Table 1.

Baseline demographics of patients undergoing eculizumab discontinuation

N = 280
Age at time of eculizumab initiation 
Median (Q1-Q3) 28 (14.25-41.0) 
N/A 54 
Sex, n (%) 
Male 85 (40.1) 
Female 127 (59.9) 
N/A 68 
Prior TMA event , n (%) 
Yes 94 (79.7) 
No 24 (20.3) 
N/A 162 
Kidney status, n (%) 
Native 201 (88.9) 
Allograft 25 (11.1) 
N/A 54 
Dialysis during CM-TMA event, n (%) 
Yes 48 (55.8) 
No 38 (44.2) 
N/A 194 
Complement gene variant, n (%) 
Yes 168 (60) 
No variant 112 (40) 
Gene variant number , n (%) 
Single variant 148 (88.1) 
>1 concomitant variant 20 (11.9) 
Single-gene variant type, n (%) 
CFH 47 (31.8) 
MCP/CD46 35 (23.6) 
C3 11 (7.4) 
CFI 15 (10.1) 
Time on eculizumab (mo) 
Median (Q1-Q3) 8 (4.5-14.0) 
N/A 67 
Dialysis at eculizumab d/c, n (%) 
Yes 12 (6) 
No 187 (94) 
N/A 81 
Follow up after eculizumab d/c (mo) 
Median (95% CI) 23 (20-24) 
N/A 62 
N = 280
Age at time of eculizumab initiation 
Median (Q1-Q3) 28 (14.25-41.0) 
N/A 54 
Sex, n (%) 
Male 85 (40.1) 
Female 127 (59.9) 
N/A 68 
Prior TMA event , n (%) 
Yes 94 (79.7) 
No 24 (20.3) 
N/A 162 
Kidney status, n (%) 
Native 201 (88.9) 
Allograft 25 (11.1) 
N/A 54 
Dialysis during CM-TMA event, n (%) 
Yes 48 (55.8) 
No 38 (44.2) 
N/A 194 
Complement gene variant, n (%) 
Yes 168 (60) 
No variant 112 (40) 
Gene variant number , n (%) 
Single variant 148 (88.1) 
>1 concomitant variant 20 (11.9) 
Single-gene variant type, n (%) 
CFH 47 (31.8) 
MCP/CD46 35 (23.6) 
C3 11 (7.4) 
CFI 15 (10.1) 
Time on eculizumab (mo) 
Median (Q1-Q3) 8 (4.5-14.0) 
N/A 67 
Dialysis at eculizumab d/c, n (%) 
Yes 12 (6) 
No 187 (94) 
N/A 81 
Follow up after eculizumab d/c (mo) 
Median (95% CI) 23 (20-24) 
N/A 62 

N/A, not available.

Prior TMA event: authors of original publication indicated that episodes for which eculizumab was started was not their first TMA episode; however, the episode described in the current cohort was the first TMA episode for which eculizumab had been used as therapy.

Patients with only likely benign or benign variants were not included in this count.

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