Demographic and clinical characteristics of the study population
| . | Total (n = 11) . | Responders (n = 5) . | Nonresponders (n = 6) . |
|---|---|---|---|
| Age, median (IQR), y | 48 (24) | 46 (40) | 53 (23) |
| Female, n | 8 | 4 | 4 |
| APS duration, median (IQR), y | 12 (15) | 13 (37) | 11 (10) |
| Inaugural CAPS | 2 | 2 | 0 |
| History of venous thrombosis | 6 | 2 | 5 |
| History of arterial thrombosis | 3 | 1 | 2 |
| History of CAPS | 2 | 0 | 2 |
| History of obstetrical manifestation | 2 | 0 | 2 |
| Associated systemic lupus | 4 | 3 | 1 |
| aPL triple positivity at diagnosis | 11 | 5 | 6 |
| CAPS clinical features | |||
| Cardiac failure | 6 | 3 | 3 |
| Cutaneous (livedo reticularis, necrosis) | 8 | 5 | 3 |
| Renal failure | 10 | 4 | 6 |
| Cerebrovascular involvement | 4 | 3 | 1 |
| Venous thrombosis | 4 | 1 | 3 |
| Peripheral artery thrombosis | 2 | 1 | 1 |
| Adrenal ischemic hemorrhage | 3 | 2 | 1 |
| Diffuse alveolar hemorrhage | 3 | 1 | 2 |
| Liver infarct | 2 | 1 | 1 |
| Gastrointestinal involvement | 3 | 1 | 2 |
| Thrombocytopenia | 11 | 5 | 6 |
| Median (IQR) platelet count before, × 109/L | 19 (96) | 14 (54) | 79 (207) |
| Median (IQR) platelet count after, × 109/L | 89 (165) | 89 (113) | 111 (212) |
| MAHA | 6 | 4 | 2 |
| Dialysis | |||
| Before eculizumab | 4 | 1 | 3 |
| After eculizumab (short term) | 5 | 1 | 4 |
| Therapy before eculizumab | |||
| Anticoagulant therapy | 11 | 5 | 6 |
| PE | 10 | 4 | 6 |
| PEs, median (IQR) | 7 (13) | 8 (11) | 5 (14) |
| Corticosteroids | 11 | 5 | 6 |
| IVIG | 4 | 3 | 1 |
| Rituximab | 3 | 3 | 0 |
| Other immunosuppressive drug* | 2 | 2 | 0 |
| . | Total (n = 11) . | Responders (n = 5) . | Nonresponders (n = 6) . |
|---|---|---|---|
| Age, median (IQR), y | 48 (24) | 46 (40) | 53 (23) |
| Female, n | 8 | 4 | 4 |
| APS duration, median (IQR), y | 12 (15) | 13 (37) | 11 (10) |
| Inaugural CAPS | 2 | 2 | 0 |
| History of venous thrombosis | 6 | 2 | 5 |
| History of arterial thrombosis | 3 | 1 | 2 |
| History of CAPS | 2 | 0 | 2 |
| History of obstetrical manifestation | 2 | 0 | 2 |
| Associated systemic lupus | 4 | 3 | 1 |
| aPL triple positivity at diagnosis | 11 | 5 | 6 |
| CAPS clinical features | |||
| Cardiac failure | 6 | 3 | 3 |
| Cutaneous (livedo reticularis, necrosis) | 8 | 5 | 3 |
| Renal failure | 10 | 4 | 6 |
| Cerebrovascular involvement | 4 | 3 | 1 |
| Venous thrombosis | 4 | 1 | 3 |
| Peripheral artery thrombosis | 2 | 1 | 1 |
| Adrenal ischemic hemorrhage | 3 | 2 | 1 |
| Diffuse alveolar hemorrhage | 3 | 1 | 2 |
| Liver infarct | 2 | 1 | 1 |
| Gastrointestinal involvement | 3 | 1 | 2 |
| Thrombocytopenia | 11 | 5 | 6 |
| Median (IQR) platelet count before, × 109/L | 19 (96) | 14 (54) | 79 (207) |
| Median (IQR) platelet count after, × 109/L | 89 (165) | 89 (113) | 111 (212) |
| MAHA | 6 | 4 | 2 |
| Dialysis | |||
| Before eculizumab | 4 | 1 | 3 |
| After eculizumab (short term) | 5 | 1 | 4 |
| Therapy before eculizumab | |||
| Anticoagulant therapy | 11 | 5 | 6 |
| PE | 10 | 4 | 6 |
| PEs, median (IQR) | 7 (13) | 8 (11) | 5 (14) |
| Corticosteroids | 11 | 5 | 6 |
| IVIG | 4 | 3 | 1 |
| Rituximab | 3 | 3 | 0 |
| Other immunosuppressive drug* | 2 | 2 | 0 |
Unless otherwise stated, the data are the number of patients.
IVIG, intravenous immunoglobulin.
Endoxan and vinblastine.