KEGG   DISEASE: Antiphospholipid syndrome
Entry
H01697                      Disease                                
Name
Antiphospholipid syndrome
Description
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.
Category
Hematologic disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A45  Antiphospholipid syndrome
    H01697  Antiphospholipid syndrome
Comment
See also H00080 Systemic lupus erythematosus
Other DBs
ICD-11: 4A45
MeSH: D016736
OMIM: 107320
Reference
  Authors
Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA
  Title
Antiphospholipid syndrome.
  Journal
Lancet 376:1498-509 (2010)
DOI:10.1016/S0140-6736(10)60709-X
Reference
  Authors
Yelnik CM, Laskin CA, Porter TF, Branch DW, Buyon JP, Guerra MM, Lockshin MD, Petri M, Merrill JT, Sammaritano LR, Kim MY, Salmon JE
  Title
Lupus anticoagulant is the main predictor of adverse pregnancy outcomes in aPL-positive patients: validation of PROMISSE study results.
  Journal
Lupus Sci Med 3:e000131 (2016)
DOI:10.1136/lupus-2015-000131
Reference
  Authors
Lefkou E, Mamopoulos A, Dagklis T, Vosnakis C, Rousso D, Girardi G
  Title
Pravastatin improves pregnancy outcomes in obstetric antiphospholipid syndrome refractory to antithrombotic therapy.
  Journal
J Clin Invest 126:2933-40 (2016)
DOI:10.1172/JCI86957
Reference
  Authors
Gezer S
  Title
Antiphospholipid syndrome.
  Journal
Dis Mon 49:696-741 (2003)
DOI:10.1016/j.disamonth.2003.10.001
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