Subcutaneous C1‐Inhibitor Concentrate for prophylaxis during pregnancy and lactation in a patient with C1‐INH‐HAE

Abstract

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The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

“…126,[217][218][219][220] Recent studies show that subcutaneous twice-weekly administration of pdC1-INH at a dose of 60 U per kilogram bodyweight provided very good and dose-dependent preventive effects on the occurrence of HAE attacks. 205 The subcutaneous route may provide more convenient administration as well as maintain improved steady-state plasma concentrations of C1-INH compared to LTP with intravenous C1-INH, allowing for better symptom control.. [221][222][223][224]…”

“…140,141 Routine prophylaxis with pdC1-INH has been shown to be safe and effective, and it improves quality of life in patients with relatively frequent HAE attacks compared with acute treatment of individual HAE attacks. 210,222,223,[225][226][227] Thromboembolic events due to C1-INH concentrate use in HAE are rare, and patients who experience such events often have underlying thromboembolic risk factors (eg, implanted central venous catheters). [228][229][230][231][232][233] There are no known interactions with other medicinal products.…”

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

“… 205 The subcutaneous route may provide more convenient administration as well as maintain improved steady-state plasma concentrations of C1–INH compared to LTP with intravenous C1–INH, allowing for better symptom control. 221 , 222 , 223 , 224 …”

“… 140 , 141 Routine prophylaxis with pdC1-INH has been shown to be safe and effective, and it improves quality of life in patients with relatively frequent HAE attacks compared with acute treatment of individual HAE attacks. 210 , 222 , 223 , 225 , 226 , 227 …”

“…C1–INH is recommended as first-line therapy for pregnant or breast-feeding HAE-1/2 patients as it is safe and effective (Recommendation 22). 222 , 325 , 326 , 327 , 328 , 329 The use of ecallantide, lanadelumab and berotralstat in pregnancy is off label and not recommended as no published experience is available as of now. Although contraindicated by label, there are isolated case reports about the administration of icatibant during pregnancy with no maternal or fetal adverse effects reported.…”