Bacteriuria increases the risk of edematous attacks in hereditary angioedema with C1‐inhibitor deficiency

Zotter, Zsuzsanna; Veszeli, Nóra; Kőhalmi, Kinga Viktória; Imreh, Éva; Kovács, Gábor; Nallbani, Marsel

doi:10.1111/all.13034

Cited by 10 publications

(9 citation statements)

References 11 publications

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“…There is no data supporting the increase in frequency of acute or chronic infections in HAE patients. Although chronic infections like Helicobacter pylori [74] or bacteriuria [75] were shown to increase HAE attack frequency, a direct comparison of their occurrence between HAE patients and the healthy population was missing from these studies. Only one study from Poland did not demonstrate an increased antibody frequency against H. pylori compared to the healthy population both in children and in adult HAE patients [76].…”

Section: Discussionmentioning

confidence: 99%

Neutrophils Are Dysregulated in Patients with Hereditary Angioedema Types I and II in a Symptom-Free Period

Grymova

Vlková

Souček

et al. 2019

Mediators of Inflammation

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Neutrophils impact on processes preceding the formation of bradykinin, a major swelling mediator in hereditary angioedema (HAE), yet their potential role in HAE pathogenesis has not been sufficiently studied. We assessed the relative mRNA expression of 10 genes related to neutrophil activation using RNA extracted from the peripheral blood neutrophils of 23 HAE patients in a symptom-free period and 39 healthy donors. Increased relative mRNA expression levels ofCD274,IL1B,IL1RN,IL8,MMP9, andTLR4, together with a lack in their mutual correlations detected in HAE patients compared to healthy controls, suggested a preactivated state and dysregulation of patients’ neutrophils. Patients’ neutrophil-alerted state was further supported by increased CD11b, decreased CD16 plasma membrane deposition, and increased relative CD274+and CD87+neutrophil counts, but not by increased neutrophil elastase or myeloperoxidase plasma levels. As CD274 mediates inhibitory signals to different immune cells, neutrophils were cocultured with T-cells/PBMC. The decrease in CD25+and IFN-γ+T-cell/PBMC ratio in patients indicated the patients’ neutrophil suppressive functions. In summary, the results showed neutrophils’ alerted state and dysregulation at the transcript level in patients with HAE types I and II even in a symptom-free period, which might make them more susceptible to edema formation. Neutrophils’ T-cell suppressive capacity in HAE patients needs to be further investigated.

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Section: Discussionmentioning

confidence: 99%

Neutrophils Are Dysregulated in Patients with Hereditary Angioedema Types I and II in a Symptom-Free Period

Grymova

Vlková

Souček

et al. 2019

Mediators of Inflammation

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“…Good dental care can reduce extractions, need for aggressive dental procedures, and prevent acute or chronic intraoral inflammation, which might reduce the threshold for attacks. Monitoring for side effects of medications is important and is outlined above We recommend that all patients with HAE should be educated about possible triggers which may induce HAE attacks.Evidence grade: C, strength of recommendation: strong, 100% agreement.…”

Section: Patient Support Home Therapy and Self‐administration And Omentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

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470

974

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?

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Section: Avoidance Of Triggersmentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

World Allergy Organization Journal

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.

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Bacteriuria increases the risk of edematous attacks in hereditary angioedema with C1‐inhibitor deficiency

Cited by 10 publications

References 11 publications

Neutrophils Are Dysregulated in Patients with Hereditary Angioedema Types I and II in a Symptom-Free Period

Neutrophils Are Dysregulated in Patients with Hereditary Angioedema Types I and II in a Symptom-Free Period

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update

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