Dramatic improvement of left ventricular function after cytotoxic therapy in lupus patients with acute cardiomyopathy: Report of 6 cases

Although lupus cardiomyopathy is thought to be clinically uncommon, we enco untered 6 patients with systemic lupus erythematosus (SLE) over a 10 year p eriod who had severe left ventricular dysfunction and showed remarkable imp rovement in their cardiac function after cytotoxic therapy. All patients me t the American College of Rheumatology criteria for classification of SLE a nd presented with signs of severe biventricular failure relatively early in their disease. Concurrent manifestations of SLE at the time of cardiomyopa thy included rash, arthritis, myalgias, pleuritis, pericarditis, and nephri tis. Four of the 6 patients were taking prednisone 20 mg/day at the time he art failure developed. In all cases the CPK were normal. Evaluation of card iac function by echocardiogram and/or radionuclide gated blood pool scintig raphy revealed a severe depression of ventricular function with initial lef t ventricular ejection fraction (LVEF) ranging from 11 to 34% (mean 19%). W ithin 6 months of initiation of cytotoxic treatment all patients showed a d ramatic response: the post-treatment LVEF ranged from 25 to 55%. This serie s of patients suggests that cardiomyopathy may be a more cmmon complication of SLE than previously reported. Cardiomyopathy occurs relatively early in the course of SLE, may lead to severe cardiac dysfunction despite corticos teroid therapy, and appears to be responsive to cytotoxic therapy.