Table 1:
Clinical and Laboratory Characteristics (historically and/or at registry entry) of Persistently Antiphospholipid Antibody (aPL)-positive Patients (overall and stratified by systemic lupus erythematosus [SLE])
| Variables n(%) unless listed differently |
All aPL-positive Patients (n=623) | “aPL Only” Patients (n=426) | “aPL with SLE” Patients (n=197) | p |
|---|---|---|---|---|
| Demographics | ||||
| Age at Registry Entry (mean ± SD) | 44.2 ± 12.8 | 44.58 ± 12.9 | 43.24 ± 12.5 | 0.22 |
| Female | 459 (74%) | 307 (72%) | 152 (77%) | 0.18 |
| Race1 | ||||
| White | 397 (71%) | 274 (71%) | 123 (71%) | |
| Latin American Mestizos | 81 (15%) | 66 (17%) | 15 (9%) | |
| Asian | 48 (9%) | 28 (7%) | 20 (12%) | |
| Black | 21(4%) | 10 (3%) | 11 (6%) | |
| American Indian or Alaskan | 1 (0.2%) | 0 | 1 (0.6%) | |
| Native American | 0 | 0 | 0 | |
| Reported as “Other” | 12 (2%) | 9 (2%) | 3 (2%) | |
| Ethnicity2 | ||||
| United States, Canada, Europe | 261 (51%) | 183 (50%) | 78 (55%) | |
| Non-Latin American | 242 (48%) | 168 (46%) | 74 (48%) | |
| Latin American | 19 (4%) | 15 (4%) | 4 (3%) | |
| South America | 124 (24%) | 96 (26%) | 28 (20%) | |
| Afro-descendent | 16 (3%) | 8 (2%) | 8 (6%) | |
| Mestizo | 67 (13%) | 54 (15%) | 13 (9%) | |
| Caucasian | 41 (8%) | 34 (9%) | 7 (5%) | |
| Australia | 3 (0.6%) | 2 (0.5%) | 1 (0.7%) | |
| Aboriginal | 0 | 0 | 0 | |
| Not Aboriginal | 3 (0.6%) | 2 (0.5%) | 1 (0.7%) | |
| Other | 121 (24%) | 85 (23%) | 36 (24%) | |
| Clinical Manifestations | ||||
| Arterial Thrombosis (AT) | 193 (31%) | 139 (33%) | 54 (27%) | 0.26 |
| Venous Thrombosis (VT) | 272 (44%) | 185 (43%) | 87 (44%) | 0.13 |
| Microthrombosis (MT) | 37 (6%) | 27 (6%) | 10 (5%) | 0.23 |
| Any Vascular Event (AT/VT/MT) | 422 (68%) | 297 (70%) | 125 (64%) | 0.12 |
| Recurrent Vascular Event | 198/422 (47%) | 163/297 (55%) | 61/125 (49%) | 0.25 |
| Pregnancy History (ever) | 318 (51%) | 221(52%) | 97 (49%) | 0.06 |
| Pregnancy Morbidity | 210 (34%) | 154 (36%) | 56 (28%) | 0.1 |
| • ≥1 Fetal Death ≥ 10th Week of Gestation | 110 (18%) | 76 (18%) | 34 (17%) | 0.15 |
| • ≥1 Premature Birth < 34th Week of Gestation | 54 (9%) | 43 (10%) | 11 (6%) | 0.09 |
| • ≥3 Consecutive Unexplained Spontaneous Abortions < 10th Week of Gestation | 23 (4%) | 19 (5%) | 4 (2%) | 0.1 |
| Catastrophic APS | 6 (1%) | 4 (1%) | 2 (1%) | 0.24 |
| Livedo Reticularis/Racemosa | 80 (13%) | 52 (12%) | 28 (14%) | 0.48 |
| Persistent Thrombocytopenia | 124 (20%) | 69 (16%) | 55 (28%) | 0.001 |
| Autoimmune Hemolytic Anemia | 32 (5%) | 9 (2%) | 23 (12%) | <0.001 |
| Echocardiography Proven Cardiac Valve Disease | 50/518 (10%) | 30/349 (9%) | 20/169 (12%) | 0.31 |
