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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