以肾梗死为首发症状的原发性抗磷脂综合征一例诊治分析

Diagnosis and Treatment of One Case of Primary Antiphospholipid Syndrome with the First Symptoms of Renal Infarction

抗磷脂综合征(APS)是由抗磷脂抗体(APL)引起的自身免疫性疾病,临床上以血栓形成、习惯性流产及血小板减少为主要表现。APS引起血栓形成主要与其影响内皮细胞蛋白、血小板功能、抗凝及纤溶系统等有关。恶性抗磷脂综合征表现为短期内进行性广泛血栓形成,造成多器官功能衰竭甚至死亡。APS的诊断主要依靠临床表现和实验室检查,还必须排除其他自身免疫病和感染、肿瘤等疾病引起的血栓。主要治疗原则是防治血栓和流产的再发及对症处理,因此患者需长期抗血小板和抗凝治疗。本文通过对1例以急性肾梗死为首发症状的原发性抗磷脂综合征(PAPS)患者的诊治情况进行分析,进一步提高对APS的血栓形成机制、诊断和治疗的认识。

Antiphospholipid syndrome(APS) is an autoimmune disease caused by antiphospholipid antibody(APL) with major clinical symptoms like thrombosis,habitual abortion and thrombocytopenia.APS influences endothelial cell protein,platelet function,and the anticoagulation and fibrinolytic system,resulting in thrombosis.Malignant APS presents as progressive wide thrombosis in the short term,which causes multi-organ failure and even death.The diagnosis of APS mainly depends on the clinical manifestations and laboratory tests.Other causes of thrombus such as autoimmune disease,infection,and cancer need to be ruled out.The main therapeutic principles include prevention and treatment of thrombus and recurrent abortion,as well as symptomatic treatment.Therefore,the patients need long-term antiplatelet therapy and anticoagulant therapy.This article discusses the diagnosis and treatment of one case of primary APS with the first symptoms of renal infarction.