Experience with Intravenous Plasma-Derived C1-Inhibitor in Pregnant Women with Hereditary Angioedema: A Systematic Literature Review.

Journal Article (Journal Article;Systematic Review)

Consensus guidelines recommend plasma-derived C1 inhibitor (C1-INH) as first-line treatment in pregnant women with hereditary angioedema (HAE). We conducted a systematic review of the literature that describes experience with plasma-derived C1-INH during pregnancy. A literature search of PubMed was conducted in November 2018 using variants of "hereditary angioedema" and "pregnancy." English language articles that presented original data about the use of plasma-derived C1-INH during pregnancy were selected for data extraction. The search returned 253 unique records, of which 40 described the use of C1-INH during pregnancy (91 patients, 136 pregnancies). When the number of doses was reported, a total of 1562 doses were administered ranging from 500 to 3000 IU. Infusions were administered during all 3 trimesters and were most commonly administered during the third trimester. Overall, 1,490,500 IU of plasma-derived C1-INH were administered during pregnancy. Of the 128 fetuses for which outcomes were reported, 3 (2%) resulted in spontaneous abortion, 1 (1%) was stillborn, and 1 (1%) was a vanishing twin. Use of plasma-derived C1-INH in women with HAE during pregnancy has been widely reported in the scientific literature and has a favorable safety profile, supporting treatment guideline recommendations.

Full Text

Duke Authors

Cited Authors

  • Brooks, JP; Radojicic, C; Riedl, MA; Newcomer, SD; Banerji, A; Hsu, FI

Published Date

  • June 2020

Published In

Volume / Issue

  • 8 / 6

Start / End Page

  • 1875 - 1880.e3

PubMed ID

  • 32251736

Electronic International Standard Serial Number (EISSN)

  • 2213-2201

Digital Object Identifier (DOI)

  • 10.1016/j.jaip.2020.03.009


  • eng

Conference Location

  • United States