Clinical characteristics of lupus myocarditis in Korea

Clinically important myocarditis is an unusual feature in systemic lupus erythematosus (SLE). We describe the clinical characteristics, management and outcomes of five SLE patients who developed severe left ventricular dysfunction. Four patients were female with mean age of 36.4 years. Three patients had both lupus myocarditis and lupus nephritis. Four patients had raised anti-dsDNA antibody titer and low complement level and two patients had positive IgG anticardiolipin antibody. Three patients were treated by high-dose corticosteroids, one patient by intravenous pulse methylprednisolone, and one patient by intravenous immunoglobulin and pulse cyclophosphamide with high dose corticosteroids. Left ventricular function improved markedly in four patients and all of them had no recurrence of lupus myocarditis up to follow-up of 33 months. However, one patient, who showed no improvement of left ventricular function, was expired due to sudden cardiac arrest. Lupus myocarditis should be treated by immunosuppressive therapy with high-dose corticosteroids and mostly the prognosis might be good with early treatment. the prevalence of cardiac involvement in SLE to be higher than 50% [1-3]. Cardiac manifestation can involve all the cardiac structures, including the pericardium, the myocardium, and the endocardium. The prevalence of myocarditis from necropsy studies had been reported to be 40-70%. However, symptomatic lupus myocarditis is reported to occur in only 5-10%, suggesting that subclinical cardiac involvement is common [4, 5]. A prospective echocardiographic study of 70 patients found myocardial abnormalities in 14 cases ( 20%) but only one patient had clinical symptoms [6]. Myocarditis may manifest itself as an acute illness or have a chronic course with the development of a cardiomyopathy due to small vessel vasculitis and thrombosis [7]. Myocarditis may rarely be the initial presentation but a potentially fatal complication of SLE. There is a little information available on clinical manifestation and outcome of myocarditis in SLE. We describe the clinical characteristics of five patients with SLE seen over a 33 month period, who presented with severe left ventricular dysfunction.