Emergency treatment of acute attacks in hereditary angioedema due to C1 inhibitor deficiency: what is the evidence?

Abstract

Hereditary angioedema (HAE) is caused by a deficiency in C1 esterase-inhibitor (C1-INH) and is characterised by skin swelling, abdominal pain and episodes of upper respiratory tract obstruction. Oedema of the larynx can result in rapid asphyxiation and requires emergency treatment. Three treatment options for emergency treatment of HAE are reviewed: fresh frozen plasma, solvent/detergent-treated plasma and C1-INH concentrate. It is concluded that while all three treatment options are theoretically effective in the emergency treatment of HAE, C1-INH is the treatment of choice.

International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

“…77,78 Secondary effects have been reviewed. 79 82 G. rhC1INH. rhC1INH (Rhucin, Ruconest; Pharming NV, Leiden, The Netherlands) is produced in transgenic rabbits.…”

Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

“…Upper airway involvement can be fatal, and mortality due to suffocation can be as high as 33% in inappropriately treated patients [7]. Estrogens, trauma, and stressful situations can worsen the course of disease and consequently, pregnancy and delivery may be special periods for female patients [8][9][10].…”

Peri‐operative management of a patient with hereditary angioedema undergoing laparoscopic cholecystectomy

“…A fibreoptic laryngoscope may be required. To facilitate re-intubation, a Cook exchange catheter could be used [26]. Measurement of cuff leak pressure intra-operatively allows detection of rapid increases in airway pressure that could trigger an acute attack of angioedema.…”

“…Replacement therapy using C1EI is the only therapy so far that has been successfully studied, for short term prophylaxis, in a prospective double-blind, placebocontrolled trial [26]. A non-randomised trial compared C1EI to conservative management in 193 episodes of laryngeal angioedema specifically [27]; the mean duration of laryngeal oedema was 15.3 h in patients who received C1EI concentrate and 101 h in those who did not.…”