Acute lupus myocarditis: clinical features and outcome of an oriental case series

Abstract

Symptomatic myocarditis in systemic lupus erythematosus (SLE) is uncommon. We describe the clinical characteristics, management and outcomes of 11 SLE patients without any atherosclerotic risk factors, who presented with acute lupus myocarditis (ALM). All patients were female, 46% Chinese with mean age of 27 +/- 10 years at diagnosis of SLE. ALM was one of the initial manifestations of SLE in eight (73%) patients. The median duration from onset ALM to initiation of treatment was two weeks (range: 0.3-8). All had clinical feature of left ventricle dysfunction. The most common electrocardiographic feature was nonspecific ST/T wave changes (91%). Common echocardiographic findings included segmental wall motion abnormalities (81%) and decreased left ventricular ejection fraction (81%). Median SLE disease activity index at presentation was 16 (range: 4-30). All patients received high dose corticosteroids and 64% received intravenous pulse cyclophosphamide. There were two deaths (18%) from infections. The remaining nine survivors had no recurrence of ALM nor suffer any SLE-related damage (median SLICC damage score of 0), up to a median follow-up of four years (range: 2.5-10.1). Repeat echocardiography after six months or later showed normal LVEF in eight patients (89%). Early immunosuppressive therapy in ALM, with high dose corticosteroids and pulse intravenous cyclophosphamide, results in good cardiac outcome.

Clinical characteristics of lupus myocarditis in Korea

“…However, the role of anti-Ro antibody in lupus myocarditis is not known so it could be further evaluated. There is a report that all patients had raised anti-dsDNA antibody, but the role of anti-dsDNA for lupus myocarditis was not mentioned [15]. In our cases four patients had increased anti-dsDNA level, but unlike the case of Law et al in which only Wve patients (44%) had concomitant active nephritis, three patients (60%) had concomitant lupus nephritis with low complements and increased anti-dsDNA levels.…”

Autologous hematopoietic stem cell transplantation in systemic lupus erythematosus patients with cardiac dysfunction: feasibility and reversibility of ventricular and valvular dysfunction with transplant-induced remission

“…This shows the procedure may be performed safely despite baseline impaired LVEF, and may reverse cardiac dysfunction in lupus-related myocardial disease, since conventional treatment of lupus-related myocarditis is immune-suppression with either steroids or cyclophosphamide. 23,24 There is no definitive modality of investigation to determine myocardial involvement by lupus however, and the diagnosis is one of exclusion of other causative factors. It is therefore uncertain which patients with abnormal LVEF will respond to the high-dose immunosuppression accorded by the transplant.…”

“…23,24 Pulmonary hypertension related to SLE has also been shown to respond to high-dose cyclophosphamide 31 and hence response to the lymphoablation of HSCT may be anticipated. On the other hand, the role of immunosuppression in Libman-Sacks endocarditis and lupus-related cardiac dysfunction is not clear.…”

Cardiogenic shock as the initial presentation of systemic lupus erythematosus

“…[1][2][3] There are few reports of heart failure and acute myocarditis as the initial manifestation of SLE. [4][5][6][7][8][9][10] These are not included within the standard diagnostic criteria for SLE developed by the American College of Rheumatology. Therefore, high suspicion is necessary, since timely therapy may help achieve full recovery.…”

“…Laboratory investigations revealed a normocytic normochromic anemia (5 An admission electrocardiogram showed sinus tachycardia with nonspecific T-wave abnormalities and prolonged QT. Troponin-I values peaked at 0.65 ng ⁄ mL (normal <0.04ng/ml).…”