Coagulation Education: Treatment Options for Antiphospholipid Syndrome

EULAR 2024 (VIENNA)—Antiphospholipid syndrome (APS) has long been recognized as a cause of thrombosis and obstetric complications, but only recently have the microvascular and non-thrombotic manifestations of the disease received greater attention. In a sweeping session at EULAR 2024 titled, How to Treat: Antiphospholipid Syndrome, Maria Tektonidou, MD, PhD, professor of rheumatology, head of the Rheumatology Unit, National and Kapodistrian University of Athens, Greece, provided current insight into patient risk profiles, co-morbidities of and future treatment directions for APS.

Complications

Dr. Tektonidou began by noting that APS is notoriously complex and hard to treat. It’s a rare condition, with a paucity of randomized clinical trials. It also has multiple subtypes, including obstetric and thrombotic types.

Obstetric APS

Women with obstetric APS may experience significant morbidity during pregnancy. Specifically, they risk eclampsia, thrombosis, miscarriage and intrauterine growth restriction. Methods of monitoring these complications include screening for hypertension and proteinuria in the mother and Doppler ultrasonography of the umbilical/uterine artery for the fetus. And recent guidelines recommend more proactive measures.

According to the 2019 EULAR Recommendations for the Management of APS in Adults, women with a history of obstetric APS should be treated on the basis of their clinical history. Low-dose aspirin plus prophylactic-dose heparin (i.e., 5,000 units given subcutaneously every 8–12 hours) is recommended for patients who have experienced three or more spontaneous abortions before the 10th week of gestation or a fetal loss at 10 weeks or more. Patients with a delivery before 34 weeks of gestation due to eclampsia, severe pre-eclampsia or placental insufficiency, low-dose aspirin alone or with prophylactic-dose heparin is recommended based on the individual’s risk profile. For patients with an indication for prophylactic-dose heparin during pregnancy, clinicians should consider continuing heparin treatment for six weeks after delivery to reduce the risk of maternal thrombosis.¹

 Dr. Tektonidou explained that in patients with recurrent pregnancy complications despite these interventions, therapeutic-dose heparin, hydroxychloroquine and low-dose prednisone (the latter in the first trimester) can be considered. It should be noted that the ACR also has published a guideline for clinician decisions in these situations. For example, a conditional recommendation in the ACR guideline is to treat pregnant patients with daily, low-dose aspirin beginning in the first trimester to prevent or delay the onset of gestational hypertensive disease.²

Thrombotic APS

In patients with thrombotic APS, secondary thromboprophylaxis strategies depend a great deal on if the prior clot was provoked (i.e., incited by surgery, trauma, etc.) or unprokoved (i.e., with no clear inciting event) and if it was venous or arterial.