Treatment of 139 Pregnancies in Antiphospholipid-positive Women Not Fulfilling Criteria for Antiphospholipid Syndrome: A Retrospective Study

Ross, Teresa Del; Ruffatti, Amelia; Visentin, Maria Serena; Tonello, Marta; Calligaro, Antonia; Favarò, Maria; Hoxha, Ariela; Punzi, Leonardo

doi:10.3899/jrheum.120576

Cited by 48 publications

(29 citation statements)

References 24 publications

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“…Data from one placebo-controlled RCT of LDA in six women with SLE42 and data from three low-quality studies (two RCTs, one retrospective cohort)43–45 of women without SLE found no difference in the prevalence of live births with use of LDA. However, these studies did not specifically include women with a high-risk aPL profile.…”

Section: Recommendationsmentioning

confidence: 95%

EULAR recommendations for the management of antiphospholipid syndrome in adults

et al. 2019

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The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. Based on evidence from a systematic literature review and expert opinion, overarching principles and recommendations were formulated and voted. High-risk antiphospholipid antibody (aPL) profile is associated with greater risk for thrombotic and obstetric APS. Risk modification includes screening for and management of cardiovascular and venous thrombosis risk factors, patient education about treatment adherence, and lifestyle counselling. Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2–3. In patients with APS with first arterial thrombosis, treatment with VKA with INR 2–3 or INR 3–4 is recommended, considering the individual’s bleeding/thrombosis risk. Rivaroxaban should not be used in patients with APS with triple aPL positivity. For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3–4 or switch to low molecular weight heparin may be considered. In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

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Section: Recommendationsmentioning

confidence: 95%

EULAR recommendations for the management of antiphospholipid syndrome in adults

et al. 2019

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“…Evidence on how to manage women who meet serological criteria for APLA but do not meet clinical criteria for obstetric APS, or alternatively those with three or more miscarriages and low titer APLA not meeting serologic criteria for APLA is limited to observational studies with conflicting results. 173, 174 Therapy with aspirin and LMWH could be considered, on an individual basis, for women with a high-risk APLA profile, concomitant SLE, or pre-eclampsia. Further clinical studies are needed to determine the optimal management of APLA-positive women.…”

Section: Management Of Obstetric Apsmentioning

confidence: 99%

Diagnosis and management of the antiphospholipid syndrome

2017

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Antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (APLA). Laboratory diagnosis of APLA depends upon the detection of a lupus anticoagulant, which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin (aCL) and anti-β2-glycoprotein-1 (β2GPI) antibodies. APLA are primarily directed towards phospholipid binding proteins. Pathophysiologic mechanisms underlying thrombosis and pregnancy loss in APS include APLA induced cellular activation, inhibition of natural anticoagulant and fibrinolytic systems, and complement activation, among others. There is a high rate of recurrent thrombosis in APS, especially in triple positive patients (patients with lupus anticoagulants, aCL and anti-β2GPI antibodies), and indefinite anticoagulation with a vitamin K antagonist is the standard of care for thrombotic APS. There is currently insufficient evidence to recommend the routine use of direct oral anticoagulants (DOAC) in thrombotic APS. Aspirin with low molecular weight or unfractionated heparin may reduce the incidence of pregnancy loss in obstetric APS. Recent insights into the pathogenesis of APS have led to the identification of new potential therapeutic interventions, including anti-inflammatory and immunomodulatory therapies. Additional research is needed to better understand the effects of APLA on activation of signaling pathways in vascular cells, to identify more predictive biomarkers that define patients at greatest risk for a first or recurrent APLA-related clinical event, and to determine the safety and efficacy of DOACs and novel anti-inflammatory and immune-modulatory therapies for refractory APS.

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“…The management strategies differ, based on the risk profile of each pregnancy. Low dose aspirin alone is generally recommended for asymptomatic women with only persistently positive aPL and no prior event, despite limited evidence [63, 64]. The group with recurrent early losses or one or more late fetal loss, but no history of systemic thrombosis, is termed Obstetric APS.…”

Section: Ante-natal Management In Sle Patientsmentioning

confidence: 99%

Managing lupus patients during pregnancy

Lateef

Petri

2013

Best Practice & Research Clinical Rheumatology

203

121

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Systemic lupus erythematosus (SLE) is an autoimmune disease, primarily affecting young females. Pregnancy in a woman with SLE remains a high risk situation with higher maternal and fetal mortality and morbidity. Although live births are achieved in majority of the pregnancies, active disease and major organ involvement can negatively affect the outcomes. Higher risk of fetal loss, pre-term birth, intra-uterine growth restriction and neonatal lupus syndromes are major fetal issues. Mothers are faced with disease flares, pre-eclampsia and other complications. Disease flares during SLE pregnancy pose the unique issue of recognition and differentiation between physiologic changes and disease state. Similarly pre-eclampsia and lupus nephritis may lead to diagnostic confusion. Treatment choices during pregnancy are limited to a few safe drugs, further restricting the options. Refractory pregnancy loss associated with anti-phospholipid antibodies and complete heart block associated with anti-Ro antibodies remain unresolved issues. A multidisciplinary approach, with close monitoring, is essential for optimal outcomes.

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Treatment of 139 Pregnancies in Antiphospholipid-positive Women Not Fulfilling Criteria for Antiphospholipid Syndrome: A Retrospective Study

Abstract: LDA treatment does not appear to improve pregnancy outcome in low-risk women not fulfilling the criteria for APS. Because antibody profile seems to influence pregnancy outcome, further studies of patients stratified according to their antibody profile are warranted.

Cited by 48 publications

References 24 publications

EULAR recommendations for the management of antiphospholipid syndrome in adults

EULAR recommendations for the management of antiphospholipid syndrome in adults

Diagnosis and management of the antiphospholipid syndrome

Managing lupus patients during pregnancy

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