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Hereditary angioedema attack: what happens to vasoactive mediators?
- Source :
-
International immunopharmacology [Int Immunopharmacol] 2020 Jan; Vol. 78, pp. 106079. Date of Electronic Publication: 2019 Dec 13. - Publication Year :
- 2020
-
Abstract
- Hereditary angioedema is a disabling, life-threatening condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) leading to bradykinin accumulation and recurrent episodes of edema attack. Vascular leakage is a complex process sustained by the coordinated production of several permeabilizing factors including vascular endothelial growth factors (VEGFs), angiopoietins (ANGPTs) and phospholipase A <subscript>2</subscript> enzymes (PLA <subscript>2</subscript> ). We previously reported that patients with C1-INH-HAE in remission have increased plasma levels of VEGFs, ANGPTs and secreted PLA <subscript>2</subscript> . In this study, we sought to analyze plasma levels of these mediators in 15 patients with C1-INH-HAE during the acute attack compared to remission. Plasma concentrations of VEGF-A, VEGF-C and VEGF-D were not altered during attack compared to remission. Moreover, VEGF-D concentrations were not altered also in remission phase compared to controls. Concentrations of ANGPT1, a vascular stabilizer, were increased during attacks compared to symptoms-free periods, whereas ANGPT2 levels were not altered. The ANGPT2/ANGPT1 ratio was decreased during angioedema attacks. Platelet activating factor acetylhydrolase activity was increased in patients with C1-INH-HAE in remission compared to controls and was decreased during angioedema attacks. Our results emphasize the complexity by which several vasoactive mediators are involved not only in the pathophysiology of C1-INH-HAE, but also during angioedema attacks and its resolution.<br /> (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)
- Subjects :
- 1-Alkyl-2-acetylglycerophosphocholine Esterase immunology
1-Alkyl-2-acetylglycerophosphocholine Esterase metabolism
Adolescent
Adult
Angiopoietin-1 immunology
Angiopoietin-1 metabolism
Angiopoietin-2 blood
Angiopoietin-2 immunology
Angiopoietin-2 metabolism
Bradykinin immunology
Bradykinin metabolism
Capillary Permeability immunology
Case-Control Studies
Complement C1 Inhibitor Protein genetics
Female
Healthy Volunteers
Hereditary Angioedema Types I and II blood
Hereditary Angioedema Types I and II genetics
Humans
Male
Middle Aged
Symptom Flare Up
Vascular Endothelial Growth Factor A blood
Vascular Endothelial Growth Factor A immunology
Vascular Endothelial Growth Factor A metabolism
Vascular Endothelial Growth Factor C blood
Vascular Endothelial Growth Factor C immunology
Vascular Endothelial Growth Factor C metabolism
Vascular Endothelial Growth Factor D blood
Vascular Endothelial Growth Factor D immunology
Vascular Endothelial Growth Factor D metabolism
Young Adult
1-Alkyl-2-acetylglycerophosphocholine Esterase blood
Angiopoietin-1 blood
Complement C1 Inhibitor Protein metabolism
Hereditary Angioedema Types I and II immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1878-1705
- Volume :
- 78
- Database :
- MEDLINE
- Journal :
- International immunopharmacology
- Publication Type :
- Academic Journal
- Accession number :
- 31841756
- Full Text :
- https://doi.org/10.1016/j.intimp.2019.106079