Background/Purpose:

Long-term anticoagulation is currently the standard treatment for arterial or venous antiphospholipid syndrome (APS). However, in daily clinical practice, the question of withdrawing anticoagulation may arise, without any evidence-based recommendations to guide this decision. We designed this study to properly assess the natural course of non-anticoagulated APS patients.

Methods:

Overall, 50 arterial or venous APS patients, recruited from our 210 APS patients database, were included if anticoagulation was withdrawn (n=30) or not introduced upon medical decision (n=20). Clinical and biological data were retrospectively collected from the inclusion to July 2014, either by consulting medical files, or by calling the patients or their attending physicians (if no follow-up was available in the past 6 months).

Results:

Mean age of the patients was 42.7 +/-17.4 years old. Systemic lupus erythematous was associated in 18% of the patients. The first APS thrombotic event was arterial for 21 patients, venous for 28 patients; one patient had catastrophic APS (CAPS). The median follow-up was 80.5 months (ranging from 14 to 144 months). No death occurred during the study. Nineteen patients had thrombotic relapses: 7 arterial events, 10 venous events and 2 CAPS. The thrombotic risk was especially increased during the first years of follow-up: 16.4% at 12 months, 31% at 48 months. After 120 months, the risk reached a plateau around 40%. Thus most of the relapses occurred before 4 years of follow-up. There was no difference in the repartition of cardiovascular risk factors nor in the initial antiphospholipid antibody profile between patients with or without relapse.

The univariate analysis showed that male gender, triple positivity at anticoagulation withdrawal, and persistence of at least one antiphospholipid antibody during follow-up were significantly associated with relapses. Conversely, long-term antiplatelet treatment and complete disappearance of antiphospholipid antibodies were significantly associated with a decrease in relapse risk.

In multivariate analysis, the absence of antiplatelet prescription (OR: 39, CI95% (4.1-372)) and the persistence of anticardiolipin antibodies during follow-up (OR: 16.3, CI95% (1.8-147)) remained significantly and independently associated with an increase risk of relapses.

Conclusion:

In our study, non-anticoagulated APS patients were exposed to a very high risk of thrombotic relapses, especially during the first 4 years of follow-up. The risk seems to increase when antiphospholipid antibodies persist for a long time, particularly in case of anticardiolipin antibodies positivity. If anticoagulation must be withdrawn, substitution by antiplatelet therapy appears to lower the risk of relapses.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/antiphospholipid-syndrome-without-anticoagulation-0utcome-of-50-patients/