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30-05-2019 | Rheumatology | News | Article

EULAR publishes recommendations for managing antiphospholipid syndrome

medwireNews: Evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adult patients have been issued by EULAR.

“[T]hese recommendations will help to improve the quality of care in patients with APS and foster future research by highlighting evidence gaps,” write the guideline authors in the Annals of the Rheumatic Diseases.

The taskforce – comprising 12 specialists in rheumatology or internal medicine, two obstetricians, two specialists in vascular medicine or thrombosis, one healthcare professional, and two patient representatives – developed three main principles and 12 recommendations on the basis of a systematic literature review and expert opinion.

Maria Tektonidou (University of Athens, Greece) and colleagues’ overarching principles state that identifying risk factors for thrombotic and obstetric adverse events “is critical in patient management,” noting that the high-risk antiphospholipid antibody (aPL) profile is a key risk factor.

They also specify that guidelines for cardiovascular disease prevention in the general population should be followed, and that individuals treated with vitamin K antagonists should be given counseling about medication adherence, monitoring, and family planning.

In their 12 specific recommendations, Tektonidou and co-authors advocate the use of low-dose aspirin for primary thromboprophylaxis. They discuss a number of options for secondary thromboprophylaxis, recommending vitamin K antagonists for people with definite APS and first venous thrombosis, with addition of low-dose aspirin or a switch to low molecular weight heparin if international normalized ratio targets are not reached.

The taskforce cautions that rivaroxaban “should not be used in patients with triple aPL positivity due to the high risk of recurrent events.”

For obstetric APS, the guidelines recommend that low-dose aspirin “should be considered” for women with a high-risk aPL profile but no history of pregnancy complications or thrombosis, and suggest combination therapy with low-dose aspirin and heparin for specific patient populations, such as those with a history of delivery before 34 weeks of gestation due to eclampsia or placental insufficiency.

And for catastrophic APS, Tektonidou et al say that therapy with glucocorticoids and heparin in combination with plasma exchange or intravenous immunoglobulins “is recommended over single agents or other combinations of therapies.” They advocate B-cell depletion with agents such as rituximab or complement inhibition with agents such as eculizumab for refractory disease, and stress the importance of “[p]rompt treatment of infections” in all catastrophic APS patients who are positive for aPL.

The authors say that the “main challenge” in developing their guidelines “was the low certainty of evidence,” with many studies having participants with various clinical features and no separation of arterial and venous thrombosis.

“This resulted in only indirect evidence for many of the treatment decisions that were examined,” they write.

Tektonidou and colleagues conclude that “well-designed observational studies and [randomized controlled trials] of homogeneous patient populations” are needed to “increase the quality of evidence for the currently used treatments and answer questions about controversial issues and new potential therapies.”

By Claire Barnard

medwireNews is an independent medical news service provided by Springer Healthcare. © 2019 Springer Healthcare part of the Springer Nature group

Ann Rheum Dis 2019; doi:10.1136/annrheumdis-2019-215213

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