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. 2016 Sep 27;54(3):412–431. doi: 10.1007/s12016-016-8585-0

Table 7.

Results of prophylactic setting studies: subtype idiopathic angioedema

Author Year Study design Size Disease duration (years) Previous therapy Therapy Dosage scheme Effect Follow-up (months)
Mansi [19] 2014 Cohort 44 n.r n.r. TA 3 g/day → 0.5–3 mg/day Reduction recurrences in 43 (98 %) n.r.
Wintenberger [55] 2014 Cohort 25 n.r. n.r. TA 2–2.5 g/day Attack frequency from 15.2 (range = 2–50) to 3.7 (0–18) per 6 months. No response in 6 (24 %) 6
Firinu [56] 2015 Cohort 16 n.r. AH + C TA 1.5–3 g/day 50 % attack frequency decrease in 8 (50 %), no response in 5 (31 %), other in 3 n.r.
Du-Thanh [58] 2010 CS 25 n.r. AH + C TA 3 g/day CR in 12 (48 %), PR in 11 (44 %), no response in 2 (8 %) 20
Cicardi [59] 1999 CS 15 Median 6 AH TA 3 g/day No further attacks in 8 (53 %), 7 attack frequency decreased by ≥75 % 10–282
Vela Vizcaino [48] 2014 CR 1 3 AH + C + E + C1INH TA 3 g/day Attack frequency decrease from weekly to 3/8 weeks n.r.
Saule [57] 2012 Cohort 20 n.r. AH Progestin n.r. Improvement in 19 (95 %) 32,4
Rijo Calderón [54] 2013 Cohort 4 n.r. AH + C + dapsone OMA 150–300/2–4 IR < 1 month n.r.
Azofra [60] 2015 CS 8 n.r. (AH + C +) TA OMA 300/4 IR = 2–14 days 6–12 m
Sands [61] 2007 CS-1 1 6 AH + C + E + H2 OMA 300/3 No further attacks 24
CS-2 1 4 AH + C + H2 OMA 375/2 No further attacks 7
CS-3 1 9 AH + C + LTRA + H2 OMA 300/4 1 minor attack in 2 years >12
Büyüköztürk [64] 2012 CS-1 1 10 AH + C + H+ O OMA 300/4 CR within 4 months n.r.
CS-2 1 15 AH + C + H + IVIG + I OMA 300/4 No further attacks n.r.
von Websky [72] 2013 CR 1 n.r. AH + C + LTRA + AB OMA 300/4 CR = 2 days 18
Suna [73] 2009 CR 1 19 AH + C + H + IVIG + I OMA 300/2 CR < 14 days 4.5
Stahl [43] 2014 CR 1 1 AH + C + E + H2 + TA + H + AB + LTRA + FFP + Ica C1INH 1000 U/ twice weekly Attack frequency decrease 5–7/month to 1.5/month n.r.
Bayer [74] 2013 CR 1 n.r. AH + C + E + H2 + LTRA + H + I C1INH n.r. Improvement after 2 doses of C1INH n.r.
Perez [65] 2010 CS-1 1 2.75 AH + C + I MTX 15/1 IR = 28 days n.r.

Dosage scheme presented as milligrams administered every x weeks, unless stated otherwise. The effect of treatment is presented as initial response (IR), complete response (CR), and partial response (PR)

CS case series, CR case report, n.r. not reported, AH antihistamine, C corticosteroids, E epinephrine, C1-inh C1 inhibitor concentrate, TA tranexamic acid, H2 H2 antagonist, FFP fresh frozen plasma, P pantoprazole, LTRA leukotriene receptor antagonist, H hormones, AB antibiotics, I immunosuppressant, MTX methotrexate, H hydroxychloroquine, IVIG intravenous immunoglobulin, Ica icatibant, O others