Management overview for people with persistent antiphospholipid antibodies and people with antiphospholipid syndrome
Management for people with persistent antiphospholipid (aPL) antibodies is different to that for people with thrombotic or obstetric antiphospholipid syndrome. Management for various clinical scenarios is summarised in Management summary for people with either persistent antiphospholipid (aPL) antibodies or antiphospholipid syndrome, and described in detail under individual subheadings.
Management for people with persistent aPL antibodies [NB2] includes the following:
- For asymptomatic people with persistent aPL antibodies who are thromboembolism-free, low-dose aspirin is not routinely recommendedErkan, 2007.
- For asymptomatic people with persistent high-risk aPL-antibody profiles who are thromboembolism-free and trying to conceive, low-dose aspirin may be recommended.
- For asymptomatic people with systemic lupus erythematosus (SLE) (or other inflammatory connective tissue disease [NB3]) and persistent aPL antibodies who are thromboembolism-free, hydroxychloroquine is the mainstay of therapy; low-dose aspirin may be recommended for primary thromboprophylaxis under specialist advice.
Management for people with known thrombotic antiphospholipid syndrome includes the following:
- For people with thrombotic antiphospholipid syndrome, based on their first thromboembolic episode, full anticoagulation is required for their thromboembolic episode (with either intravenous unfractionated heparin or subcutaneous low molecular weight [LMW] heparin), followed by lifelong anticoagulation with warfarin (for secondary thromboprophylaxis). A direct-acting oral anticoagulant (DOAC) is not appropriate for the majority of patientsWilliams, 2022.
- For people with thrombotic antiphospholipid syndrome with recurrent thromboembolic episodes, full anticoagulation is required (as for a first thromboembolic episode). This should be followed by lifelong warfarin, in combination with 1 or more of: low-dose aspirin, dipyridamole plus aspirin, or hydroxychloroquine.
Management for people with known obstetric antiphospholipid syndrome includes the following:
- For nonpregnant people with a history of obstetric antiphospholipid syndrome only, low-dose aspirin may be considered after assessment of risks versus benefits.Arnaud, 2014Tektonidou, 2019
- For people with a history of obstetric antiphospholipid syndrome who are trying to conceive or pregnant, specialist management of anticoagulation is recommended throughout preconception, pregnancy and the postpartum period. Ideally, pregnancy should be a planned event, especially for patients with both SLE and antiphospholipid syndromeBranch, 2019Sammaritano, Bermas, 2020Tektonidou, 2019.
Management for people with both thrombotic antiphospholipid syndrome and a history of obstetric antiphospholipid syndrome includes the following:
- For people with thrombotic antiphospholipid syndrome who develop pregnancy complications [NB4], specialist management is recommended.
- For people with a history of obstetric antiphospholipid syndrome who develop a first thromboembolic episode [NB4], management is as per patients with a first thromboembolic episode in thrombotic antiphospholipid syndrome.
NB1: For detailed management advice, see the specific sections for these groups below.
NB2: Persistent aPL antibodies are defined as 2 positive assays at least 12 weeks apart.
NB3: SLE is the most common inflammatory connective tissue disease associated with antiphospholipid syndrome; less frequently, Sjögren syndrome, rheumatoid arthritis and systemic sclerosis may be associated.
NB4: Thrombotic and obstetric antiphospholipid syndrome can coexist.