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镰状细胞性状患者以心肌心包炎和心包积液为系统性红斑狼疮的首发表现:一例报告

Myopericarditis and Pericardial Effusion as the Initial Presentation of Systemic Lupus Erythematosus in a Patient with Sickle Cell Trait: A Case Report.

作者信息

Rojas-Cadena Marlon, Rodríguez-Arcentales Felipe, Narváez-Cajas Jose, Arias-Intriago Marlon, Morales Orbe Karen, Izquierdo-Condoy Juan S

机构信息

Medical Science Faculty, Universidad Católica del Ecuador, Quito 170525, Ecuador.

Department Section of Histology, Faculty of Medical Science, Universidad Central del Ecuador, Quito 170402, Ecuador.

出版信息

J Clin Med. 2025 Jan 30;14(3):920. doi: 10.3390/jcm14030920.

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with rare but severe cardiac manifestations, including myocarditis and pericarditis. The coexistence of SLE with sickle cell trait (SCT), an inherited hemoglobinopathy prevalent among individuals of African descent, is exceptionally rare and presents significant diagnostic challenges due to overlapping clinical features. To describe the case of an Afro-Ecuadorian male with SLE and sickle cell trait who developed an uncommon presentation of myopericarditis and pericardial effusion. A 48-year-old African American male with no prior medical history presented with persistent fever, polyarticular arthralgias, and pleuritic chest pain. Investigations revealed sickle cell trait (SCT) and myopericarditis with pericardial effusion, marking the initial manifestation of SLE. Diagnostic delays occurred due to overlapping symptoms and a family history of sickle cell disease. Laboratory findings showed elevated hemoglobin S (<50%), positive ANA (1:1280, coarse speckled pattern), and anti-Smith/RNP antibodies, meeting EULAR/ACR 2019 criteria for SLE. Cardiac MRI confirmed myopericarditis. Treatment with pulse methylprednisolone, oral prednisone, and mycophenolate mofetil resulted in clinical improvement, with stable disease control on immunomodulatory therapy during follow-up. This case highlights the diagnostic complexity of SLE in patients with SCT, particularly when presenting with myopericarditis as the initial manifestation. It emphasizes the importance of a comprehensive diagnostic approach and timely initiation of immunosuppressive therapy to optimize clinical outcomes. This report broadens the understanding of overlapping syndromes involving SLE and SCT.

摘要

系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,具有罕见但严重的心脏表现,包括心肌炎和心包炎。SLE与镰状细胞性状(SCT)共存,镰状细胞性状是一种在非洲裔个体中普遍存在的遗传性血红蛋白病,极为罕见,由于临床特征重叠,带来了重大的诊断挑战。描述一名患有SLE和镰状细胞性状的非洲裔厄瓜多尔男性出现罕见的心肌心包炎和心包积液表现的病例。一名无既往病史的48岁非裔美国男性出现持续发热、多关节疼痛和胸膜炎性胸痛。检查发现镰状细胞性状(SCT)以及伴有心包积液的心肌心包炎,这是SLE的初始表现。由于症状重叠和镰状细胞病家族史,诊断出现延迟。实验室检查结果显示血红蛋白S升高(<50%)、抗核抗体阳性(1:1280,粗颗粒型)以及抗史密斯/核糖核蛋白抗体,符合2019年欧洲抗风湿病联盟/美国风湿病学会SLE标准。心脏磁共振成像证实为心肌心包炎。采用脉冲甲基强的松龙、口服强的松和霉酚酸酯治疗后临床症状改善,随访期间免疫调节治疗使病情得到稳定控制。该病例突出了SCT患者中SLE的诊断复杂性,尤其是以心肌心包炎作为初始表现时。强调了全面诊断方法和及时启动免疫抑制治疗以优化临床结局的重要性。本报告拓宽了对涉及SLE和SCT重叠综合征的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6362/11818129/c4fbb49ac95f/jcm-14-00920-g001.jpg

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