The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey.
OBJECTIVE
The course of hereditary angioedema (HAE) and the efficacy and safety of human C1-INH concentrate were appraised during pregnancy and the postpartum period, in patients with HAE.


METHODS
Retrospective analysis of clinical data on 118 pregnancies (82 full-term and 36 abortions) in 41 female patients, extracted from the National HAE Registry, medical charts and patient diaries.


RESULTS
HAE attack frequency increases in 48% of pregnancies, whereas 33% of pregnancies were associated with mitigation of clinical signs and 19% of the pregnancies had no influence on the course of HAE, as compared to disease severity seen during the 2-year period preceding the pregnancy. During 46 full-term pregnancies, 26 patients reported attacks; 52% of these occurred in the third trimester. Abdominal attacks are the most common presentation of HAE during pregnancy. Attack number was significantly higher in patients who had sustained their initial attack before 8 years of age. Attack number increased during the third trimester if the fetus was afflicted by HAE. During the postpartum period, attacks occurred in 6/82 pregnancies. Patients received 91 vials of C1-INH concentrate altogether for the relief of acute attacks and for short- or long-term prophylaxis during pregnancy. This therapy was effective in all instances; no adverse effects were observed.


CONCLUSIONS
Pregnancy can either aggravate or mitigate edematous attacks, or alternatively, it may have no influence on the severity of the disease. According to our experience, C1-INH concentrate is an effective and safe therapeutic option during pregnancy.
Czaller
Ibolya
I
Semmelweis University, 3rd Department of Internal Medicine, Budapest, Hungary.
Visy
Beáta
B
Csuka
Dorottya
D
Füst
George
G
Tóth
Ferenc
F
Farkas
Henriette
H
D000028:Abortion, Induced / Q000706:statistics & numerical data; D000022:Abortion, Spontaneous / Q000453:epidemiology; D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D003613:Danazol / Q000627:therapeutic use; D036861:Delivery, Obstetric; D005260:Female; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy; D012189:Retrospective Studies; D055815:Young Adult
D000933:Antifibrinolytic Agents; D050718:Complement C1 Inhibitor Protein; D003613:Danazol