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Table 2 Comparison of the therapies most often applied in InH-AAE

From: Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials

  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
  1. cr complete remission, pr partial remission, nr no response, NA not available, FFP fresh frozen plasma