[A wolf in sheep's clothing: atypical systemic lupus erythematosus (SLE) presenting as cardiovascular disease]

A 51-year-old woman diagnosed as having valvular cardiomyopathy since age 34 was admitted for an evaluation for a heart transplant because of progressive congestive heart failure. When antiphospholipid antibodies were detected, the diagnosis of a thus far undetected systemic lupus erythematosus (SLE) was confirmed, manifesting primarily by cardiac involvement and an antiphospholipid antibody syndrome. Despite an advanced stage of heart failure, the patient responded well to azathioprine. Nevertheless, the potential necessity of a heart transplant remained. Its atypical presentation impeded a timely diagnosis of SLE significantly, however, in retrospect the correct diagnosis would have been possible at an earlier time point.

Though rare, SLE represents an important differential diagnosis in cases of severe valvular disease and cardiomyopathy, particularly in young women.