular involvement in EGPA exhibited apical hypokinesis with an EF of 55% [26]. However, upon evaluation with CMR, there were several areas of delayed enhancement in the LV myocardium, and decreased perfusion in mid to apical septal and inferior segments through the cardiac apex [26]. The study conclusively demonstrated the strengths of CMR use as part of a new strategy to treat these patients before the progression of damage becomes insurmountable.…”

“…ECG has reliably shown its ability to detect the presence of heart failure in EGPA, with its main limitation being that it shows damage after it has already been inflicted [20]. CMR has consistently outperformed ECG in the early detection of cardiovascular system involvement in EGPA [25,26]. A study by Dennert et al indicated that ECG showed cardiac abnormalities in 50% of EGPA patients, whereas the same group showed various abnormalities in the cardiovascular system when evaluated with CMR.…”

Myopericarditis as a presentation of eosinophilic granulomatosus with polyangiitis (EGPA)

“…A second group detailed a case of cardiac tamponade leading to a new diagnosis of EGPA, even rarer than myopericarditis, and indicative of increased severity of disease 15. In all cases, the increasingly recognised role for CMRI is highlighted7 16 although this does not form part of the official guidelines for the management of EGPA, and more extensive studies are certainly warranted for its use in this setting, especially given the paucity of data, mostly derived from individual case reports.…”