PDF(Pubmed)
Abstract：
Antiphospholipid antibodies (aPL) are both laboratory evidence and causative factors for a broad spectrum of clinical manifestations of antiphospholipid syndrome (APS), with thrombotic and obstetric events being the most prevalent. Despite the aPL-triggered vasculopathy nature of APS, vasculitic-like manifestations rarely exist in APS and mainly appear associated with other concurrent connective tissue diseases like systemic lupus erythematous. Several studies have characterized pulmonary capillaritis related to pathogenic aPL, suggesting vasculitis as a potential associated non-thrombotic manifestation. Here, we describe a 15-year-old girl who develops hepatic infarction in the presence of highly positive aPL, temporally related to prior non-severe COVID-19 infection. aPL-related hepatic vasculitis, which has not been reported before, contributes to liver ischemic necrosis. Immunosuppression therapy brings about favorable outcomes. Our case together with retrieved literature provides supportive evidence for aPL-related vasculitis, extending the spectrum of vascular changes raised by pathogenic aPL. Differentiation between thrombotic and vasculitic forms of vascular lesions is essential for appropriate therapeutic decision to include additional immunosuppression therapy. We also perform a systematic review to characterize the prevalence and clinical features of new-onset APS and APS relapses after COVID-19 for the first time, indicating the pathogenicity of aPL in a subset of COVID-19 patients.
摘要：
抗磷脂抗体（aPL）是抗磷脂综合征（APS）的广泛临床表现的实验室证据和致病因素，血栓性和产科事件最为普遍。尽管APS具有aPL触发的血管病变性质，APS中很少存在血管样表现，主要与其他并发结缔组织疾病如系统性红斑狼疮有关。一些研究已经描述了与致病性aPL相关的肺毛细血管炎，提示血管炎是一种潜在的非血栓性表现。这里,我们描述了一个15岁的女孩在aPL高阳性的情况下发展为肝梗塞，与先前非严重COVID-19感染时间相关。aPL相关的肝血管炎，以前没有报道过，导致肝脏缺血性坏死。免疫抑制治疗带来有利的结果。我们的病例和检索到的文献为aPL相关血管炎提供了支持性证据，扩展致病性aPL引起的血管变化谱。区分血管病变的血栓性和血管性形式对于包括其他免疫抑制疗法的适当治疗决定至关重要。我们还进行了系统评价，以首次描述新发APS和COVID-19后APS复发的患病率和临床特征，表明aPL在COVID-19患者亚群中的致病性。
