258. Cardiac involvement in EGPA: interval CMR Imaging to monitor response to treatment
Creators: name: Mukunthan Srikantharajah1
name: Prendecki1, Maria
name: Ward1, Katie
name: Cairns1, Tom
name: Cole1, Graham
name: Mcadoo1, Stephen
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Cardiac involvement in EGPA is common and associated with both early and late morbidity and mortality. However, optimum approaches to diagnosis, treatment and follow up are unclear. <strong>Methods:</strong> Retrospective review of patients with myocardial involvement in EGPA treated with steroids, rituximab and cyclophosphamide, who underwent interval Cardiac MR imaging to monitor response. Data are presented as median +IQR unless stated otherwise. <strong>Results: </strong><em>Clinical Presentation: </em>Six patients (3 male; age 54 years [46-63]) presented between 2016-20. All had history of asthma, and 4 had prior diagnosis of sinusitis/ nasal polyposis. At EGPA diagnosis, peak eosinophil count was 9.9 x10<sup>9</sup>/L [5.3-17.0] and all tested patients had negative cytogenetics / molecular diagnostics to exclude haematologic hypereosinophilic syndromes. Median CRP and ESR were 157 mg/L [100-223] and 70 mm/hr [27-79], respectively, and 3 patients were MPO-ANCA positive (median titre 134 iu/mL). The proportion of patients affected by active/new ENT, lung, cutaneous and neurological disease at diagnosis were 4/6, 5/6, 3/6 and 3/6, respectively. All patients had some evidence of kidney involvement (serum creatinine 117 µmol/L [86-191], urinary protein 89 mg/mmol [58-116]). Three patients underwent kidney biopsy, confirming a pauci-immune glomerulonephritis.All patients had cardiac disease presenting with reduced exercise tolerance (4/6), oedema (3/6) and/or chest pain (1/6). ECG changes were present in 4/6 and abnormal echocardiographic findings were present in 4/6. All had elevated cardiac troponin concentrations (peak 10,762 ng/L [399-64,102) and CMR evidence of myocarditis (see below). <em>Treatment &amp; Outcome: </em>Patients were followed for an average of 30 months [14-69]. All received treatment with high-dose steroids, iv cyclophosphamide and rituximab, with azathioprine maintenance. By 3 months all achieved clinical disease remission with improvements in laboratory parameters: median eosinophil count 0.1 x10<sup>9</sup>/L , CRP 0.75 mg/L, sCr 106 µmol/L, uPCR 16.5 mg/mmol, and MPO-ANCA (median titre 4.9 iu/mL). Serum troponin normalised in all patients. At 12 months, all patients were in sustained remission. <em>CMR findings: </em>All patients had baseline and interval CMR imaging (Figure), with 1st follow up scan performed at 3.5 months [3.2. - 3.7]. Average LV function at baseline was 47 EF units (range 25-61 EF units) and at follow-up was 56 EF units (range 36-65). The average change in LV function with treatment was +9 EF units. All cases had evidence of oedema on the initial scan, and this improved in all cases (though remained borderline abnormal in all cases when quantified by T2 mapping). 5/6 cases had late gadolinium enhancement on their scan at presentation and this persisted in every case though with significantly lower burden. Apical thrombus was present in 3/6 cases at baseline but persisted in only 1/6 at follow-up. <strong>Conclusions:</strong> Cardiac MRI identifies a range of phenotypes in EGPA including different scar patterns, the presence of apical thrombus, and ongoing disease activity. Although response to treatment is often significant, residual myocardial scarring (and low grade oedema) is common. Further work will elucidate the implications of this convalescent phenotype on disease course and prognosis, and the optimum treatment for cardiac disease in EGPA. <strong>Disclosures:</strong> The authors have no relevant financial disclosures