Antiphospholipid syndrome

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Clinic

  • APS or antiphospholipid antibody syndrome (APLS) is an autoimmune hypercoagulable state caused by antiphospholipid antibodies.
  • APS provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia.
  • Although the exact etiology of APS is still not clear, genetics is believed to play a key role in the development of the disease.
  • The diagnostic criteria require one clinical event (i.e. thrombosis or pregnancy complication) and two positive blood test results spaced at least three months apart that detect lupus anticoagulant, anti-apolipoprotein antibodies, or anti-cardiolipin antibodies.
  • APS can be primary or secondary.
    • Primary antiphospholipid syndrome occurs in the absence of any other related disease.
    • Secondary antiphospholipid syndrome occurs with other autoimmune diseases, such as SLE.

Signs / Symptoms

  • Arterial or venous blood clots in any organ system
  • Pregnancy-related complications
  • The most common venous event is deep vein thrombosis
  • The most common arterial event is stroke.
  • Recurrent miscarriage, intrauterine growth restriction, and preterm birth, Placental infarctions
  • APS is responsible for most of the miscarriages in later trimesters.

Other common findings, although not part of the APS classification criteria, are low platelet count, heart valve disease, and livedo reticularis. There are also associations between antiphospholipid antibodies and different neurologic manifestations including headache, migraine, epilepsy, and dementia. Some studies have shown the presence of antiphospholipid antibodies in the blood and spinal fluid of patients with psychological symptoms. Cancer is also observed to comorbid in patients with APS.

Entity

Thrombosis


Miasms

CMV [1]

EBV [2]

HCV [3]

  1. Denham C, Tissier G, Golding A. Antiphospholipid antibody syndrome with thrombotic splenic infarcts associated with acute cytomegalovirus infection. Access Microbiol. 2019 Jun 10;1(7):e000032. doi: 10.1099/acmi.0.000032. PMID: 32974541; PMCID: PMC7481732.
  2. Ben-Chetrit E, Wiener-Well Y, Fadeela A, Wolf DG. Antiphospholipid antibodies during infectious mononucleosis and their long term clinical significance. J Clin Virol. 2013 Apr;56(4):312-5. doi: 10.1016/j.jcv.2012.12.011. Epub 2013 Jan 3. PMID: 23290387.
  3. Ordi-Ros J, Villarreal J, Monegal F, Sauleda S, Esteban I, Vilardell M. Anticardiolipin antibodies in patients with chronic hepatitis C virus infection: characterization in relation to antiphospholipid syndrome. Clin Diagn Lab Immunol. 2000 Mar;7(2):241-4. doi: 10.1128/CDLI.7.2.241-244.2000. PMID: 10702499; PMCID: PMC95855.