Hereditary angioedema attack: what happens to vasoactive mediators?

Ferrara, Anne Lise; Bova, María; Petraroli, Angelica; Veszeli, Nóra; Galdiero, Maria Rosaria; Braile, Mariantonia; Marone, Giancarlo; Cristinziano, Leonardo; Marcella, Simone; Modestino, Luca; Farkas, Henriette; Loffredo, Stefania

doi:10.1016/j.intimp.2019.106079

Cited by 9 publications

(11 citation statements)

References 25 publications

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“…eNOS is known to increase in patients with HAE both in the attack and in the remission phase and there are reports of significant differences in levels of other vasoactive mediators-vascular endothelial growth factors (VEGFs), angiopoietins (ANGPTs) and phospholipase A2 enzymes (PLA2) [29,39]. We cannot exclude that the edema of the phar-…”

Section: Discussionmentioning

confidence: 98%

Exhaled Nitric Oxide Level in Pharynx Angioedema

Moos

Zając

Brzoza

2022

JCM

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Airway inflammation is related to increased nitric oxide production. It can be assessed noninvasively with exhaled nitric oxide measurement. As airway inflammation was supposed to be present in chronic urticaria and angioedema patients we hypothesized increased exhaled nitric oxide in this group. Twenty-six symptomatic chronic urticaria patients with an acute episode of pharynx angioedema (17 women and 9 men, median age 35) were included in the study group. None of the patients reported a history of asthma, allergic rhinitis or cigarette smoking. The control group consisted of 29 non-smoking healthy subjects (19 women and 10 men, median age 22) without any history of atopy. Exhaled nitric oxide measurement was performed in all subjects. Exhaled nitric oxide levels in the angioedema group did not differ statistically significantly from those detected in healthy subjects (15.5 ppb and 17.0 ppb respectively). Our results indicate the lack of airway inflammation in chronic urticaria patients with pharynx angioedema.

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Section: Discussionmentioning

confidence: 98%

Exhaled Nitric Oxide Level in Pharynx Angioedema

Moos

Zając

Brzoza

2022

JCM

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“…Studies in this field involved vascular endothelial cadherin (transmembrane adhesive protein) [ 72 , 76 ], von Willebrand factor (marker of endothelial damage), soluble E-selectin (cytokine-induced adhesion molecule), endothelin-1 (vasomotor activity regulator) [ 30 , 77 , 78 ], arginine vasopressin , adrenomedullin [ 78 ], atrial natriuretic peptide [ 79 ], as well as endothelial-derived endocan and vascular cell adhesion molecule-1 (markers of endothelial function) [ 80 ]. The subsequently studied modulators of vascular permeability included vascular endothelial growth factors , angiopoietin-1 (which promotes endothelial stabilization) and angiopoietin-2 (which facilitates vascular permeability) [ 81 , 82 ], secreted phospholipases A2 (particularly the 2A group) [ 83 ], and platelet-activating factor acetylhydrolase [ 81 ]. Studies investigated changes in the above factors in small groups of patients with C1-INH-HAE during attacks and symptom-free periods, in comparison with healthy controls, as shown in Table 1 .…”

Section: Biochemical Biomarkersmentioning

confidence: 99%

Biomarkers in Hereditary Angioedema

Porębski

Kwitniewski

Reshef

2021

Clinic Rev Allerg Immunol

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A biomarker is a defined characteristic measured as an indicator of normal, biologic, pathogenic processes, or biological responses to an exposure or intervention. Diagnostic biomarkers are used to detect a disease or a subtype of a disease; monitoring biomarkers are measured serially to assess a medical condition; response biomarkers are used to check biologic response following a medical intervention; predictive biomarkers are used to identify patients who are more likely to respond to a medical intervention; and prognostic biomarkers are used to assess the future likelihood of a clinical event. Although biomarkers have been extensively investigated and validated in many diseases and pathologies, very few are currently useful for the diagnosis, evaluation of disease activity, and treatment of hereditary angioedema (HAE). Pathophysiologic pathways involved in HAE reveal a plethora of molecules from the complement, coagulation, and fibrinolysis systems or from the vascular endothelium, which may serve as biomarkers. The most promising candidates, together with their laboratory readout systems, should be evaluated with regard to their analytical and clinical validity and utility. To be highly specific, such biomarkers should be linked to the pathomechanisms of HAE, particularly the bradykinin-generating cascade. Additionally, major advances in high-throughput omics-based technologies may facilitate the discovery of new candidate biomarkers in the future. This review will cover the existing as well as future potential biomarkers that will support the diagnosis, monitor disease activity, and can be used to assess the efficacy of new avenues of therapy of HAE and other forms of angioedema.

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“…sPLA 2 group IIA (PLA2G2A) in C1-INH-HAE increases endothelial permeability and impairs C1-INH functional activity in vitro [ 46 ]. ANGPT1, a unique vascular stabilizer, is further increased during angioedema attack, whereas sPLA 2 activity is decreased [ 46 , 47 ] (Table 1 ).…”

Section: Innate Immune Systemmentioning

confidence: 99%

“…Different isoforms of sPLA 2 s (e.g., groups II, V, and X) can activate human PMN inducing NE, CXCL8, or angiogenic factors (VEGFs and ANGPTs) release [ 76 – 80 ]. Therefore, increase of PLA2G2A in C1-INH-HAE patients could affect the activation of PMN and be responsible of release for the VEGFs and ANGPTs [ 14 , 46 , 47 ] (Fig. 1 ).…”

Section: Innate Immune Systemmentioning

confidence: 99%

Roles of Immune Cells in Hereditary Angioedema

Ferrara

Cristinziano

Petraroli

et al. 2021

Clinic Rev Allerg Immunol

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Hereditary angioedema (HAE) is a rare genetic disease, characterized by recurrent and unexpected potentially life-threatening mucosal swelling. HAE may be further classified into HAE with C1‐inhibitor deficiency (C1‐INH‐HAE) and HAE with normal C1‐INH activity (nlC1‐INH‐HAE), mostly due to mutations leading to increased vascular permeability. Recent evidence implicates also the innate and adaptive immune responses in several aspects of angioedema pathophysiology. Monocytes/macrophages, granulocytes, lymphocytes, and mast cells contribute directly or indirectly to the pathophysiology of angioedema. Immune cells are a source of vasoactive mediators, including bradykinin, histamine, complement components, or vasoactive mediators, whose concentrations or activities are altered in both attacks and remissions of HAE. In turn, through the expression of various receptors, these cells are also activated by a plethora of molecules. Thereby, activated immune cells are the source of molecules in the context of HAE, and on the other hand, increased levels of certain mediators can, in turn, activate immune cells through the engagement of specific surface receptors and contribute to vascular endothelial processes that lead to hyperpemeability and tissue edema. In this review, we summarize recent developments in the putative involvement of the innate and adaptive immune system of angioedema.

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Hereditary angioedema attack: what happens to vasoactive mediators?

Cited by 9 publications

References 25 publications

Exhaled Nitric Oxide Level in Pharynx Angioedema

Exhaled Nitric Oxide Level in Pharynx Angioedema

Biomarkers in Hereditary Angioedema

Roles of Immune Cells in Hereditary Angioedema

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