Usefulness of the adjusted Global Antiphospholipid Syndrome Score (aGAPSS) to determine thrombotic risk in a cohort of patients with primary antiphospholipid syndrome

Abstract

Abstract: 

Objective: to assess the adjusted Global Antiphospholipid Syndrome Score (aGAPSS) instrument to estimate the risk of vascular thrombosis in primary antiphospholipid syndrome (pAPS). A retrospective study was done between 2013-2020, including patients with pAPS by Sydney criteria. The presence of arterial and venous thrombosis (tAPS), obstetrical comorbidity (oAPS), non-criteria manifestations (NCM), antiphospholipid antibodies (APLA), recurrent thrombosis and mortality were evaluated. Patients were grouped in tAPS, oAPS or both; the last one was used when the stratification was done. In the first visit, aGAPSS was calculated by adding: arterial hypertension: 1, hyperlipidemia: 3, moderate-high titles of anti-cardiolipin antibody (aCL): 5, anti-β2glicoprotein I (aB2GPI): 4 and lupus anticoagulant (LA): 4. aGAPSS ≥ 10 was considered high. Results: 85 patients entered the study, 74,11% completed the follow-up. 87,1% were women, mean age was 36 years (32,75-40,25), illness of 53 months (25-114). 62,35% had oAPS, 24,5% tAPS and 12,94% both. There were 18 arterial thrombosis, 11 venous thrombosis and 3 in both sites. Patients with tAPS had longer illness (p=0,04), higher rates of aB2GP1 (p=<0,001) and triple positivity (p=0,0003). The mean aGAPSS was 9 (5-12); in tAPS the mean value was 9 (8,75-13) and in oAPS 9 (5-9); p=0,008. There was no difference between patients with criteria manifestations and NCM. New trombosis (12,69%) happened in tAPS; the mean time was 26 months (15-49). There were 4 arterial thrombosis, 2 venous thrombosis and 2 in both sites. The mean aGAPSS in patients with recurrence was 13 (9-13), 62,50% had aGAPSS ≥ 10; and 87% had MNC. 4 (6,34%) patients died, all of them with recurrent thrombosis. aGAPSS ≥ 10 predicted new thrombosis (p=0,016). Patients with recurrence had a worse survival curve (p= 0,00000). Conclusion: assessing the risk of thrombosis in APS with aGAPSS could identify individuals at high risk of recurrence, monitor them, and intervene to prevent future events.