Background: Hereditary angioedema caused by C1 inhibitor deficiency can be life threatening. Acute exacerbations are treated with intravenous purified, pasteurized C1 esterase inhibitor concentrate at doses of 500-1000 IU.
Methods: We reviewed the literature about safety and efficacy of the C1 inhibitor concentrate used in Canada (Berinert P) or relevant to it.
Results: Post-marketing experience since 1985 is reassuring. There were few adverse events and no transmission of infection with the pasteurized product. A number of case reports, retrospective studies and few randomized or non-randomized prospective studies have shown good efficacy measured as clinical resolution of symptoms or in time to resolution, time to relief or time to improvement.
Conclusions: There are numerous observational studies showing good efficacy and safety and a long post-marketing experience although few randomized placebo-controlled trials.