The Role of Mast Cells Mediators in Angioedema Without Wheals

Sala‐Cunill, Anna; Guilarte, Mar

doi:10.1007/s40521-015-0067-8

Cited by 6 publications

(6 citation statements)

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“…Such a disruption causes increased production of eosinophils, mast cells, and proinflammatory mediators; this, in turn, results in increased production of cysteinyl leukotrienes (a family of inflammatory lipid mediators) ( 17 ). Cysteinyl-leukotrienes increase vascular permeability, and in-vitro studies suggest that cysteinyl leukotrienes can induce histamine hyperresponsiveness by increasing the expression of histamine receptors ( 18 , 19 ). Altogether, angioedema ensues.…”

Section: Histamine-mediated Angioedema Pathophysiologymentioning

confidence: 99%

Pathophysiology of bradykinin and histamine mediated angioedema

Lima,

Zheng,

Wong

et al. 2023

Front. Allergy

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Angioedema is characterized by swelling localized to the subcutaneous and submucosal tissues. This review provides an overview of angioedema, including the different types, triggers, and underlying pathophysiologic mechanisms. Hereditary and acquired angioedema are caused by dysregulation of the complement and kinin pathways. In contrast, drug-induced and allergic angioedema involve the activation of the immune system and release of vasoactive mediators. Recent advances in the understanding of the pathophysiology of angioedema have led to the development of targeted therapies, such as monoclonal antibodies, bradykinin receptor antagonists, and complement inhibitors, which promise to improve clinical outcomes in patients with this challenging condition. To accurately diagnose and manage angioedema, an understanding of this condition's complex and varied pathophysiology is both necessary and critical.

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Section: Histamine-mediated Angioedema Pathophysiologymentioning

confidence: 99%

Pathophysiology of bradykinin and histamine mediated angioedema

Lima,

Zheng,

Wong

et al. 2023

Front. Allergy

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“…Disease activity was determined by the following questionnaires: Angioedema Activity Score for 7 consecutive days (AAS7) in both CHA and CSU-AE patients and Urticaria Activity Score over 7 Days (UAS7) in CSU-AE patients. The AAS7 score was categorized into four score levels: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and high (19-105). 17 UAS7 was also divided into four score bands: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate and high (28-42).…”

Section: Patients and Clinical Datamentioning

confidence: 99%

“…The AAS7 score was categorized into four score levels: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and high (19-105). 17 UAS7 was also divided into four score bands: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate and high (28-42). 18 The impact on quality of life was measured by the Angioedema Quality of Life (AE-QoL) in CHA and CSU-AE patients and by the Chronic Urticaria Quality of Life (CU-Q 2 oL) questionnaires in CSU-AE patients.…”

Section: Patients and Clinical Datamentioning

confidence: 99%

“…1,2 According to the indicated vasoactive mediator, AE can be classified into two types: histamine-mediated AE (induced by mast cell activation and basophil degranulation) and bradykinin-mediated AE (due to excessive bradykinin), as observed in hereditary angioedema, acquired angioedema with C1 inhibitor deficiency (related to lymphoproliferative and autoimmune diseases) and angioedema related to angiotensin-converting enzyme (ACE) inhibitors. 3,4 Histamine-mediated AE is the most frequent form of angioedema, 2 and it is classified as chronic histaminergic acquired angioedema (CHA) when allergies or other causes have been excluded and positive treatment responses to antihistamines, corticosteroids, adrenaline, or omalizumab 5 have been reported. Histaminemediated AE can occur isolated as CHA or associated with wheals in chronic spontaneous urticaria (CSU).…”

Section: Introductionmentioning

confidence: 99%

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Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Sabaté‐Brescó

Rodríguez‐Garijo

Azofra

et al. 2021

Preprint

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Background: Chronic histaminergic angioedema (CHA) is defined as recurrent episodes of isolated angioedema (without hives) of unknown cause that respond to the same treatment as chronic spontaneous urticaria (CSU). Quality of life (QoL) studies have not been performed for CHA, except those carried out in the context of CSU associated with angioedema attacks (CSU-AE). Moreover, biomarkers for monitoring disease activity in CHA have not been identified. We aim to describe the burden of CHA and impact on patient QoL, compare the findings to those in CSU-AE patients, and investigate biomarker associations with disease severity and QoL parameters. Methods: We performed a prospective multicenter study that included 68 patients with CHA and 63 patients with CSU-AE. Demographic and clinical variables were collected. Validated patient-reported questionnaires were employed to analyze the quality of life and disease activity. Blood and serological parameters, including blood cell count, C-reactive protein, D-dimer and total IgE, were also analyzed. Results: Angioedema disease activity was significantly higher in CSU-AE patients (median AAS7, IQR: 1, [0–1]) than CHA patients (0, [0–1]; p= 0.022). A considerable impact on QoL was found in both groups, although significantly worse values were found for CSU-AE (median AEQoL, IQR: 37, [10–65]; p=0.005). CHA patients were older than CSU-AE patients, and female predominance was not observed. Conclusions: Angioedema severity and QoL impacts are significantly worse in CSU than in chronic histaminergic angioedema. Angioedema should be included in severity urticaria scores (UAS) as well as in specific quality of life urticaria scales.

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“…On the other hand, there is no commonly agreed classification and nomenclature for angioedema without wheals, which generates confusion in the terms used in the clinical guidelines; to date, there are different classification proposals based on the pathogenic mechanism (histaminergic or bradykinergic) [ 7 – 9 ], endotype (due to bradykinin excess, mast cell/basophil degranulation, and idiopathic) [ 10 ], or phenotype and genotype (acquired or hereditary, with or without C1 inhibitor [C1-INH] deficiency) [ 5 , 11 , 12 ].…”

Section: Introductionmentioning

confidence: 99%

Classification and Treatment of Angioedema without Wheals: A Spanish Delphi Consensus

2022

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Introduction Problems in the definition and classification of angioedema, leading to difficulties in its diagnosis and treatment, have been identified; therefore, an improvement in the current classification of angioedema is required. Objective The aim of this study was to propose a practical classification of angioedema without wheals that helps to establish a differential diagnosis and take appropriate therapeutic decisions. Methods An initial proposal of classification of angioedema without wheals was agreed by a scientific committee of experts and was subsequently validated by a panel of experts by means of consensus based on the Delphi methodology. Forty-five items on the classification, diagnosis, and treatment of angioedema without wheals were proposed for the survey. Results Most items (93.8%) were agreed after two rounds. All panelists agreed with the proposed classification, as well as with most of the clinical and treatment characteristics. The angioedema without wheals classification established three groups: histamine-mediated, bradykinin-mediated, and unknown mechanism angioedema. The clinical characteristics of the proposed types of angioedema were also agreed, except for the allergic histamine-mediated and unknown mechanism angioedema, which generated debate. Regarding treatments, although there was broad agreement with the proposed items, a lack of knowledge about some treatments in this pathology was observed. Conclusion The proposed classification of angioedema without wheals was accepted with a high degree of agreement; however, knowledge of available treatments needs to be increased and the definition of angioedema of unknown mechanism needs to be improved. Supplementary Information The online version contains supplementary material available at 10.1007/s40257-022-00735-7.

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The Role of Mast Cells Mediators in Angioedema Without Wheals

Cited by 6 publications

References 55 publications

Pathophysiology of bradykinin and histamine mediated angioedema

Pathophysiology of bradykinin and histamine mediated angioedema

Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Classification and Treatment of Angioedema without Wheals: A Spanish Delphi Consensus

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