CASE PRESENTATION: A 34-year-old woman with a history of
asthma,
nasal polyp, and
sinusitis presented with
ventricular fibrillation after severe
chest pain. Emergent coronary angiography showed no
coronary stenosis. After admission, she suffered from hypoxaemia and recurrent
chest pain with ST-segment changes, suggesting vasospastic angina (VSA). Chest computed tomography (CT) showed centrilobular nodular shadows, suggesting
bronchiolitis. Since she had
hypereosinophilia, we administered oral
prednisolone, which resulted in improvements of
hypereosinophilia, hypoxaemia, and recurrent
chest pains in 3 days. Right heart catheterization showed severe
pulmonary hypertension (PH) with a mean pulmonary artery pressure (mPAP) of 48 mmHg and pulmonary vascular resistance (PVR) of 12 Wood units (WU).
Ergonovine provocation test induced severe diffuse
spasm of the left coronary artery including the left main trunk. Based on
asthma,
sinusitis,
hypereosinophilia, and chest CT findings, the diagnosis of EGPA associated with VSA and PH was made. Thereafter, we started intravenous
cyclophosphamide (IV-CY) pulse
therapy in addition to
prednisolone and pulmonary
vasodilators. Six months after IV-CY
therapy, mPAP and PVR decreased to 34 mmHg and 5.1 WU, respectively. Moreover, repeated
ergonovine provocation test was negative without coronary
spasm or electrocardiogram (ECG) changes.
DISCUSSION: This case indicates that EGPA can cause severe PH, refractory VSA, and
bronchiolitis, which could be markedly improved by treating underlying conditions with immunosuppressive therapy.