Heart failure as presenting manifestation of cardiac involvement in systemic lupus erythematosus.
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 instituted promptly.
Deptartment of Rheumatology, Leiden University Medical Center, the Netherlands.
N E
NE
van der Laan-Baalbergen
S A
SA
Mollema
H
H
Kritikos
A
A
Schoe
T W J
TW
Huizinga
J J
JJ
Bax
D T
DT
Boumpas
J M
JM
van Laar
D000328:Adult; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006333:Heart Failure / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D008775:Methylprednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D014463:Ultrasonography; D016277:Ventricular Function, Left; D055815:Young Adult
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine; D008775:Methylprednisolone