Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report

Introduction Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by tissue and blood eosinophilia, vasculitis of small to medium-sized vessels, and allergy symptoms, and can cause various manifestations, including heart, lung, gastrointestinal, skin, and peripheral nerve disorders. 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 48mmHg 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 34mmHg 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.