Management of hereditary angioedema with normal C1Inh: a series of 163 French patients
HAE patients with a normal C1inh have specific clinical features, including a later age at first attack than HAE patients with a normal C1inh, high sensitivity to estrogens of HAE-FXII and the location of the HAE-PLG on the tongue. The treatments used for HAE patients with C1Inh deficiency appear to be effective and safe. Low-dose progestin-only pills are good contraceptive options.
Authors
- F. Pelletier (ORCID: https://orcid.org/0009-0008-2233-9466)
- G. Armengol
- David Launay (ORCID: https://orcid.org/0000-0003-1840-1817)
- Alexis Bocquet (ORCID: https://orcid.org/0000-0003-0182-0409)
- G. Hardy (ORCID: https://orcid.org/0009-0002-2678-3528)
- Laurence Bouillet (ORCID: https://orcid.org/0000-0001-8245-4767)
- Alban Deroux (ORCID: https://orcid.org/0000-0002-9098-7368)
- Delphine Gobert
- L. Sailler (ORCID: https://orcid.org/0000-0003-3482-6903)
- M Arnaud
- M.-C. Taquet
- Nicolas Ozanne
- Olivier Fain
- P Jeandel
- Isabelle Boccon-Gibod
Institutions
- Inserm (FR)
- Centre Hospitalier Universitaire de Grenoble (FR)
- Centre Hospitalier Universitaire de Lille (FR)
- Sorbonne Université (FR)
- Centre Hospitalier Universitaire de Besançon (FR)
- Hôpital Charles-Nicolle (FR)
- Centre Hospitalier Universitaire de Montpellier (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Hôpital Saint-Antoine (FR)
- Laboratoire National de Référence (MA)
- Hôpital l'Archet (FR)
- Lille Inflammation Research International Center (FR)
- Centre de Recherche Saint-Antoine (FR)
- Hôpital Purpan (FR)
- Institut de Biologie Structurale (FR)
- Hôpital Civil, Strasbourg (FR)
- Université de Strasbourg (FR)
- Université Grenoble Alpes (FR)
- Université de Franche-Comté (FR)
Publication Details
- Journal
- Orphanet Journal of Rare Diseases
- Published
- 2025-12-02
- DOI
- https://doi.org/10.1186/s13023-025-04155-8
- Primary Topic
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Type
- article
- Field-Weighted Citation Impact
- 0.00