Successful Treatment with Mepolizumab for Coronary Spastic Angina Associated with Eosinophilic Granulomatosis with Polyangiitis

Abstract

A 46-year-old man with a history of bronchial asthma and chronic sinusitis presented to our hospital with chest pain. We suspected angina evoked by epicardial coronary spasm and performed an ergonovine provocation test to diagnose coronary spastic angina (CSA). The patient also met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis (EGPA) and was treated with 60 mg prednisolone (PSL) for EGPA-associated CSA. After PSL administration, eosinophils decreased, and angina attacks disappeared. However, when PSL was tapered to 12.5 mg, chest pain recurred. We administered mepolizumab subcutaneously and chest pain disappeared. Additional mepolizumab may be effective for EGPA with CSA.

Keywords: EGPA; bronchial asthma; coronary spastic angina; eosinophilia; mepolizumab.

Figure 1.

Twelve-lead electrocardiogram (ECG) findings during chest pain attack. The ECG showed ST-segment elevation in aVR and ST-segment depression in I, II, aVL, and aVF.

Figure 2.

Imaging studies on admission. A: Computed tomography (CT) of the paranasal sinus showed sinusitis (arrows). B: Chest CT on admission showed reticular shadows around broncho-vascular bundles in the bilateral lower lobes and bronchial wall thickening (arrows).

Figure 3.

A coronary angiogram obtained during the ergonovine provocation test (A: control, B: ergnovine). A: Before the ergonovine provocation test of coronary artery spasm. No significant stenosis in the right coronary artery. B: After intra-coronary infusion of ergonovine (20 μg), a severe coronary artery spasm was provoked in the right coronary artery.

Figure 4.

The clinical course after late March 2020. The patient noticed persistent chest pain attacks for 10-15 minutes every day in early April 2020. Marked elevation of eosinophil count was observed, and the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) with coronary spastic angina was made. He was started on high-dose prednisolone (PSL) therapy of 60 mg (1 mg/kg) and chest pains improved. Following PSL administration, the eosinophil count markedly decreased, and angina attacks disappeared. When PSL was tapered to 12.5 mg, he occasionally experienced mild chest pain with slight progression of eosinophilia. The administration of 300 mg mepolizumab kept the eosinophils count under 100 cells/μL. Two years after the onset of EGPA, PSL was tapered to 6 mg, but there was no recurrence of chest pain or discomfort.