Case Presentation:

Systemic Lupus Erythematous (SLE) is one of the most common multisystem autoimmune disorders in the world with a variable prevalence of 6:100,000. There are a number of possible cardiac manifestation of SLE, the most common forms being pericarditis, myocarditis, ischemic coronary artery disease, congestive heart failure. Here we present a rare case of lupus induced non–ischemic cardiomyopathy.

Discussion:

A 42–year–old African American female with a three year history of systemic lupus erythematous. She presented with shortness of breath, arthritis, and anasarca from her lower extremity to mid–thighs. Medications included cellcept, hydroxychloroquine, lisinopril, and prednisone. Vitals were noted as blood pressure of 161/94, pulse rate 153, respiratory rate 36, saturation 92% on 6 liters of nasal cannula. On examination, moderate distress, unable to speak in full sentences, mild conjunctival pallor, diminished breath sounds with crackles at bases bilateral, 3+ pitting edema to the level of mid–thigh. Notable serum studies were hemoglobin 9.2, wbc 11.2, ABG revealed metabolic acidosis. UA revealed >3 grams of protein, large bilirubin, large blood, moderate leuko esterase, WBC 50–75, RBC 25–50, 3+ bacteria. CXR showed low lung volumes and bilateral pleural effusions. Low complement levels (C3 = 48, C4 = < 8), positive dDNA, negative hepatitis panel and any circulating antibodies. Echocardiogram revealed markedly depressed left ventricular ejective fraction 10–15% with global hyopkinesis. One month previously, her ejection revealed 55% without any hypokinesis. Angiography revealed normal coronary arteries. She was intubated, started on furosemide, 1 day later her creatinine trended up to and she because anuric. Dialysis was initiated. She received 5 consecutive days of plasmapharesis, 1 gram of methylprednisone for 3 days, cellcept and plaqueril. Renal biopsy showed she had a thrombotic microangiopathy with secondary segmental sclerosis and mild interstitial fibrosis and no evidence of lupus nephritis. On day 8, repeat echo showed ejection fraction of 35% with improved hypokinesis, creatinine returned to baseline, dialysis was ceased.

Conclusions:

This is a rare case of non–ischemic lupus cardiomyopathy with congestive heart failure. When coronary artery atherosclerosis, hypertensive cardiomyopathy, toxic and viral etiologies can be ruled out, lupus specific cardiomyopathy should be considered. When heart failure is related to lupus cardiomyopathy, treatment with steroids should be given, along with conventional therapy for heart failure, including beta blockers and angiotensin converting enzyme inhibitors.