Hereditary Angioedema with and Without C1-Inhibitor Deficiency in Postmenopausal Women.
Age Factors
Aged
Aged, 80 and over
Alleles
Angioedemas, Hereditary
/ blood
Biomarkers
Complement C1 Inhibitor Protein
/ genetics
Disease Progression
Disease Susceptibility
Female
France
Genotype
Hormones
/ metabolism
Humans
Middle Aged
Mutation
Postmenopause
Retrospective Studies
Risk Factors
Severity of Illness Index
Symptom Assessment
Hereditary angioedema
aging
androgen
estrogen
female
menopause
Journal
Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137
Informations de publication
Date de publication:
01 2021
01 2021
Historique:
received:
01
05
2020
accepted:
26
10
2020
pubmed:
2
11
2020
medline:
15
1
2022
entrez:
1
11
2020
Statut:
ppublish
Résumé
Most types of hereditary angioedema (HAE) are worsened by endogenous or exogenous estrogens. Conversely, androgens can improve HAE with abnormal C1-Inhibitor (C1-INH) by increasing C1-INH concentrations. Menopause is associated with an extinction of ovarian estrogenic and androgenic secretion. There is currently insufficient information on postmenopausal women with HAE. The objective of this study was to describe the activity of HAE in postmenopausal women. This was a French retrospective, multicenter study in postmenopausal women with HAE with or without C1-INH deficiency/dysfunction. The patients were classified before and after menopause with a previously validated HAE disease severity score. We included 65 women from 13 centers in France. The mean age was 62.7± 9.2 years, and the mean time between menopause and inclusion was 12.5± 9.1 years. HAE was associated with C1-INH deficiencyin 88% (n = 57) of the patients, a mutation of factor 12 in 8% (n = 5), a mutation in plasminogen gene in one, and unknown HAE for two. The HAE course was not different after menopause in 46.1% (n = 30), improved in 38.5% (n = 25), and worsened in 15.4% (n = 10). Improvement was correlated with estrogen sensitivity of angioedema before menopause (p = 0.06 for improvement vs no effect or worsening). In addition, we observed that only ten women received treatment (transdermal or oral estradiol+ progestogen) for their menopause symptoms. Among them, only 3 experienced worsening of symptoms (2 on transdermal and 1 on oral estradiol). Following menopause, most women with HAE remain stable but some worsen. Improvement was mainly observed in patients with previous estrogen sensitivity. More research is required in menopausal women with HAE to better understand how to manage climacteric symptoms.
Identifiants
pubmed: 33130967
doi: 10.1007/s10875-020-00902-7
pii: 10.1007/s10875-020-00902-7
doi:
Substances chimiques
Biomarkers
0
Complement C1 Inhibitor Protein
0
Hormones
0
SERPING1 protein, human
0
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
163-170Références
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