THU0601 REFRACTORY ACUTE CORONARY SYNDROME AND CARDIOGENIC SHOCK AS ATYPICAL ONSET OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

• Published in: Annals of the rheumatic diseases Vol. 79; no. Suppl 1; pp. 542 - 543
• Main Authors: Ravagnani, V., Vinco, G., Aroldi, M., Frigato, M., Izzo, A., Costantino, M. T., Lettieri, C.
• Format: Journal Article
• Language: English
• Published: 01.06.2020
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2020-eular.6482

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is an ANCA-associated vasculitis, characterized by eosinophilic infiltration in tissues, intravascular and extravascular granuloma formation. It is a rare disease, affecting between 0 and 4 per million population per year. The cardiac involvement occurs in 15-60% of EGPA patients (generally ANCA-negative); it is often insidious, underestimated and it has a poor prognosis. The disease usually shows a prodromal phase characterized by asthma and allergic manifestations. Objectives: We report the case of a young patient with acute coronary syndrome (ACS) complicated by cardiogenic shock as the first manifestation of EGPA. Methods: A 36 year old Indian male patient, with a previous history of asthma, rhinitis, Raynaud syndrome and allergy to ketoprofen, presented to the emergency department with a complaint of chest pain and dyspnea. Cardiac troponin was elevated and he was admitted to intensive care unit with a diagnosis of ACS. The patient conditions rapidly deteriorated due to acute cardiogenic shock and an urgent coronary angiogram was performed. An occlusion of the left main coronary artery was treated with angioplasty and two drug-eluting stents. Echocardiography showed severe left ventricular dysfunction requiring inotropic and intra-aortic balloon pump support. A mild dermatitis after salicylic acid administration resolved with intravenous hydrocortisone 1 g. The thrombophilia screening was negative, as well as cardiovascular risk factors. Over the next days, the clinical conditions rapidly improved with recovery of normal ventricular function on discharge. However, two weeks later he was readmitted with recurrent mandibular and chest pain. Troponin levels were elevated and fluctuated, suggesting recurrent ischemic events. Repeated ECG during angina crisis showed ischemic alterations in different coronary territories. The coronary angiogram detected coronary vasospasm of the circumflex artery, reversible after nitroglycerin (Figure 1). Nitroglycerin and calcium channel blockers were initiated, but did not resolve the vasospastic angina crisis, occurring daily. Laboratory tests revealed eosinophilia (4390 cells/mcl), increased C reactive protein (9.4 mg/l) and positive antinuclear antibodies (1:320). The other serological and immunological tests were negative, including MPO-ANCA and PR3-ANCA. An abdomen and chest CT scan was negative. Figure 1. Coronary angiogram showing coronary vasospasm in the left circumflex artery (panel A, yellow arrow), reversible after nytroglicerin infusion (panel B, yellow arrow) Results: The eosinophilia and the history of asthma rose the suspect of EGPA vasculitis. The patient was treated with intravenous methylprednisolone 250 mg once daily for 3 days, followed by oral prednisone 1 mg/kg/day, with rapid and complete resolution of the recurrent angina episodes. Intravenous cyclophosphamide 10 mg/kg was administered every 2 weeks for 2 times, then 12 mg/kg every 4 weeks. Oral corticosteroid was tapered, with the persistence of a complete remission of the symptoms, after 2 months of immunosuppressive therapy. Conclusion: Coronary involvement in EGPA can mimic atherosclerotic artery disease and can be life threatening, if not promptly recognized. An accurate medical history and complete serological and immunological tests are crucial to detect an atypical onset of EGPA, prompting early immunosuppressive therapy which is pivotal for the patient survival. References: [1]MR Hazebroek et al. Prevalence and Prognostic Relevance of Cardiac Involvement in ANCA-associated Vasculitis: Eosinophilic Granulomatosis With Polyangiitis and Granulomatosis With Polyangiitis. Int J Cardiol 2015, 199: 170-9. Disclosure of Interests: None declared DOI: 10.1136/annrheumdis-2020-eular.6482