Table 1.

Baseline characteristics of patients with thrombotic microangiopathy associated with SLE.

CharacteristicsADAMTS13 Inhibitor–negative, n = 13ADAMTS13 Inhibitor–positive, n = 6p
Demographic data
  Age, yrs21.8 ± 6.625.8 ± 11.60.351
  Female11 (84.6)6 (100.0)0.517
  New-onset lupus11 (84.6)5 (83.3)1.000
  Interval from disease onset to TMA, mos14.6 (1.4–93.3)0.8 (0.3–110.4)0.628
  Interval from Dx of SLE to onset of TMA, mos0.7 (0.6–2.4)0.4 (0.2–31.8)0.177
Overall disease activity
  SLEDAI20.2 ± 4.420.2 ± 8.60.997
  Complement C3 level, mg/dl37.0 ± 17.374.9 ± 14.4< 0.001
  Complement C4 level, mg/dl7.2 ± 5.410.8 ± 6.90.288
  Positive anti-dsDNA antibody7 (53.8)2 (33.3)0.628
Hematological abnormalities
  Severe ADAMTS13 deficiency0 (0)6 (100)< 0.001
  Platelet count, ×109/l25.0 ± 17.87.3 ± 5.10.005
  Hemoglobin, g/l51.3 ± 11.862.8 ± 8.00.046
  Lactate dehydrogenase, U/l860.5 ± 453.31301.7 ± 680.50.110
Renal involvement
  Renal impairment, n13 (100.0)0 (0)< 0.001
  Serum creatinine, μmol/l358.7 ± 193.464.3 ± 9.9< 0.001
  eGFR, ml/min/1.73 m221.6 ± 12.0112.7 ± 18.0< 0.001
  Mean arterial pressure, mmHg117.5 ± 13.195.3 ± 13.60.008
  Proteinuria, g/d8.1 (5.2–14.0)0.6 (0.2–2.5)0.011
CNS involvement, n3 (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.