Background: Heart failure in systemic lupus erythematosus (SLE ) is rare, and its long-term outcome
is unknown. The aim of this study was to analyse the
long-term outcome of six SLE patients with heart failure
as first manifestation of cardiac involvement and to review previously reported cases. Methods: We conducted a retrospective chart review of SLE patients from two tertiary referral centres who presented between 1999 and 2004 with clinical and echocardiographic signs of heart failure as their first manifestation of cardiac involvement. Details of the clinical presentation and follow-up and serial findings at echocardiography were collected. A retrospective review of the literature was performed using the PubMed database. Results: Six cases were identified who presented with heart failure, as confirmed by echocardiography (left ventricular ejection fraction (LVEF ) ranging from 23 to 37%). Treatment with high-dose glucocorticoids, cytotoxic treatment (azathioprine in one patient, cyclophosphamide in five patients), intravenous immunoglobulins (in one patient) and temporary inotropic support (two patients) resulted in complete resolution of symptoms and improvement of LVEF , with a mean follow-up of 77 months (range 43 to 113). Twenty-one additional cases of heart failure as manifestation of cardiac involvement in SLE have been reported, most with favourable short-term outcome
following institution of immunosuppressive therapy.
Conclusions: Heart failure is a rare but life-threatening
manifestation of cardiac involvement in SLE . Long-term
outcome can be excellent when aggressive treatment is