“Nuts and Bolts” of Laboratory Evaluation of Angioedema

Farkas, Henriette; Veszeli, Nóra; Kajdácsi, Erika; Cervenak, László

doi:10.1007/s12016-016-8539-6

Cited by 46 publications

(31 citation statements)

References 87 publications

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“…It suggests that C4a could become a useful biomarker for predicting the occurrence of EAs. In our knowledge, such a biomarker is not currently available …”

Section: Discussionmentioning

confidence: 99%

Complete kinetic follow‐up of symptoms and complement parameters during a hereditary angioedema attack

Veszeli

Kőhalmi

Kajdácsi

et al. 2017

Allergy

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We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in a self-limited edematous attack (EA) in a patient with hereditary angioedema due to C1-INH deficiency to better understand the pathomechanism of the evolution, course, and complete resolution of EAs. C1-INH concentration and functional activity (C1-INH ), C1(q,r,s), C3, C4, C3a, C4a, C5a, and SC5b-9 levels were measured in blood samples obtained during the 96-hour observation period. The highest C1-INH , C4, and C1(q,r,s) levels were measured at baseline, and their continuous decrease was observed during the entire observation period. C4 depletion started at prodromal phase, and C4 was lowest after the maximum severity peak. Compared to baseline, C4a level was four times higher 7 hours before the onset of the attack. C1-INH did not increase after resolution of the attack suggesting that factors other than C1-INH may be important in this process. C4a may be a useful biomarker for the prediction of EAs.

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“…It suggests that C4a could become a useful biomarker for predicting the occurrence of EAs. In our knowledge, such a biomarker is not currently available …”

Section: Discussionmentioning

confidence: 99%

Complete kinetic follow‐up of symptoms and complement parameters during a hereditary angioedema attack

Veszeli

Kőhalmi

Kajdácsi

et al. 2017

Allergy

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“…C1q may be normal in AAE‐C1‐INH particularly in patients taking anabolic androgens. Many patients with AAE‐C1‐INH have autoantibodies that inactivate C1‐INH …”

Section: Differential Diagnosismentioning

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

438

925

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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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“…Approximately 85% of patients have decreased C1-INH levels (< 50% of those with type I disease); the remaining 15% have normal to elevated C1-INH levels but impaired function. C4 levels are low in C1-INH-HAE patients, because of its consumption in the absence of C1-INH activity [65]. In addition, normal C1q levels rule out the rare acquired form of C1-INH deficiency.…”

Section: Angioedema With Normal C1-inh Activitymentioning

confidence: 99%

Hereditary angioedema: the plasma contact system out of control

Maat

Hofman

Maas

2018

Journal of Thrombosis and Haemostasis

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The plasma contact system contributes to thrombosis in experimental models. Even though our standard blood coagulation tests are prolonged when plasma lacks contact factors, this enzyme system appears to have a minor (if any) role in hemostasis. In this review, we explore the clinical phenotype of C1 esterase inhibitor (C1-INH) deficiency. C1-INH is the key plasma inhibitor of the contact system enzymes, and its deficiency causes hereditary angioedema (HAE). This inflammatory disorder is characterized by recurrent aggressive attacks of tissue swelling that occur at unpredictable locations throughout the body. Bradykinin, which is considered to be a byproduct of the plasma contact system during in vitro coagulation, is the main disease mediator in HAE. Surprisingly, there is little evidence for thrombotic events in HAE patients, suggesting mechanistic uncoupling from the intrinsic pathway of coagulation. In addition, it is questionable whether a surface is responsible for contact system activation in HAE. In this review, we discuss the clinical phenotype, disease modifiers and diagnostic challenges of HAE. We subsequently describe the underlying biochemical mechanisms and contributing disease mediators. Furthermore, we review three types of HAE that are not caused by C1-INH inhibitor deficiency. Finally, we propose a central enzymatic axis that we hypothesize to be responsible for bradykinin production in health and disease.

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“Nuts and Bolts” of Laboratory Evaluation of Angioedema

Cited by 46 publications

References 87 publications

Complete kinetic follow‐up of symptoms and complement parameters during a hereditary angioedema attack

Complete kinetic follow‐up of symptoms and complement parameters during a hereditary angioedema attack

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

Hereditary angioedema: the plasma contact system out of control

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