Myocarditis with Cardiac Failure: Initial Manifestation of Systemic Lupus Erythematosus

Abstract

Cardiac involvement is very rare in Systemic Lupus Erythematosus (SLE). Pericarditis is the leading cardiovascular manifestation in SLE followed by endocarditis, conduction abnormality, coronary artery disease and myocardial dysfunction. Myocardial dysfunction is most often subclinical, but may rarely progress to ventricular dysfunction, cardiogenic shock or severe congestive heart failure. Myocarditis leading to cardiac failure is a very unusual presentation of childhood lupus. We present a four and half-year-old young boy who presented to us with features of congestive heart failure subsequently diagnosed to be secondary to fulminant lupus myocarditis. Despite intensive management, the child ultimately succumbed. His echocardiography revealed dilated left ventricle with an Ejection Fraction (EF) of 18.2%. A positive titre of p-ANCA was also seen in this case. Although, ANCA positivity has been shown as a bad prognostic marker for lupus nephritis, its association with lupus myocarditis is still unknown. Decongestive measures along with immunosuppressant drugs remain the preferred treatment. This case highlights the protean presentation of SLE in the form of lupus myocarditis leading to congestive heart failure that merits an urgent intervention because of the potentially devastating consequences.

Keywords

• cyclophosphamide
• heart failure
• pericarditis