Overview
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and recurrent thrombotic events or pregnancy morbidity 3. Secondary APS occurs in the context of another autoimmune disease, most commonly systemic lupus erythematosus (SLE) 3.Diagnosis
Diagnosis requires the presence of antiphospholipid antibodies (anticardiolipin, anti-beta-2 glycoprotein I, or lupus anticoagulant) along with clinical criteria such as thrombosis or pregnancy morbidity 3.
Risk stratification for pregnancy includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension, and medication use 3.
Assessment of disease activity, renal function, and serological markers is important for diagnosing flares and monitoring obstetrical outcomes 3.Management
Antithrombotic therapy is a cornerstone of management for venous thromboembolism (VTE) in APS 12.
For women with APS, family planning should be discussed early after diagnosis 3.
Hormonal contraception and menopause replacement therapy may be used in patients with stable/inactive disease and low thrombosis risk 3.
Fertility preservation with gonadotropin-releasing hormone analogues should be considered before alkylating agent use 3.
Assisted reproduction techniques can be used in patients with stable/inactive disease 3.Special Populations
Pregnancy: Most women with APS can have successful pregnancies with measures to reduce maternal or fetal risks 3. Patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin during assisted reproduction 3. Fetal monitoring includes Doppler ultrasonography and fetal biometry, especially in the third trimester 3.Key Recommendations
Family planning should be discussed as early as possible after diagnosis in women with SLE and/or APS 3. (Evidence: Expert opinion)
Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis 3. (Evidence: Expert opinion)
Patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin when undergoing assisted reproduction techniques 3. (Evidence: Expert opinion)References
1 Stevens SM, Woller SC, Kreuziger LB, Bounameaux H, Doerschug K, Geersing GJ et al.. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest 2021. link
2 Stevens SM, Woller SC, Baumann Kreuziger L, Bounameaux H, Doerschug K, Geersing GJ et al.. Executive Summary: Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest 2021. link
3 Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N et al.. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Annals of the rheumatic diseases 2017. link