Highlights
- •In our small population, patients with EGPA in clinical remission showed significant cardiovascular burden.
- •CMR showed signs of inflammation and the presence of myocardial fibrosis.
- •A ventricular thrombus was detected in about 25% of our patients.
Abstract
Background
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis
characterized by hypereosinophilia. EGPA typically develops in three clinical phases,
beginning with asthma, followed by tissue eosinophilia and finally systemic vasculitis.
Cardiac involvement is the most important predictor of mortality; it occurs in approximately
15–60% of EGPA patients, a significant proportion of whom are asymptomatic and have
normal electrocardiogram (ECG) and echocardiogram. Early detection and management
of cardiac disease could positevely affect prognosis. Cardiovascular magnetic resonance
(CMR) has emerged as the gold standard cardiac imaging technique in the evaluation
of cardiomyopathies, due to its ability to reliably assess anatomy, function, and
tissue characterization.
Aim
Purpose of this study was to assess the role of CMR in detecting cardiac disease in
patients with EGPA in clinical remission.
Methods
A dedicated CMR protocol including functional analysis, and pre and post-contrast
tissue characterization was performed in 11 patients with EGPA and the results were
compared with 11 healthy subjects.
Results
EGPA patients had lower left ventricular ejection fraction compared to controls (56 ± 19 vs 68.7 ± 5.2, p value 0.02). Late gadolinium enhancement (LGE), representing replacement fibrosis,
was positive in 9/11 (82%) patients, mainly with a non-ischemic pattern. In 3/11 (27%)
patients a left ventricular thrombus was detected; in 3/11 (27%) patients myocardial
edema was detected. CMR parameters of interstitial fibrosis were significantly more
elevated in EGPA patients compared to controls.
Conclusions
Patients with EGPA in clinical remission showed a high cardiovascular burden as demonstrated
by lower EF, signs of active inflammation, presence of interstitial and replacement
fibrosis and intraventricular thrombosis. Further studies on wider populations are
warranted to better understand how these findings could impact on prognosis and eventually
guide therapy.
Keywords
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Article info
Publication history
Published online: October 07, 2016
Accepted:
September 14,
2016
Received in revised form:
September 8,
2016
Received:
April 27,
2016
Identification
Copyright
© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
