Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies

Aygören‐Pürsün, Emel; Magerl, Markus; Maetzel, Andreas; Maurer, Marcus

doi:10.1186/s13023-018-0815-5

Cited by 130 publications

(100 citation statements)

References 35 publications

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“…The highest prevalence was noted in Sweden (1.56 patients per 100,000 inhabitants), followed by Italy and Norway with confirmed prevalence rates of 1.51 per 100,000 and 1.50 per 100,000 respectively . A recent comparison of national prevalence rates for C1‐INH‐HAE revealed that the epidemiology of this condition is stable; its prevalence was about 1.50 per 100.000 across the six European countries that were compared . Accurate estimates of the prevalence of C1‐INH‐HAE in a given population are hindered by the rare occurrence of the disease, poor awareness of the condition, the absence of established registries, the small number of studies on the prevalence and epidemiology of HAE, and diverse methods of data collection (registries, data from patient organizations or surveys).…”

Section: Discussionmentioning

confidence: 99%

Hereditary angioedema in Austria: prevalence and regional peculiarities

Schöffl

Wiednig

Koch

et al. 2019

J Deutsche Derma Gesell

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SummaryBackgroundData on the prevalence and clinical features of Austrian patients with hereditary angioedema (HAE) with C1‐inhibitor (C1‐INH) deficiency (HAE‐1) or dysfunction (HAE‐2) are lacking.MethodsCurrent baseline data were collected in a national survey. The records of HAE patients at the Medical University of Graz were analyzed with regard to clinical characteristics.ResultsA total of 137 patients were identified, yielding a prevalence of 1 : 64,396. The median age at the onset of symptoms was 6.5 years, and the median age at the time of correct diagnosis 21.0 years. The median delay in diagnosis was 15.0 years for newly diagnosed patients without a family history of HAE. Patients with a family history of HAE received an immediate diagnosis. HAE patients without a family history of HAE and born before 1960 had to wait a median of 16.0 years until they were diagnosed correctly. Patients born after 1980 still experienced a median diagnostic delay of 6.5 years.ConclusionPatients with this condition still face an excessive diagnostic delay in some parts of Austria, or their disorder may even remain unrecognized by specialists. This underlines the need for better awareness of the disease.

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Section: Discussionmentioning

confidence: 99%

Hereditary angioedema in Austria: prevalence and regional peculiarities

Schöffl

Wiednig

Koch

et al. 2019

J Deutsche Derma Gesell

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“…Die Häufi gkeit von HAE-Attacken kann von wenigen bis zu mehr als 100 pro Jahr variieren [ 6,7 ] . Die in der Literatur geschätzte Prävalenz der Krankheit reicht von 1 : 10.000 bis 1 : 150.000 [8][9][10] . HAE betrifft alle ethnischen Gruppen [ 11 ] .…”

