Modification of Peripheral Blood T-Lymphocyte Surface Receptors and Langerhans Cell Numbers in Hereditary Angioedema

Cillari, Enrìco; Misiano, Gabriella; Aricò, Mario; Rocca, Eleonora La; Lio, Domenico; Leonardo, Salvatore dl; Brai, M.

doi:10.1093/ajcp/85.3.305

Cited by 7 publications

(4 citation statements)

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“…Various cells of the immune system seem to be involved [11], and C1INH synthesis is subject to complex positive and negative control by cytokines [12][13][14][15]. In fact, recombinant IFNγ and to the lesser extent GM-CSF, tumour necrosis factor-alpha (TNFα) and IL-6 regulate C1INH synthesis in various cell types [12,13].…”

Section: Introductionmentioning

confidence: 99%

The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

et al. 2018

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Hereditary angioedema (HAE) is a rare autosomic-dominant disorder characterized by a deficiency of C1 esterase inhibitor which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways that are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis during attack and remission state and n = 19 healthy subjects. The samples were tested for a panel of IL (Interleukin)-17-type cytokines (IL-1β, IL-6, IL-10, granulocyte-macrophage colony stimulating factor (GM-CSF), IL-17, IL-21, IL-22, IL-23) and transforming growth factor-beta (TGF-β) subtypes. Data indicate that there are variations of cytokine levels in HAE subjects comparing the condition during the crisis respect to the value in the remission phase, in particular type 17 signature cytokines are increased, whereas IL-23 is unmodified and TGF-β3 is significantly reduced. When comparing healthy and HAE subjects in the remission state, we found a significant difference for IL-17, GM-CSF, IL-21, TGF-β1 and TGF-β2 cytokines. These results confirm and extend our previous findings indicating that in HAE there is operating an inflammatory activation process, which involves also T helper 17 (Th17) cytokines and TGF-β isoforms, associated with localized angioedema attacks and characterized by elevated bradykinin levels.

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Section: Introductionmentioning

confidence: 99%

The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

et al. 2018

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“…Since the cells mediate adaptive immune responses, they seem to be uninvolved in HAE [ 17 ]. Only a few studies have reported abnormal T and B cell counts or abnormal distribution of T cell surface IgG-receptors [ 18 , 19 ].On the contrary, the study from Lopez-Lera et al on the profiling of the RNA expression of peripheral blood mononuclear cells (PBMC) from C1-INH-HAE patients did not prove alterations in the expression pattern of PBMC in association with frequency and severity of disease [ 20 ]. We do believe that our results provide some clues on the role of lymphocytes that differ among this study cohorts.…”

Section: Discussionmentioning

confidence: 99%

The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency

Dyga

Obtułowicz

Mikołajczyk

et al. 2022

IJMS

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Background: Hereditary angioedema (HAE) is a rare, genetic disease caused by the decreased level or function of the C1 inhibitor. The primary mediator of symptoms in HAE is bradykinin acting through its two receptors, namely receptors 1 (BR1) and 2 (BR2). Although BR2 is well characterized, the role of BR1 remains unclear. Objective: To study the role of bradykinin receptors 1 (BR1) in the etiopathogenesis of HAE. Methods: A total of 70 individuals, 40 patients with HAE, and 30 healthy subjects were recruited to the study. HAE was diagnosed in accordance with the international guideline. The level of bradykinin receptors was determined in populations of CD3+, CD4+, CD8+, and CD14++CD16−, CD14++CD16+ monocytes. In addition, the level of disease activity-specific markers was measured. Results: There were statistically significant differences in the subpopulation of lymphocytes and monocytes between patients with HAE compared to healthy subjects. The level of BR1 and BR2 on PBMCs was comparable in healthy subjects and HAE patients during remission with significant overexpression of both receptors, triggered by HAE attack. Moreover, a significant increase in TNF-alpha and IL-1 plasma levels was observed among HAE patients. Conclusions: BR1 expression may play an important role in the pathomechanism of HAE.

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“…Adaptive immunity has been poorly studied in HAE. Few reports have reported abnormal T and B cell counts, abnormal distribution of T cell surface IgG-receptors, and reduced Langerhans cell numbers in HAE patients [ 126 , 127 ].…”

Section: Adaptive 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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Modification of Peripheral Blood T-Lymphocyte Surface Receptors and Langerhans Cell Numbers in Hereditary Angioedema

Cited by 7 publications

References 0 publications

The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency

Roles of Immune Cells in Hereditary Angioedema

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