Baseline characteristics of patients with thrombotic microangiopathy associated with SLE.
| Characteristics | ADAMTS13 Inhibitor–negative, n = 13 | ADAMTS13 Inhibitor–positive, n = 6 | p |
|---|---|---|---|
| Demographic data | |||
| Age, yrs | 21.8 ± 6.6 | 25.8 ± 11.6 | 0.351 |
| Female | 11 (84.6) | 6 (100.0) | 0.517 |
| New-onset lupus | 11 (84.6) | 5 (83.3) | 1.000 |
| Interval from disease onset to TMA, mos | 14.6 (1.4–93.3) | 0.8 (0.3–110.4) | 0.628 |
| Interval from Dx of SLE to onset of TMA, mos | 0.7 (0.6–2.4) | 0.4 (0.2–31.8) | 0.177 |
| Overall disease activity | |||
| SLEDAI | 20.2 ± 4.4 | 20.2 ± 8.6 | 0.997 |
| Complement C3 level, mg/dl | 37.0 ± 17.3 | 74.9 ± 14.4 | < 0.001 |
| Complement C4 level, mg/dl | 7.2 ± 5.4 | 10.8 ± 6.9 | 0.288 |
| Positive anti-dsDNA antibody | 7 (53.8) | 2 (33.3) | 0.628 |
| Hematological abnormalities | |||
| Severe ADAMTS13 deficiency | 0 (0) | 6 (100) | < 0.001 |
| Platelet count, ×109/l | 25.0 ± 17.8 | 7.3 ± 5.1 | 0.005 |
| Hemoglobin, g/l | 51.3 ± 11.8 | 62.8 ± 8.0 | 0.046 |
| Lactate dehydrogenase, U/l | 860.5 ± 453.3 | 1301.7 ± 680.5 | 0.110 |
| Renal involvement | |||
| Renal impairment, n | 13 (100.0) | 0 (0) | < 0.001 |
| Serum creatinine, μmol/l | 358.7 ± 193.4 | 64.3 ± 9.9 | < 0.001 |
| eGFR, ml/min/1.73 m2 | 21.6 ± 12.0 | 112.7 ± 18.0 | < 0.001 |
| Mean arterial pressure, mmHg | 117.5 ± 13.1 | 95.3 ± 13.6 | 0.008 |
| Proteinuria, g/d | 8.1 (5.2–14.0) | 0.6 (0.2–2.5) | 0.011 |
| CNS involvement, n | 3 (23.1) | 6 (100.0) | 0.003 |
Values are expressed as the mean ± SD or median (interquartile range). Attribute values are expressed as number (percentage). Significant p values are in bold face. CNS: central nervous system; Dx: diagnosis; eGFR: estimated glomerular filtration rate; SLE: systemic lupus erythematosus; SLEDAI: SLE Disease Activity Index; TMA: thrombotic microangiopathy.