Antiphospholipid syndrome: an update

被引:26
|
作者
Merashli, Mira [1 ]
Noureldine, Mohammad Hassan A. [2 ]
Uthman, Imad [3 ]
Khamashta, Munther [4 ]
机构
[1] Royal London Hosp, Fac Med, Div Rheumatol, London E1 2DR, England
[2] Lebanese Amer Univ, Med Ctr, Rizk Hosp, Fac Med, Beirut, Lebanon
[3] Amer Univ Beirut, Fac Med, Div Rheumatol, Beirut, Hamra, Lebanon
[4] Kings Coll London, Rayne Inst, St Thomas Hosp, Div Womens Hlth,Graham Hughes Lupus Res Lab, London SE1 7EH, England
关键词
Antiphospholipid antibodies; antiphospholipid syndrome; management; pathogenesis; risk stratification; MOLECULAR-WEIGHT HEPARIN; INTERNATIONAL CONSENSUS STATEMENT; SYSTEMIC-LUPUS-ERYTHEMATOSUS; THROMBOTIC RISK-ASSESSMENT; 1ST TRIMESTER TROPHOBLAST; RECURRENT PREGNANCY LOSS; TASK-FORCE REPORT; ANTIBODY SYNDROME; CONTROLLED-TRIAL; RENAL-TRANSPLANTATION;
D O I
10.1111/eci.12449
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BackgroundAntiphospholipid syndrome (APS) or Hughes syndrome' is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). More than three decades have passed, and experts are still uncovering new pieces of this disease complex pathogenesis and management. Materials and methodsWe searched in literature using MEDLINE and PubMed databases focusing on the latest development on disease pathogenesis, risk assessment of thrombosis and treatment of APS. ResultsThe phosphatidylinositol 3-kinase (PI3K)-AKT-mTORC pathway was most recently identified to have a crucial role in activating inflammation among endothelial vessel wall causing vascular lesions in APS. Additionally, new variables are being implemented to assess the risk of thrombosis in patients with APS. Global APS Score (GAPSS) utilizes cardiovascular risk factors and new autoimmune antibodies as part of the score assessment and is the most valid so far. It can be a promising tool in the future for prediction of thrombosis. Anticoagulation remains the cornerstone in APS; however, many new potential therapeutic agents are developing and are currently under investigation. ConclusionsThe most recent advances in pathogenesis, risk stratification and treatment provide a platform for high yield studies with the ultimate goal of providing the optimal management to patients with APS.
引用
收藏
页码:653 / 662
页数:10
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