Outcomes in patients with atypical hemolytic uremic syndrome treated with eculizumab in a long-term observational study

Table 3 Genetic and autoimmune complement abnormalities in patients in the study

Complement Abnormality by Risk Level,^a n (%)	Never Discontinued (n = 51)	Discontinued (n = 42)
Complement Abnormality by Risk Level,^a n (%)	Never Discontinued (n = 51)	Reinitiated (n = 21)	Not Reinitiated (n = 21)
High risk	25 (49)	8 (38)	6 (29)
CFH	14 (27)^b	6 (29)	3 (14)^c
C3	6 (12)	1 (5)	1 (5)^d
CFH autoantibodies	5 (10)	1 (5)	1 (5)
CFB	0 (0)	0 (0)	1 (5)
Low/moderate risk	6 (12)	6 (29)	3 (14)
CD46 (MCP)	3 (6)^e	3 (14)	2 (10)
CFI	3 (6)^f	3 (14)	1 (5)
Deletions	0 (0)	0 (0)	1 (5)
CFHR1, CHFR3	0 (0)	0 (0)	1 (5)
No identified abnormality	20 (39)	7 (33)	11 (52)

CFB complement factor B; CFH complement factor H; CFHR1, CFHR3 complement factor H–related protein 1, complement factor H–related protein 3; CFI complement factor I; MCP membrane cofactor protein
^aRisk stratification as proposed by Goodship et al. [17]
^bIncludes one patient who also had a CFI mutation, and one patient who also had a CD46 (MCP) mutation
^cIncludes one patient who also had a C3 mutation
^dExcludes one patient who also had a CFH mutation
^eIncludes one patient who also had a CFI mutation. Excludes one patient who also had a CFH mutation
^fExcludes two patients who also had CFH or CD46 mutations

ISSN: 1471-2369