Reference | Number of patients | Drug/doses | Response: cr/pr/nr (number of patients) | |
---|---|---|---|---|
Medical influence of coagulation and contact activation pathway | ||||
Tranexamic acid | [9] | 2 | 1–4 g per day | cr 2 |
[10] | 1 | NA | cr 1 | |
[11] | 15 | 0.5–3 g per day | cr 8/pr 7 | |
[12] | 23 | 0.5–3 g per day | cr 12/pr 11 | |
[13] | 19 | 1–3 g per day | cr 4/pr 15 | |
[3] | 38 | 0.5–3 g per day | pr 37/nr 1 | |
Ecallantide | [15] | 1 | 30 mg (during attacks) | cr 1 |
[16] | 1 | 30 mg (during attacks) | cr 1 | |
[14] | 1 | NA | pr 1 | |
Icatibant | [17] | 1 | 30 mg (during attacks) | cr 1 |
[18] | 1 | 30 mg (during attacks) | cr 1 | |
[14] | 1 | NA | pr 1 | |
[19] | 2 | 30 mg (during attack) | pr 2 | |
C1-INH | [14] | 1 | 1000U twice weekly | cr 1 |
[19] | 2 | NA | pr 2 | |
[20] | 1 | 1000U twice weekly | cr 1 | |
Medical influence of the hormonal axis | ||||
Progestin | [21] | 20 | Various dosages depending the progestin | cr 6/pr 10/nr 4 |
Medical influence of IgE antibodies and mast cell (Omalizumab) | ||||
Omalizumab | [46] | 3 | 300 mg every 3–4 weeks; 375 mg every 2 weeks | Cr 3 |
[23] | 1 | 300 mg every 4 weeks initially, then reduced to 300 mg every 8 weeks | cr 1 | |
[24] | 1 | 300 mg every 4 weeks | cr 1 | |
[25] | 8 | 300 mg every 4 weeks initially, then reduced to doses and intervals according to symptoms | cr 8 | |
[27] | 2 | 375 mg every 2 weeks initially, then reduced to 375 mg every 4 weeks | cr 2 | |
[26] | 5 | 300 mg every 2–4 weeks | cr 5 | |
Other immunosuppressants or immunomodulatory therapies | ||||
Dapsone | [29] | 1 | 50 mg per day | cr 1 |
Ciclosporin | [28] | 1 | 300 mg per day | nr 1 |
FFP | [30] | 1 | 4 units | cr 1 |
Rituximab | [31] | 1 | 560 mg (375 mg/m2 body surface area) weekly for 4 weeks | cr 1 |
Cannabis | [32] | 1 | 20 g per month, inhaled 2–3× per week | cr 1 |