Management of very high risk pregnancy with secondary anti-phospholipid syndrome and triple positivity to the anti-phospholipid antibodies

Abstract

There is a significantly increased risk of pregnancy complications in women with anti-phospholipid syndrome (APS). The risk is further heightened in those with previous arterial or venous thromboembolism and so-called 'triple positivity' for anti-phospholipid antibodies (i.e., when lupus anticoagulant, and anti-cardiolipin (aCL) and anti-β2 glycoprotein-I (anti-β2Gp-I) antibodies are all detected). Management of these cases is extremely difficult and little is available in the medical literature to guide therapy. This report describes the use of regular plasma exchanges (PEx) to bring about a successful pregnancy outcome in a woman with secondary APS and previous recurrent miscarriages. The patient was also anticoagulated with enoxaparin and administered aspirin, prednisolone, azathioprine and hydroxychloroquine. Through regular PEx and immunomodulation therapy, levels of aCL and anti-β2Gp-I antibodies were monitored and documented to fall as pregnancy progressed. Although the outcome in this case was successful, further experience is required before this regimen can be accepted as the standard of care for these patients at very high risk of pregnancy loss.

Additional Treatments for High-Risk Obstetric Antiphospholipid Syndrome: a Comprehensive Review

“…Mar et al [26] 2014 Case report 0.5 mg kg −1 month −1 from week 8 (7 total doses) Favourable Watanabe et al [27] 2014 Case series 400 mg kg −1 day −1 for 5 days from week 6 or 7 Live birth rate 100 % Rose et al [28] 2014 Case report 1 g kg −1 week −1 from week 9 IUGR, stillbirth at 21 weeks IVIG intravenous immunoglobulins, LDA low-dose aspirin, IUGR intrauterine growth restriction Table 2 Effect of apheresis procedures administered in addition to other treatments in high-risk pregnancies of patients with antiphospholipid syndrome…”

“…Bortolati et al [35] 2009 Case reports Pt 1: Plasma exchange plus IVIG, therapeutic heparin and low-dose aspirin Favourable Pt 2: Immunoadsorption plus IVIG, therapeutic heparin and low-dose aspirin Rose et al [28] 2014 Case reports Plasma exchange, therapeutic heparin, low-dose aspirin, HCQ, low-dose prednisolone and azathioprine…”

“…Whilst steroids and aphaeresis procedures are currently considered additional therapies [72,73], data on the usefulness of IVIG in obstetric APS are conflicting [18][19][20][21][22][23][24][25][26][27][28][74][75][76]. The use of IVIG in pregnant APS women is rare and is generally considered a second-line treatment when other more standardized therapies have failed [77].…”

“…Both extracorporeal blood purification techniques are known to lower [87,88]. Their use in pregnant APS patients has, nevertheless, been circumscribed, and descriptions in the literature are limited to case reports [28,30,31,35,36] and case series [32][33][34]37]. Both aphaeresis procedures have, in any case, always been administered in addition to other treatments, including steroids, heparin/LDA or IVIG, and have been associated to a live birth rate ranging between 71.4 and 100 % of the treated cases [33,34,37].…”

“…The protocols outlined above fail in about 20 % of pregnant APS women [17], and additional treatments including intravenous immunoglobulins (IVIG) [18][19][20][21][22][23][24][25][26][27][28] (Table 1), low-dose prednisolone [29], or aphaeresis procedures such as plasma exchange or immunoadsorption [30][31][32][33][34][35][36][37] have, at times, been combined with conventional therapies (Table 2). Due to the low number of treated cases, the efficacy of these protocols is at present not well established.…”