Table 3.
SIE incidence rate divided by diagnosis.
| Patients (n) | Months of FU (total) |
SIE (n) |
SIE (incidence *100 p/m/r) |
95% CI | Cumulative incidence (%) | |
|---|---|---|---|---|---|---|
| Global cohort | ||||||
| Diagnosis | ||||||
| ALPS-like | 47 | 3,132 | 13 | 0.42 | 0.24–0.71 | 27.66 |
| Other AICs | 49 | 3,488 | 3 | 0.09 | 0.03–0.27 | 6.12 |
| Overall | 96 | 6,620 | 16 | 0.24 | 0.14–0.39 | 16.67 |
| Treatment | ||||||
| MMF | 35 | 2,494 | 1 | 0.04 | 0.01–0.28 | 2.86 |
| SR | 33 | 2,091 | 9 | 0.43 | 0.22–0.83 | 27.27 |
| Sequential MMF+SR | 28 | 2,035 | 6 | 0.29 | 0.13–0.66 | 21.43 |
| Patients diagnosed after 2015 | ||||||
| Diagnosis | ||||||
| ALPS-like | 22 | 1,181 | 5 | 0.42 | 0.18–1.02 | 22.73 |
| Other AICs | 27 | 1,685 | 1 | 0.06 | 0.01–0.42 | 3.7 |
| Overall | 49 | 2,866 | 6 | 0.21 | 0.09–0.47 | 12.24 |
| Treatment |
Months of therapy
(total) |
|||||
| MMF | 16 | 573 | 0 | 0.00 | 0 | |
| SR | 17 | 558 | 3 | 0.54 | 0.17–1.67 | 17.65 |
| Sequential MMF+SR | 16 | 490 | 3 | 0.61 | 0.20–1.90 | 18.75 |