Therapeutic Strategies for Lupus Myocarditis Complicated by Cardiogenic Shock

Abstract

Purpose Systemic lupus erythematosus (SLE) is a systemic disease which commonly involves the heart. Lupus myocarditis can present as a catastrophic event requiring aggressive therapy including mechanical circulatory support (MCS). We analyzed reported cases of cardiogenic shock in lupus in order to study utilized strategies and outcomes. Methods Three investigators independently searched PubMed, Ovid/Medline, and Google Scholar using terms “cardiogenic shock”, “cardiac shock” AND “lupus”, “systemic lupus erythematosus”, and “lupus myocarditis” for papers published in English in 1988-2020. We then manually searched the references in relevant articles. We included all cases of adult patients where individual patient data were present. Results We identified 22 cases, 18 (82%) females, mean age 27.7 +/- 3.35 years. All patients except two recovered (one patient died and one received heart transplantation). Left ventricular ejection fraction at the baseline was 20.5 +/- 3.49%, and after treatment it recovered to 46.1 +/- 6.2%. Management included inotropes and immunotherapy in all cases. In terms of immunotherapy, all received corticosteroids, but only 5 patients (25%) did not require any other immunotherapy. In six more patients, combination of steroids and cyclophosphamide appeared to be sufficient. In 10 of 20 patients, either plasmapheresis or intravenous immunoglobulin was also given. Remarkably, half of the patients (10 out of 21 patients who survived) received MCS: intra-aortic balloon bump placement (6 cases), venoarterial extracorporeal membrane oxygenation (5), left ventricular assist device (1), and biventricular support (1). Conclusion Based on reported cases, cardiogenic shock in lupus myocarditis has a high potential for recovery, can be treated successfully, but it requires an aggressive approach with immunotherapy going beyond corticosteroids and cyclophosphamide, and utilization of mechanical circulatory support.