(APS, Hugues syndrome)

Autoimmune disease, characterized by the association of obstetrical problems (loss of a fetus after the 10th week of pregnancy or recurrence of 3 spontaneous abortions before the 10th week of pregnancy without further explanation, severe preeclampsia) and/or thrombosis (venous or arterial) with the presence of antiphospholipid antibodies.

Antiphospholipid autoantibodies can present as:

• a circulating lupus anticoagulant, leading to an  in vitro prolonged ACT but with an in vivo thrombotic tendency
• anti-cardiolipin antibodies: rarely associated with thrombotic complications
• anti-beta2-glycoprotein I antibodies: these antibodies directed against the domain 1 of this protein are the most associated with clinical complications

Two types are to be distinguished:

-        primary APS (see this term): isolated presence of antiphospholipid antibodies without any other associated autoimmune disease; women are 4 to 5 times more affected than men.

-        secondary APS: associated with other autoimmune diseases such as disseminated lupus erythematosus; women are 9 times more affected than men.

The most common complications are thrombosis, thrombopenia and livedo reticularis ( mesh-shaped purple blue coloration of the skin); more rarely: proteinuria. There is often involvement of a heart valve, so regular echocardiography is recommended.

There is an extremely rare but severe form of APS, known as the "catastrophic" anti-phospholipid syndrome (see Asherson syndrome), which manifests itself by the simultaneous onset  of sudden severe thrombosis in at least 3 organs. This form may be a complication of the "classic" form of APS, but can also occur unexpectedly, i.e. without any known history of APS. It can be triggered by infection or surgery and/or a sudden discontinuation of anticoagulant treatment.

Treatment: vitamin K antagonists with an INR between 2 and 3 as a target. Aspirin is added if there is a history of arterial thrombosis.

Anesthetic implications: 

echocardiography; check blood platelets counts and INR;  vitamin K antagonist treatment: LMWH relay during the perioperative periods.

References : 

-        de Moerloose P, Tirefort-Dimitrova Y, Noger F, Spycher-Elbes R, Boehlen F.
Syndrome des anticorps antiphospholipides : beaucoup de nouveautés.
Rev Méd Suisse ; 2010 ; 6 : 298-301

Updated: April 2021