Initial treatment efficacy | |||||
---|---|---|---|---|---|
Literature (n = 79) | Present series (n = 20) | Pooled | |||
Treatment | n | Efficacy | n | Efficacy | Efficacy |
Corticosteroids alone | 75 | 51 (68%) | 20 | 10 (50%) | 61/95 (64%) |
IVIg | 21 | 10 (48%)a | 6 | 1 (17%) | 11/27 (41%) |
Cyclosporine | 15 | 12 (80%) | 3 | 1 (33%) | 13/18 (72%) |
Anakinra (IL1Ra) | 4 | 4 (100%) | 5 | 4 (80%) | 8/9 (89%) |
Etoposide | 0 | 0 (0%) | 3 | 2 (67%) | 2/3 (67%) |