一例从暴发性心肌炎到灾难性抗磷脂综合征的诊疗启示

Insights from a case of fulminant myocarditis progressing to catastrophic antiphospholipid syndrome

  • 摘要: 灾难性抗磷脂综合征(CAPS)是抗磷脂综合征(APS)最严重且危急的临床类型,以短期内广泛血栓形成导致多器官功能衰竭为特点,死亡率极高。CAPS的临床表现具有高度异质性,常模拟脓毒症、血栓性微血管病或暴发性心肌炎(FM)等急重症,极易误诊。其中,以急性心力衰竭或心肌炎为突出表现的CAPS尤为罕见,早期识别极为困难。本文通过一例曾被误诊为FM的青少年CAPS病例,系统分析其临床特征、诊断思路及管理策略,旨在提高临床医生对CAPS心脏受累的认识,强调早期开展抗磷脂抗体筛查及多学科协作治疗的重要性。

     

    Abstract: Catastrophic antiphospholipid syndrome (CAPS) represents the most severe clinical manifestation of antiphospholipid syndrome (APS), characterized by rapid onset of extensive thrombosis resulting in multi-organ failure and a markedly high mortality rate. The clinical presentations of CAPS exhibit considerable heterogeneity, often mimicking acute and severe conditions such as sepsis, thrombotic microangiopathy, or fulminant myocarditis (FM), which can lead to misdiagnosis. Notably, CAPS that presents with acute heart failure or myocarditis as its primary symptom is particularly uncommon, making early identification exceedingly challenging. This article examines a case of adolescent CAPS that was misdiagnosed as FM, systematically analyzing its clinical features, diagnostic considerations, and management strategies. The objective is to enhance clinicians' awareness of cardiac involvement in CAPS and to underscore the necessity of early antiphospholipid antibody screening and multidisciplinary collaborative treatment.

     

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