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Published on: May 16, 2020
Dilated cardiomyopathy: An unusual and severe condition in juvenile systemic lupus erythematosus
Kenza Bouayed1, Tiaaza Faid1, Asmaa Sakhi1
1Department of Pediatric Rheumatology and Internal Medicine, A. Harouchi Mother-Child Hospital, Ibn Rochd University Hospital, 92957Hassan II University, Casablanca, Morocco.
Insights
Systemic juvenile lupus erythematosus can cause rare dilated cardiomyopathy. This case shows full recovery with timely treatment and monitoring, highlighting the importance of cardiac evaluation.
Area of Science:
- Pediatric Rheumatology
- Cardiology
Background:
- Systemic juvenile lupus erythematosus (sJLE) can manifest with cardiac involvement, though dilated cardiomyopathy is an uncommon complication.
- While pericarditis and endocarditis are more frequent, this report focuses on a rare instance of dilated cardiomyopathy in a child with sJLE.
Observation:
- A 7-year-old girl presented with constitutional symptoms, alopecia, and serological markers of lupus.
- Clinical examination revealed signs of heart failure, including dyspnea, tachycardia, and hypotension, with imaging confirming significantly reduced left ventricular ejection fraction.
Findings:
- The patient was diagnosed with systemic lupus erythematosus-related dilated cardiomyopathy and treated with standard heart failure therapy, corticosteroids, and hydroxychloroquine.
- She experienced a concurrent episode of macrophage activation syndrome, which was successfully managed with methylprednisolone.
Implications:
- Complete recovery of cardiac function was observed within four months of treatment initiation.
- This case underscores the critical need for echocardiography in children with juvenile lupus presenting with any cardiac symptoms, given the potential for severe but reversible cardiomyopathy.
Background:
Systemic juvenile lupus erythematosus can affect any organ including the heart. While pericarditis and endocarditis are the most common cardiac complications, dilated cardiomyopathy remains rare. We report the full recovery of dilated cardiomyopathy in a girl with juvenile lupus.
Case Presentation:
A 7-year-old girl presented with persistent fever, weight loss, alopecia, positive anti-lupus antibodies, and decreased complement. Examination found dyspnea, tachycardia, and hypotension. Chest X-ray revealed an enlarged cardiac silhouette, and echocardiography showed a left ventricular ejection fraction of 35%. After excluding other causes, systemic lupus erythematosus related dilated cardiomyopathy was selected. Heart failure treatment, Prednisone, and hydroxychloroquine were prescribed. On day three, she developed macrophage activation syndrome which responded to methylprednisolone boluses. Clinical and biological improvement was observed after 4 weeks, normalization of echocardiography after 4 months.
Conclusion:
A review of the literature confirmed the rare and severe nature of dilated cardiomyopathy in juvenile lupus emphasizing the importance of performing echocardiography at the slightest cardiac sign.
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