Department of Pediatric Rheumatology and Internal Medicine, A. Harouchi Mother-Child Hospital, Ibn Rochd University Hospital, 92957Hassan II University, Casablanca, Morocco.
Department of Cardiology, Ibn Rochd University Hospital, 92957Hassan II University, Casablanca, Morocco.
Lupus. 2022 Apr;31(5):619-623. doi: 10.1177/09612033221086467. Epub 2022 Mar 20.
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.
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.
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.
系统性幼年特发性关节炎可影响包括心脏在内的任何器官。虽然心包炎和心内膜炎是最常见的心脏并发症，但扩张型心肌病仍然很少见。我们报告了一例幼年狼疮女孩扩张型心肌病完全恢复的情况。
一名 7 岁女孩出现持续性发热、体重减轻、脱发、抗狼疮抗体阳性和补体减少。检查发现呼吸困难、心动过速和低血压。胸部 X 线显示心脏轮廓增大，超声心动图显示左心室射血分数为 35%。排除其他原因后，选择了系统性红斑狼疮相关扩张型心肌病。给予心力衰竭治疗、泼尼松和羟氯喹。第三天，她发生了巨噬细胞活化综合征，对甲泼尼龙冲击治疗有反应。4 周后临床和生物学指标改善，4 个月后超声心动图恢复正常。
对文献的回顾证实了幼年特发性关节炎扩张型心肌病的罕见和严重性，强调了在出现任何心脏体征时进行超声心动图检查的重要性。
