Table 1.

Characteristics of 38 patients with atypical hemolytic uremic syndrome before and after eculizumab discontinuation

Patient CharacteristicsValue
Age at eculizumab discontinuation, yr30 (2–79)
 <189 (24)
 ≥1829 (76)
Complement genes variantsa21 (55)
 CFHb11 (29)
 MCP8 (21)
 C31 (2.5)
 CFI1 (2.5)
 No variant16 (42.5)
Anti-CFH antibodies1 (2.5)
Patients with ≥1 aHUS episode before eculizumab use9 (23)c
SCr at eculizumab start, mg/dl
 Adults7.9 (1.3–19)
 Children1.9 (0.2–3.9)
Patients requiring dialysis at eculizumab start18 of 38 (47)d
Duration of eculizumab treatment, mo17.5 (2–50)
SCr at eculizumab discontinuation, mg/dl
 Adults1 (0.7–2.8)
 Children0.4 (0.25–0.46)
Patients with CKD4 (11)
Duration of follow-up after eculizumab discontinuation, mo22 (5–43)
Patients with aHUS relapse12 of 38 (32)
 CFH8 of 11 (72)
 MCP4 of 8 (50)
 No variant0 of 16 (0)
Time to relapse, mo7.5 (3–29)
SCr at last follow-up, mg/dl
 Adults1 (0.7–2.8)
 Children0.44 (0.25–1)
  • Values are shown as n (%) or median (full range). CFH, complement factor H; MCP, membrane cofactor protein; CFI, complement factor I; aHUS, atypical hemolytic uremic syndrome; SCr, serum creatinine.

  • a Variant with minor allele frequency <0.1%.

  • b Including one combined CFH/CFI variants (p.Ile340Thr).

  • c Patients 2, 4, 9, 12–16, and 31.

  • d One child (patient 27 required dialysis).