Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder characterized by asthma, rhinitis, peripheral blood eosinophilia, and necrotizing vasculitis of small vessels. Cardiac involvement occurs in approximately 10-30% of patients with EGPA and has been identified as a major predictor of mortality. Cardiac manifestations can be diagnosed clinically, as well as through electrocardiogram and echocardiogram. Cardiac magnetic resonance (CMRI) has higher sensitivity to detect cardiac involvement, especially late gadolinium enhancement (LGE), which can indicate myocardial inflammation and/or fibrosis. However, the exact role of CMRI at diagnosis and the utility of repeat CMRI to monitor evolution of cardiac manifestations in EGPA remain unclear. Our aim was to report on the use and findings of CMRI in our EGPA patient cohort, determine the added value of repeat CMRI, and evaluate the outcome and prognostic value of persistent LGE.

Methods: In this monocentric retrospective descriptive cohort study, we included 75 patients diagnosed with EGPA who were seen at least twice between 2015 and January 2023, of which 30 patients had undergone CMRI, with 11 patients undergoing repeat CMRI during follow-up. All patients satisfied the 2022 ACR/EULAR Classification Criteria for EGPA.

Results: 75 patients (male 56%, mean age at diagnosis 46 years, 2022 ACR/EULAR score range 6-13) seen at least twice in the clinic between 2015 and January 2023 were included. Antineutrophil cytoplasmic antibody (ANCA) was positive in 29 patients (39%), with 22 (39%) being anti-myeloperoxidase antibody (anti-MPO) positive and 4 (5%) being perinuclear ANCA (p-ANCA) positive. 17 (23%) had cardiac symptoms, such as chest pain, orthopnea, and syncope. 30 patients underwent CMRI, 9 (30%) of whom had cardiac symptoms. 11 underwent one or more repeat CMRI. All 30 patients (100%) were treated with glucocorticoids, with 13 (43%) also receiving cyclophosphamide. 11/11 patients who underwent repeat CMRI had LGE on first CMRI, 10/11 had persistent LGE on second CMRI, 4/4 had LGE on third CMRI, and 2/2 had LGE on fourth CMRI. The average time between first CMRI and second CMRI was 22 months (range 4-81 months). Of the 30 patients, 3 (10%) had cardiovascular events at follow-up, 2 of whom had atrial fibrillation (neither had LGE on their CMRIs), and 1 of whom had coronary artery disease requiring stenting (with LGE on his 4 CMRIs).

Conclusion: The role of initial and further serial CMRIs to study and monitor cardiac involvement in EGPA remains unclear. Late gadolinium enhancement most often persists despite treatment, with no major differences in adverse cardiac outcomes in this cohort.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/utility-of-follow-up-cardiac-magnetic-resonance-imaging-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis/