Myocardial involvement in eosinophilic granulomatosis with polyangiitis evaluated with cardiopulmonary magnetic resonance

Josephine H Naish,Jakub Lagan,Matthias Schmitt,Erik B Schelbert,Nazia Chaudhuri,David Clark,Christopher A Miller,Christien Fortune,Robert Niven,Joshua Bradley

doi:10.1007/s10554-020-02091-1

Abstract

Patients with eosinophilic granulomatosis with polyangiitis (EGPA) most commonly die from cardiac causes, however, cardiac involvement remains poorly characterised and the relationship between cardiac and pulmonary disease is not known. This study aimed to characterise myocardial and pulmonary manifestations of EGPA, and their relationship. Prospective comprehensive cardiopulmonary investigation, including a novel combined cardiopulmonary magnetic resonance imaging (MRI) technology, was performed in 13 patients with stable EGPA. Comparison was made with 11 prospectively recruited matched healthy volunteers. Stable EGPA was associated with focal replacement and diffuse interstitial myocardial fibrosis (myocardial extracellular volume 26.9% vs. 24.7%; p = 0.034), which drove a borderline increase in left ventricular mass (56 ± 9 g/m2 vs. 49 ± 8 g/m2; p = 0.065). Corrected QT interval was significantly prolonged and was associated with the severity of myocardial fibrosis (r = 0.582, p = 0.037). Stable EGPA was not associated with increased myocardial capillary permeability or myocardial oedema. Pulmonary tissue perfusion and capillary permeability were normal and there was no evidence of pulmonary tissue oedema or fibrosis. Forced expiratory volume in one second showed a strong inverse relationship with myocardial fibrosis (r = −0.783, p = 0.038). In this exploratory study, stable EGPA was associated with focal replacement and diffuse interstitial myocardial fibrosis, but no evidence of myocardial or pulmonary inflammation or pulmonary fibrosis. Myocardial fibrosis was strongly associated with airway obstruction and abnormal cardiac repolarisation. Further investigation is required to determine the mechanisms underlying the association between heart and lung disease in EGPA and whether an immediate immunosuppressive strategy could prevent myocardial fibrosis formation.

Highlights

Eosinophilic granulomatosis with polyangiitis (EGPA), for‐ merly known as Churg-Strauss syndrome, is a hybrid condi‐ tion comprising a systemic antineutrophil cytoplasmic anti‐ body (ANCA)-associated vasculitis and a hypereosinophilic disorder with frequent lung involvement that is associated with asthma [1, 2]
We show that stable EGPA is associated with focal replacement and diffuse interstitial myocardial fibrosis, which drives a borderline increase in left ventricular (LV) mass, but is not associated with myocardial oedema or increased capillary permeability
We show that stable EGPA is associated with myocardial fibrosis and an absence of myocardial inflammation, findings which are in keeping with the known histol‐ ogy, described earlier

Summary

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), for‐ merly known as Churg-Strauss syndrome, is a hybrid condi‐ tion comprising a systemic antineutrophil cytoplasmic anti‐ body (ANCA)-associated vasculitis and a hypereosinophilic disorder with frequent lung involvement that is associated with asthma [1, 2].Whilst a wide range of cardiac manifestations are described, including pericarditis, pericardial effusion, tam‐ ponade, myocarditis, cardiomyopathy, myocardial infarction and heart failure [2], clinically evident cardiac manifesta‐ tions are uncommon [3, 4]. Magnetic resonance imaging (MRI) provides unparalleled evaluation of cardiac structure, function and tissue charac‐ terisation and its application in EGPA has provided insight into cardiac disease expression [5, 8,9,10,11]. Contem‐ porary cardiac MRI techniques, which provide quantitative characterisation of myocardial injury and adaptation, have been applied sparsely and the relationship between cardiac and pulmonary disease is not known. We sought to apply a novel combined cardio‐ pulmonary MRI technology, which provides comprehensive and simultaneous interrogation of cardiac and pulmonary structure, function and tissue character, in patients with stable EGPA in order to: (1) Characterise myocardial and pulmonary manifestations of EGPA; and (2) Investigate the relationship between cardiac and pulmonary disease in EGPA

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