Primary Anti-Phospholipid Antibody Syndrome: Real-World Defining Features of Rethrombosis in the Course of Disease
Table 1
Demographic, clinical, and laboratory parameters and risk profile at onset and end of follow-up, represented as frequencies, according to the occurrence of repeated thrombotic events in patients under anticoagulation.
Parameters
Overall (), (%)
(a) Recurrent thrombotic events (RTE) under anticoagulation (n = (10))
(b) No thrombotic events under anticoagulation (n = (44))
At onset:
Age (), median (IQR) at diagnosis
40 (31–49)
31 (26–41)
41.5 (32–50)
Male, (%)
17 (31)
5 (50)
12 (27)
Caucasian, (%)
50 (93)
10 (100)
40 (91)
Arterial hypertension, (%)
23 (43)
3 (30)
20 (45.5)
Dyslipidaemia, (%)
21 (39)
6 (60)
15 (34)
Active smoking
21 (39)
4 (40)
17 (39)
Type 2 diabetes mellitus, (%)
6 (11)
1 (10)
5 (11)
Obesity, (%)
14 (36)
4 (40)
10 (23)
Hormonal contraception, (%)
10 (28)
0
10 (23)
Immobility
5 (9)
0
5 (11)
Perioperative
1 (2)
0
1 (2)
Single ab positive, (%)
23 (43)
2 (20)
21 (48)
Double ab positive, (%)
9 (17)
1 (10)
8 (18)
Triple positive, (%)
22 (41)
7 (70)
15 (34)
LA, (%)
50 (93)
10 (100)
40 (91)
Anticardiolipin IgG, (%)
26 (48)
8 (80)
18 (41)
Anti-β(2)GPI IgG, (%)
26 (48)
7 (70)
19 (43)
High-risk profile at onset, (%)
49 (91)
10 (100)
39 (88)
aGAPSS ≥8, (%)
40 (74)
8 (80)
32 (73)
Clotting events prior to APS diagnosis:
Recurrent, (%)
22 (41)
3 (30)
19 (43)
Potentially life-threatening, (%)
30 (56)
7 (70)
23 (52)
Severe arterial, (%)
13 (24)
2 (20)
11 (25)
Severe venous, (%)
18 (33.3)
5 (50)
13 (29.5)
Time after diagnosis when clotting event reoccurred after APS diagnosis (), median (IQR)
—
5 (1.75–10.25)
NA
Type of event:
DVT,
4
NA
PE,
3
NA
Arterial,
3
NA
INR within therapeutic range when clotting event recurred after APS diagnosis, (%)
—
2/2 (INR unknown for 8 patients)
NA
% time in therapeutic range >75%, in the last year of follow-up (%)
25/46 (54) data not known in 18 patients
2/6 (33) data not known in 4 patients
23/40 (57.5) data not known in 14 patients
At end of follow-up:
Follow-up (), median (IQR)
11.5 (6–16)
13 (9–19.5)
11 (5–14)
Number of aPL and LA measurements over last three years of follow-up, median (IQR)
3 (2–3)
2.5 (2–3)
3 (2–3)
LA, (%)
30 (56)
8 (80)
22 (50)
Anticardiolipin IgG, (%)
22 (41)
7 (70)
15 (34)
Anti-β(2)GPI IgG, (%)
20 (37)
7 (70)
13 (29.5)
Sustained high-risk profile, (%)
31 (57)
8 (80)
23 (59) data not known in 5 patients
Reversion to negative status (aPL and LA), (%)
13 (24)
2 (20)
11 (25)
Time after APS diagnosis (years) at which reversion to negative status (aPL and LA) was detected, median (IQR)
