Researchers presented the case study of a patient within eosinophilic granulomatosis with polyangiitis (EGPA), a form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), who was successfully treated with mepolizumab for coronary spastic angina. 

The report was published in the journal Internal Medicine.

A 46-year-old man first presented in 2012 with frequent asthma attacks. He was prescribed 0.25 mg of oral betamethasone from then until 2020. Discontinuation of oral corticosteroids resulted in symptoms of nausea, abdominal pain, anorexia, and diarrhea. He previously was known to have a history of gastric ulcer.

A gastrointestinal endoscopy was carried out, showing eosinophilic esophagitis. One month later, he noted chest pain (lasting for approximately 10-15 minutes), progressive cough, shortness of breath, nasal congestion, and anosmia. The patient’s physicians suspected coronary spastic angina and he was started on benidipine hydrochloride and isosorbide dinitrate tape, which did not achieve adequate symptomatic control. 

Read more about AAV etiology 

He later experienced chest pain radiating to his left shoulder as well as nausea. An electrocardiogram (ECG) demonstrated ST-segment depression in I, II, aVL, and aVF, and ST elevation in aVR. Echocardiography revealed hypokinesis and asynergy in the inferior base apex. Chest computed tomography (CT) demonstrated a number of tiny lung infiltrates and bilateral lower lobe ground-glass opacities; these findings were suggestive of vasculitis processes. Coronary angiogram showed no evidence of coronary artery stenosis. 

The patient’s physicians became more convinced of the diagnosis of coronary spastic angina and loaded him with 20 μg of ergonovine to induce coronary artery spasm. An ergonovine provocation test induced severe coronary artery spasm in the right coronary artery. On ECG, this was associated with ST-segment elevation in II, III, and aVF, which improved after the patient was injected with 10 mg of isosorbide.

Laboratory investigations revealed raised eosinophils (980 /μL). Proteinase 3- and myeloperoxidase-ANCA were negative. In view of the patient’s bronchial asthma, eosinophilia, and weight loss of 10 kg within 6 months, the patient was diagnosed with EGPA. He was started on high-dose prednisolone therapy of 60 mg (1 mg/kg), following which he experienced minimal chest pains and the resolution of his gastrointestinal symptoms. 

The patient demonstrated sustained clinical improvement and was discharged with a 30 mg of high-dose prednisolone therapy. During outpatient visits over the following 4 months, the patient complained of mild chest pain, accompanied by an increase in eosinophil count, which prompted his physicians to add on 300 mg of mepolizumab to safely taper his corticosteroids. Two years later, his prednisolone was further tapered to 6 mg, and there were no more complaints of chest pain or discomfort. He continued to receive 300 mg of mepolizumab subcutaneously every 4 weeks, which was well tolerated. 

“Mepolizumab combined with oral steroid treatment seems to be useful for EGPA complicated with cardiac involvement,” the authors concluded. 

Reference

Takigawa Y, Fujiwara K, Tabuchi I, et al. Successful treatment with mepolizumab for coronary spastic angina associated with eosinophilic granulomatosis with polyangiitisIntern Med. Published online August 15, 2023. doi:10.2169/internalmedicine.0930-22