Prevalence, outcome and management of patients with SLE and secondary antiphospholipid antibody syndrome after aPL seroconversion

Abstract

Objective The withdrawal of oral anticoagulation (OAC) in patients with SLE and secondary aPL syndrome (SAPS) who become seronegative has not been clearly investigated to date. Our aim was to evaluate the prevalence of aPL seroconversion and the prognosis of SLE patients with SAPS who withdrew OAC after aPL negativization. Methods We retrospectively analysed data of all SLE patients (ACR criteria) with SAPS (Sydney criteria) prospectively followed-up in our clinic. aPL seroconversion was defined as negativization of lupus anticoagulant, aCL, and anti-β2glycoprotein-1 antibodies on two or more consecutive measurements, at least 12 weeks apart. OAC discontinuation was defined as the definitive withdrawal of all anticoagulants. Results Fifty-five out of 513 (10.7%) SLE patients had vascular SAPS. Sixteen patients (29.1%) became aPL seronegative during follow-up. Immunosuppressive therapy predicted aPL negativization (odds ratio 5.211, 95%CI 1.341, 20.243), whereas APS diagnosis prior to that of SLE (odds ratio 0.078, 95%CI 0.008, 0.799) and triple-positive profile (odds ratio 0.264, 95%CI 0.115, 0.609) were negative predictors of aPL negativization. OAC was discontinued in 13/55 patients (23.6%), after a median follow-up of 45 months (range 1–276) from aPL seroconversion. SLE-related modifiable risk factors for thrombosis were observed in 10/13 patients (77%) at the time of the thrombotic event. No thrombotic recurrences were observed during a mean follow-up time of 44 (19) months from OAC discontinuation. Conclusions Our results suggest that OAC can be safely discontinued in SLE patients who became persistently seronegative for aPL, at least when aPL-related thrombotic events occurred in presence of other thrombotic risk factors.

Lupus anticoagulant test persistence over time and its associations with future thrombotic events

“…However, the proportion of patients in our cohort who remained persistently LA positive (72%) was similar to the rates in other studies that used different definitions of LA stability and positivity (78% in the study by Gkrouzman et al, 71% in the study by Zen et al, and 87% in study by Erkan et al). 9,10,36 Finally, we only analyzed the effect of the first negative test on future events. We did not compare patients who became transiently LA negative to patients who became persistently negative, as the number of those latter patients was very low (only 14/42 patients remained persistently negative).…”

“…There have been a number of reports of safe discontinuation of anticoagulation in patients with APS who became persistently negative for aPL and our analysis cannot be applied to these patients. 10,37 However, these studies also included patients with only aCL or aβ2GPI, which are regarded as weaker risk factor for thrombosis. 35,[38][39][40] Similar to findings by Khawaja et al and Zen et al 10,29 , we found that the majority of patients who had a negative LA test subsequently had at least one additional positive test.…”

“…Treatment with immunosuppression was predictive of development of seronegativity, whereas triple positivity and diagnosis of APS prior to SLE were predictive of aPL stability over time. 10 Overall, limited prospective data on the variability of aPL over time and no data on the clinical implications of this variability, specifically, if it is predictive of future thrombotic events or mortality, are available We conducted a prospective observational cohort study on patients persistently positive for LA with and without a history of thrombosis or pregnancy complications: the Vienna Lupus Anticoagulant and Thrombosis Study (LATS). The aim of this present analysis of LATS is to evaluate the development of a negative LA test over time in these patients, to explore variables that are associated with a negative LA test, and to determine the effect of a negative LA test on the risk of future thrombotic events and mortality.…”

Coronary Artery Embolism and Antiphospholipid Syndrome: A Case Report and Review of the Literature

“…The titers of aPL frequently decrease and eventually become negative during the follow-up period [29]. The negativization has been related to a lower risk of thrombotic event, allowing to discontinuation of anticoagulation after aPL seroconversion seems to be reasonably safe, at least in primary APS [30].…”

Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review

“… 73 Indeed, thrombotic events frequently occur in the setting of antiphospholipid antibody syndrome (APS), whose prevalence in SLE is estimated to be around 30%. 74 – 76 Noteworthy, recurrent episodes of VTE may lead to chronic thromboembolic pulmonary disease and, eventually, pulmonary arterial hypertension (PAH). Therapy of pulmonary thromboembolism is mainly based on anti-coagulants.…”