KEGG   DISEASE: 抗リン脂質抗体症候群
エントリ  
H01697                                                             
名称    
抗リン脂質抗体症候群
概要    
Antiphospholipid syndrome (APS) is characterized by a variety of clinical and immunological manifestations. The clinical hallmarks of this syndrome are thrombosisand poor obstetric outcomes in the presence of antiphospholipid antibodies. APS can occur within the context of several diseases, mainly autoimmune disorders, and is then called secondary APS. About 40% of patients with systemic lupus erythematosus (SLE) have antiphospholipid antibodies, but less than 40% of them will eventually have thrombotic events. On the other hand, it may be also be present without any recognizable disease, or so-called primary APS. Pregnancy complications in APS, such as recurrent unexplained abortions, fetal loss, preeclampsia, and premature birth, have been attributed to placental thrombosis and infarcts, and management of these patients is based on attenuating the procoagulant state. Treatment with low-dose aspirin (LDA) and heparinoids has become a conventional option for pregnant women with APS. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs.
カテゴリ  
血液疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  非器官特異的全身性自己免疫疾患
   4A45  抗リン脂質抗体症候群
    H01697  抗リン脂質抗体症候群
指定難病 [jp08407.html]
 H01697
コメント  
See also H00080 Systemic lupus erythematosus
リンク   
ICD-11: 4A45
ICD-10: D68.8
MeSH: D016736
OMIM: 107320
文献    
  著者
Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA
  タイトル
Antiphospholipid syndrome.
  雑誌
Lancet 376:1498-509 (2010)
DOI:10.1016/S0140-6736(10)60709-X
文献    
  著者
Yelnik CM, Laskin CA, Porter TF, Branch DW, Buyon JP, Guerra MM, Lockshin MD, Petri M, Merrill JT, Sammaritano LR, Kim MY, Salmon JE
  タイトル
Lupus anticoagulant is the main predictor of adverse pregnancy outcomes in aPL-positive patients: validation of PROMISSE study results.
  雑誌
Lupus Sci Med 3:e000131 (2016)
DOI:10.1136/lupus-2015-000131
文献    
  著者
Lefkou E, Mamopoulos A, Dagklis T, Vosnakis C, Rousso D, Girardi G
  タイトル
Pravastatin improves pregnancy outcomes in obstetric antiphospholipid syndrome refractory to antithrombotic therapy.
  雑誌
J Clin Invest 126:2933-40 (2016)
DOI:10.1172/JCI86957
文献    
  著者
Gezer S
  タイトル
Antiphospholipid syndrome.
  雑誌
Dis Mon 49:696-741 (2003)
DOI:10.1016/j.disamonth.2003.10.001
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