| Biopsy Proven aPL-associated Nephropathy | 19/577 (3%) | 11/397 (3%) | 8/180 (4%) | 0.30 |
| Skin Ulcers | 32 (5%) | 21 (5%) | 11 (6%) | 0.12 |
| Cognitive Dysfunction | 19/148 (13%) | 14/90 (16%) | 5/58 (9%) | <0.001 |
| Complement Levels | ||||
| Low Complement C3 | 93/240 (39%) | 29/126 (23%) | 64/114 (56%) | <0.001 |
| Low Complement C4 | 92/240 (38%) | 30/126 (24%) | 62/114 (54%) | <0.001 |
| Antiphospholipid Antibodies | ||||
| Lupus Anticoagulant (LA) | 417 (67%) | 288 (68%) | 129 (66%) | 0.6 |
| Anticardiolipin Antibodies (aCL) | ||||
| IgG (cut-off 20 GPL)* | 357 (57%) | 245 (58%) | 112 (57%) | 0.87 |
| IgG (cut-off 40 GPL)** | 280 (45%) | 202 (47%) | 78 (40%) | 0.07 |
| IgM (cut-off 20 MPL)* | 223 (36%) | 154 (36%) | 69 (35%) | 0.79 |
| IgM (cut-off 40 MPL)** | 139 (22%) | 96 (23%) | 43 (22%) | 0.84 |
| IgA (cut-off 20 APL)* | 41/149 (28%) | 24/89 (27%) | 17/60 (28%) | 0.85 |
| IgA (cut-off 40 APL)** | 26/149 (17%) | 15/89 (17%) | 11/60 (18%) | 0.81 |
| Anti-β₂GPI Antibodies (aβ₂GPI) | ||||
| IgG (cut-off 20 GPL)* | 265 (43%) | 194 (46%) | 71 (36%) | 0.03 |
| IgG (cut-off 40 GPL)** | 208 (33%) | 157 (37%) | 51 (26%) | 0.01 |
| IgM (cut-off 20 GPL)* | 173 (28%) | 124 (29%) | 49 (25%) | 0.27 |
| IgM (cut-off 40 GPL)** | 114 (18%) | 81 (19%) | 33 (17%) | 0.5 |
| IgA (cut-off 20 GPL)* | 58/160 (36%) | 30/104 (29%) | 28/56 (50%) | 0.02 |
| IgA (cut-off 40 GPL)** | 37/160 (23%) | 19/104 (18%) | 18/56 (32%) | 0.04 |
| Double aPL-Positivity (LA + aCL, LA + aβ₂GPI, or aCL + aβ₂GPI) | 187 (30%) | 121 (28%) | 66 (34%) | 0.1 |
| Triple aPL Positivity (LA + aCL + aβ₂GPI) | 209 (34%) | 158 (37%) | 51 (26%) | 0.1 |
| Medications*** | ||||
| Low-dose Aspirin | 273 (44%) | 183 (43%) | 90 (44%) | 0.52 |
| Warfarin | 344 (55%) | 245 (58%) | 99 (50%) | 0.09 |
| Direct Oral Anticoagulants | 15 (2%) | 10 (2%) | 5 (3%) | 0.89 |
| Corticosteroids | 111 (18%) | 39 (9%) | 72 (37%) | <0.001 |
| Hydroxychloroquine | 276 (44%) | 133 (31%) | 143 (72%) | <0.001 |
| Immunosuppressive Agents | ||||
| Intravenous Immunoglobulin | 2 (0.3%) | 1 (0.2%) | 1 (1%) | 0.58 |
| Rituximab | 7 (1%) | 3 (1%) | 4 (2%) | 0.14 |
| Azathioprine | 46 (7%) | 11 (3%) | 35 (18%) | <0.001 |
| Cyclophosphamide | 8 (1%) | 2 (1%) | 6 (3%) | 0.008 |
| Cyclosporine | 4 (1%) | 2 (1%) | 2 (1%) | 0.43 |
| Methotrexate | 17 (3%) | 4 (1%) | 13 (7%) | <0.001 |
| Mycophenolate Mofetil | 45 (7%) | 11 (3%) | 34 (17%) | <0.001 |
*
more than low titer
**
more than moderate titer
***
at the time of registry entry.
1
Races were allowed to be collected in a total of 560 patients (387 in “aPL only” group and 173 in “aPL with SLE” group).
2
Ethnicities were allowed to be collected in a total of 509 patients (366 in “aPL only” group, 143 in “aPL with SLE” group).