Section: Resultsunclassified

Unnecessary abdominal interventions in patients with hereditary angioedema

Hahn

Hoess

Friedrich

et al. 2018

J Deutsche Derma Gesell

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Hintergrund : Das hereditäre Angioödem (HAE) ist eine seltene genetische Erkrankung mit rezidivierenden Schwellungen der Schleimhäute und der Haut. Es wird durch Bradykinin vermittelt und kann praktisch jeden Teil des menschlichen Körpers betreffen, einschließlich des Gastrointestinaltrakts, der Extremitäten sowie des Kehlkopfs und des Rachens. Zur akuten und prophylaktischen Behandlung des HAE stehen sichere und wirksame Arzneimittel zur Verfügung. Aufgrund seiner unspezifischen Symptome und besonderen Manifestationen wird das HAE jedoch häufig verkannt und falsch behandelt. Insbesondere abdominelle Ödeme können einer Appendizitis oder Cholezystitis ähneln, weshalb sie häufig unnötig chirurgisch behandelt werden. Wir haben eine Fragebogen-Studie zur Quantifizierung dieses Problems durchgeführt. Patienten und Methoden: Es wurden Fragebögen für HAE-Patienten und Probanden ohne HAE entworfen. Das Verhältnis von Patienten zu Kontrollpersonen betrug 1: 2. Ergebnisse : Im Vergleich zur Gesamtbevölkerung wird bei HAE-Patienten eine signifikant höhere Zahl invasiver diagnostischer und therapeutischer Interventionen durchgeführt. Unsere Studie zeigt, dass bei HAE-Patienten die Wahrscheinlichkeit abdominalchirurgischer (wie gynäkologischer und urologischer) Eingriffe 2,5mal höher ist als bei Probanden ohne HAE (p = 0,007). Die Zahl der Operationen pro Patient korrelierte mit dem Zeitraum (Anzahl der Jahre) zwischen Einsetzen der Symptome und Diagnose stellung (Korrelationskoeffizient 0,511; Spearman). Außerdem wurden bei HAE-Patienten doppelt so viele Appendektomien durchgeführt (18,9 % vs. 37.7 %). Unsere Ergebnisse zeigen auch, dass Gastroskopien bei HAE-Patienten 2,3mal häufiger waren als bei Kontrollpersonen ohne HAE (p = 0,003). Schlussfolgerungen : Patienten mit seltenen Krankheiten wie dem HAE leiden nicht nur an den Auswirkungen der Krankheit selbst. Die Krankheiten werden aufgrund mangelnden Wissens über solche Störungen und unspezifischer Symptome häufig jahrelang verkannt. Dies führt nicht nur zu falscher und wirkungsloser Behandlung, sondern auch zu unnötiger invasiver Diagnostik und Therapie. Daher ist es wichtig, Kenntnisse zu und Aufmerksamkeit gegenüber seltenen Krankheiten wie HAE zu stärken, um Diagnostik und Behandlung zu verbessern, das Leiden der Patienten zu minimieren und ihre Lebensqualität zu steigern.

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“…A systematic investigation of epidemiological studies found an estimated prevalence of ACEi AE between 0.7 and 1.7 per 10,000 inhabitants in the USA and between 1.0 and 2.6 per 10,000 inhabitants in Germany [3]. The weighted incidence of AE due to ACE inhibitor treatment was 0.3% in a large meta-analysis of randomized trials [4].…”

Section: Introductionmentioning

confidence: 99%

The Influence of ACE Inhibition on C1-Inhibitor: A Biomarker for ACE Inhibitor-Induced Angioedema?

Hahn¹,

Nordmann-Kleiner²,

Bönner

et al. 2019

Biomed Hub

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Aims: Angioedema is a rare side effect of angiotensin-converting enzyme (ACE) inhibitors. It remains unclear why it is only induced in a few patients taking ACE inhibitors, often after a long period of uneventful treatment. The aim of this study was to analyze the influence of ACE inhibitor treatment on C1-inhibitor (C1-INH) levels. Methods: Captopril (5 mg/25 mg) was added to blood samples of 5 healthy subjects. C1-INH levels were measured before and after incubation for 180 min. The second section of the study was done with 17 patients who received therapy with an ACE inhibitor for the first time. C1-INH levels were measured before ACE inhibitor treatment, 24 h after first drug administration, and 4 weeks later. Results: After incubation of blood samples with 5 mg captopril, there was no detectable change in C1-INH levels. After incubation with 25 mg, C1-INH activity was decreased by an average of 29% and the C1-INH concentration was decreased by an average of 0.06 g/L. In the second study section, inconsistent effects on C1-INH levels were detected. In the majority of patients, 24 h after the first ACE inhibitor administration C1-INH activity was tending to be increased. Conclusions: A dose-dependent effect on C1-INH levels in captopril-incubated blood samples of healthy test persons was shown. In patients with new ACE inhibitor treatment, heterogeneous reactions of C1-INH values were detected. Larger studies are needed over a longer period of time to find correlations between the effect of ACE inhibitor therapy on C1-INH levels and the clinical course/development of side effects.

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Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies

Cited by 130 publications

References 35 publications

Hereditary angioedema in Austria: prevalence and regional peculiarities

Hereditary angioedema in Austria: prevalence and regional peculiarities

Unnecessary abdominal interventions in patients with hereditary angioedema

The Influence of ACE Inhibition on C1-Inhibitor: A Biomarker for ACE Inhibitor-Induced Angioedema?

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