July 08, 2019
3 min read

EULAR: Identifying thrombotic, obstetric risk factors key to managing antiphospholipid syndrome

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Identifying high-risk factors for thrombotic and obstetric events is crucial in managing antiphospholipid syndrome, and all women with antiphospholipid syndrome should be counseled on contraceptive use, pregnancy planning and postmenopausal hormone therapy, according to new EULAR recommendations published in the Annals of the Rheumatic Diseases.

“Clinical practice in [antiphospholipid syndrome (APS)] is highly variable, in part because it is a rare disorder, and because knowledge about its diagnosis/classification, clinical spectrum and management is continuously advancing,” Maria G. Tektonidou, MD, PhD, of the University of Athens in Greece, and colleagues wrote. “There is a great heterogeneity among studies on the laboratory and clinical criteria used to define APS and the treatment approaches used over the past four decades. These factors make it often difficult to know the best approach to apply in daily practice.”

To develop a set of evidence-based recommendations for the management of antiphospholipid syndrome in adults, Tektonidou and colleagues formed a task force with members from 11 European countries, including 12 rheumatology or internal medicine specialists, two obstetricians, two physicians from vascular medicine or thrombosis centers, one health care professional and two patient representatives. Members first met in December 2017 to develop a set of 31 research questions, and later conducted a systematic literature review of relevant English-language articles published through Jan. 31, 2018.

Identifying high-risk factors for thrombotic and obstetric events is crucial in managing antiphospholipid syndrome, according to new EULAR recommendations.
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The literature search resulted in 7,534 articles, of which 670 were selected for a full-text review. Of those, 188 were deemed relevant and included in the analysis. The task force met for a second meeting in September 2018, where they discussed the literature review’s results and drafted recommendations in working groups. The final set of overarching principles and recommendations were then rated and put to a vote. The final recommendations were approved by all task force members and the EULAR executive committee prior to submission.

The task force drafted and finalized a set of three overarching principles and 12 recommendations for the management of antiphospholipid syndrome. Among those principles and recommendations are calls for identifying the presence of high-risk factors associated with thrombotic and obstetric events, and a warning that high-risk antiphospholipid antibody profile is linked with a greater risk for thrombotic and obstetric antiphospholipid syndrome.

Measures to reduce a patient risk can include screening for cardiovascular and venous thrombosis risk factors, and managing those risks, as well as counseling patients regarding treatment adherence and lifestyle changes. In addition, all women with antiphospholipid syndrome should be counseled on contraceptive use, pregnancy planning and postmenopausal hormone therapy.


The recommendations also suggest low-dose aspirin for patients with antiphospholipid antibodies who are asymptomatic, as well as those with systemic lupus erythematosus without prior thrombotic or obstetric antiphospholipid syndrome, and nonpregnant women with a history of obstetric antiphospholipid syndrome only — who all may have high-risk antiphospholipid antibody profiles. Patients with antiphospholipid syndrome who experience first unprovoked venous thrombosis should be treated with long-term vitamin K antagonists, with a target international normalized ratio (INR) of 2 to 3.

The task force also recommends vitamin K antagonists — with a target INR of 2 to 3 or 3 to 4 — for patients with antiphospholipid syndrome and first arterial thrombosis. The recommendations warn against using rivaroxaban in antiphospholipid syndrome with triple antiphospholipid antibody positivity. For patients with recurrent arterial or venous thrombosis, despite adequate treatment, the task force recommends the addition of low-dose aspirin, an increase of INR target to 3 to 4, or switching to low molecular weight heparin.

Women with previous obstetric antiphospholipid syndrome should receive combination treatment with low-dose aspirin and prophylactic dosage heparin during pregnancy. Those with recurrent pregnancy complications, should have their heparin increased to a therapeutic dose. The addition of hydroxychloroquine or an addition of low-dose prednisolone in the first trimester may also be considered.

“Better understanding of the pathophysiological mechanisms of APS will help to identify new therapeutic targets, and a balance between anticoagulation and immunomodulatory drugs for different APS manifestations,” Tektonidou and colleagues wrote. “In addition, studies that examine homogeneous patient groups can better evaluate the efficacy and safety of the currently available and new treatments. ... The task force members believe that these recommendations will help to improve the quality of care in patients with APS and foster future research by highlighting evidence gaps.” – by Jason Laday

Disclosure: The researchers report no relevant financial disclosures.