Dilated Cardiomyopathy in Systemic Lupus Erythematosus: A Pediatric Case Report

Abstract Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect any organ in the body, nonetheless cardiac involvement is frequent. Pericarditis is the most common type of heart attack followed by endocarditis, whereas myocarditis is rare. Despite improvements in the survival and quality of life of children with SLE, morbidity from the disease continues to be a major cause of death. Over the past three decades, lupus-related mortality has declined in all categories except cardiovascular disease. Heart disease is currently recognized as a major cause of death and morbidity in children with SLE. In fact, in the majority of cohort studies, the mean cardiovascular mortality is between 7 and 30%. We report full recovery of a little girl with SLE who developed dilated cardiomyopathy and review the literature. Case presentation: A 7-year-old girl presented with persistent fever, severe weight loss, alopecia, purpuric spots, positive anti-lupus antibodies, and decreased complement. She was diagnosed with SLE and treated with oral corticosteroids and hydroxychloroquine. On the third day of hospitalization, she developed a macrophage activation syndrome which improved with the methylprednisolone bolus. Additional examinations have been performed, including chest x-ray and echocardiography. They showed respectively marked enlargement of the cardiac silhouette and dilated cardiomyopathy with a left ventricular ejection fraction (LVEF) of approximately 35% associated with low pericardial effusion. She was diagnosed with SLE-related dilated cardiomyopathy and treated with furosemide, captopril, digoxin, spironolactone, and acetyl-salicylic acid. Clinical improvement was observed and the echocardiographic examination after 4 months of evolution was normalized, allowing cardiac treatments to be stopped while maintaining captopril.Conclusions: It is mandatory to check cardiac involvement in all patients with pediatric lupus to make an early diagnosis and ensure optimal management of cardiomyopathy, which is a rare, severe and silent complication.