Background/Purpose: APS ACTION International Clinical Database and Repository (“Registry”) was created to study the natural course of disease over 10 years in persistently aPL-positive patients with/without other systemic autoimmune diseases. The objective of this preliminary analysis is to report the baseline characteristics of currently enrolled patients.
Methods: A secure web-based data capture system is used to store patient information including demographics, aPL/APS history, and aPL data. The inclusion criteria are positive lupus anticoagulant (LA) test based on the ISTH recommendations, medium-to-high titer (> 40U and/or > 99th percentile) anticardiolipin antibody (aCL) IgG/M/A, and/or medium-to-high titer anti-β2Glycoprotein-I (aβ2GPI) IgG/M/A tested at least twice within one year prior to enrollment. Clinical and blood collection (for core laboratory aPL confirmation and mechanistic studies) are performed once a year, or when patients develop new events. We used chi-square or Fisher’s exact test for group comparisons, and linear-by-linear association test to analyze for trend.
Results: As of June 2014, 408 patients have been recruited from 17 centers globally (mean age at entry: 43.7±12.8y; female: 310 [76%]; white: 261 [69%]; and other systemic autoimmune diseases: 130 [32%]). Table shows the distribution of the aPL-related events (historically and/or at registry entry) as well as the association between the aPL-related events and the aPL profile (triple, double, or single positivity based on inclusion tests). The results did not change when the single aPL group was restricted to LA-positive patients only (data not shown). The percentages of patients tested for LA, aCL, and aβ2GPI were 93%, 100%, and 76%, respectively in the double aPL-positive group; and 97%, 99%, and 77% in the single aPL-positive group. The percentages of the patients with positive LA, aCL, and aβ2GPI were 65%, 89%, and 39%, respectively in the double aPL-positive group; and 72%, 23%, and 5% in the single aPL-positive group.
|
aPL-related Events (# of Patients) N: 403 (5 excluded-pending aPL entry) |
Triple aPL 135 (34%) |
Double aPL 123 (30%) |
Single aPL 145 (36%) |
p- value* |
|
aPL, No Criteria Met (n:85) |
30 (35%) |
28 (33%) |
27 (32%) |
0.88 |
|
Thrombotic APS (n:221) |
76 (34%) |
64 (29%) |
81 (37%) |
0.21 |
|
Obstetric APS only (n:40) |
12 (30%) |
13 (33%) |
15 (38%) |
0.77 |
|
Thrombotic & Obstetric APS (n:57) |
17 (30%) |
18 (32%) |
22 (39%) |
0.57 |
|
Venous Thrombosis (n:169) |
59 (35%) |
44 (26%) |
66 (39%) |
0.34 |
|
Arterial Thrombosis (n:143) |
47 (33%) |
47 (33%) |
49 (34%) |
0.29 |
|
Catastrophic APS (n:3) |
3 (100%) |
0 (0%) |
0 (0%) |
0.044 |
|
Microthrombosis without CAPS (n:23) |
9 (39%) |
9 (39%) |
5 (22%) |
0.23 |
|
Fetal Loss (n:76) |
25 (33%) |
22 (29%) |
29 (38%) |
0.60 |
|
Premature Birth (EC/PEC/PI) (n:42) |
13 (31%) |
13 (31%) |
16 (38%) |
0.95 |
|
3 Consecutive Embryonic Losses (n:17) |
4 (24%) |
7 (41%) |
6 (35%) |
0.72 |
|
Livedo reticularis/racemosa (n:59) |
19 (32%) |
13 (22%) |
27 (46%) |
0.16 |
|
Persistent Thrombocytopenia1 (n:73) |
24 (33%) |
28 (38%) |
21 (29%) |
0.22 |
|
Autoimmune Hemolytic Anemia (n:23) |
9 (39%) |
8 (35%) |
6 (26%) |
0.31 |
|
Cardiac Valve Disease2 (n:34) |
18 (53%) |
10 (29%) |
6 (18%) |
0.02 |
|
aPL-associated Nephropathy2 (n:11) |
2 (18%) |
7 (64%) |
2 (18%) |
0.053 |
|
Skin Ulcers (n:21) |
5 (24%) |
8 (38%) |
8 (38%) |
0.58 |
|
Cognitive Dysfunction3 (n:10) |
3 (30%) |
4 (40%) |
3 (30%) |
0.27 |
|
MRI White Matter Changes (n:57) |
18 (32%) |
16 (28%) |
23 (40%) |
0.22 |
PEC: preeclampsia; EC: eclampsia; PI: placental insufficiency; (1) <100,000/mcL twice at least 12w apart; (2) Based on APS Classification Criteria Definitions; (3) neuropsychological test proven. * similar results with linear-by-linear association test for p-value trend.
Conclusion: Our preliminary analysis suggests that: a) there is no association between the aPL-related events and aPL profile (triple, double, or single positivity), except for Catastrophic APS and cardiac valve disease, based on a small number of patients; and b) LA positivity is the most important determinant of event risk in aPL-positive patients. Further analysis of the APS ACTION registry, i.e., detailed aCL/aβ2GPI titers, potential confounders, and core laboratory aPL profile, will clarify if triple, double, and single aPL profiles are associated with different risk profiles.
Disclosure:
D. Erkan,
APS ACTION,
2,
APS ACTION,
9;
D. Andrade,
APS ACTION,
2;
M. Tektonidou,
APS ACTION,
2;
A. Ugarte,
APS ACTION,
2;
A. Banzato,
APS ACTION,
2;
A. Tincani,
APS ACTION,
2;
P. L. Meroni,
APS ACTION,
2;
R. Cervera,
APS ACTION,
2;
P. R. Fortin,
APS ACTION,
2;
R. A. Levy,
APS ACTION,
2;
O. B. O. APS Action,
None.
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