Chen Yu-Jhen, Lin Ying-Jui, Guo Mindy Ming-Huey
Department of Pediatric Allergy Immunology and Rheumatology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Department of Pediatric Cardiology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Front Pediatr. 2022 Jan 26;10:772422. doi: 10.3389/fped.2022.772422. eCollection 2022.
Systemic lupus erythematosus (SLE) is an autoimmune disease that may cause vital organ damage. Although not rare for child-onset SLE to have cardiovascular or pulmonary involvement, myocarditis, and pulmonary hypertension are infrequent features and can be life-threatening. In this case report, we describe an 11-year-old girl with SLE who initially presented with fulminant myocarditis pulmonary hypertension, and massive pericardial effusion. Initial immunosuppressive therapy with methylprednisolone pulse therapy, and IVIG were administered, followed by cyclophosphamide, which was ultimately successful, with no residual pulmonary hypertension and no recurrence of myocarditis for over 3 years after the initial episode. Our case highlights the need for clinicians to be aware of systemic lupus erythematosus as a possible diagnostic entity in pediatric patients with severe myocarditis or pulmonary hypertension. Aggressive immunosuppressive therapy should be strongly considered in such cases, as it may lead to good short-term and long-term outcomes.
系统性红斑狼疮（SLE）是一种可能导致重要器官损害的自身免疫性疾病。虽然儿童期SLE出现心血管或肺部受累并不罕见，但心肌炎和肺动脉高压并不常见，且可能危及生命。在本病例报告中，我们描述了一名11岁的SLE女孩，她最初表现为暴发性心肌炎、肺动脉高压和大量心包积液。最初给予甲泼尼龙冲击疗法和静脉注射免疫球蛋白进行免疫抑制治疗，随后使用环磷酰胺，最终治疗成功，初始发作后3年多来无残留肺动脉高压，心肌炎也未复发。我们的病例强调临床医生需要意识到系统性红斑狼疮可能是患有严重心肌炎或肺动脉高压的儿科患者的一个诊断实体。在这种情况下，应强烈考虑积极的免疫抑制治疗，因为这可能带来良好的短期和长期预后。
