Saigal Renu, Kansal Amit, Mittal Manoop, Singh Yadvinder, Ram Hari
Department of Medicine, Sawai Man Singh Medical College and Hospital, Jaipur, India.
J Assoc Physicians India. 2010 Mar;58:176-84.
The 2006 International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Syndrome has increased the time between the two laboratory studies required for diagnosis from 6 to 12 weeks. Antibody to beta2 glycoprotein 1 has been included as a criterion. Various non-criteria diagnostic clues such as livedo reticularis, heart valve disease, thrombocytopenia, renal thrombotic microangiopathy, neurological manifestations, non-criteria antibodies (IgA aCL, IgA anti-beta2 glycoprotein I) and some research laboratory-identified antibodies (antiphosphatidylserine antibodies, antiphosphatidylethanolamine antibodies, antibodies against prothrombin alone and antibodies to the phosphatidylserine-prothrombin complex) have been recognised. New concepts of pathogenesis now implicate complement activation and participation of the innate immune system upstream to thrombosis. Warfarin remains the treatment of choice for patients who have suffered thrombosis, but antiplatelet agents and heparin are other options. Target INR is 2.0-3.0. The other drugs which are used in resistant cases are: rituximab, hydroxychloroquine, thrombin inhibitors and statins.
2006年《抗磷脂综合征分类标准更新国际共识声明》将诊断所需的两项实验室检查之间的时间间隔从6周延长至12周。抗β2糖蛋白1抗体已被纳入诊断标准。各种非标准诊断线索，如网状青斑、心脏瓣膜病、血小板减少、肾血栓性微血管病、神经系统表现、非标准抗体（IgA aCL、IgA抗β2糖蛋白I）以及一些研究实验室鉴定出的抗体（抗磷脂酰丝氨酸抗体、抗磷脂酰乙醇胺抗体、单独的抗凝血酶原抗体以及抗磷脂酰丝氨酸-凝血酶原复合物抗体）已得到认可。目前发病机制的新概念涉及补体激活以及先天性免疫系统在血栓形成上游的参与。华法林仍然是血栓形成患者的首选治疗药物，但抗血小板药物和肝素也是其他选择。目标国际标准化比值（INR）为2.0 - 3.0。用于难治性病例的其他药物有：利妥昔单抗、羟氯喹、凝血酶抑制剂和他汀类药物。
