Therapeutic management of hereditary angioedema: past, present, and future

Valerieva, Anna; Nedeva, Denislava; Yordanova, V.; Petkova, Elena; Staevska, Maria

doi:10.5152/balkanmedj.2021.21094

Cited by 7 publications

(7 citation statements)

References 116 publications

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“…We found an increase in the number of attacks experienced by HAE patients during the pandemic. In addition, these patients In contrast to the treatment of HAE attacks, short-term prophylaxis aims to prevent attacks under conditions of expected stress, and long-term prophylaxis aims to reduce the number, severity, and burden of angioedema attacks (15,16).…”

Section: Discussionmentioning

confidence: 99%

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Management of Patients with Hereditary Angioedema During the COVID-19 Pandemic

Yıldız¹,

Arslan²,

Çölkesen³

et al. 2021

Asthma Allergy Immunol

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Objective: The aim of this study was to determine the clinical course and treatment outcomes of patients with hereditary angioedema (HAE) after infection with coronavirus disease 2019 (COVID-19). Materials and Methods: Thirty-nine patients with HAE were included in this study. These patients were regularly followed up over phone calls since the first COVID-19 case was seen in our country. Patients were asked to visit the hospital if there was a history of contact with a confirmed COVID-19 patient or if the patient developed clinical symptoms of COVID-19.Results: There were 21 (54%) patients with type I HAE, and 18 (46%) with type II HAE. All patients received treatment for angioedema attacks (C1-inhibitor [C1-INH], icatibant), and seven (20%) received long-term prophylaxis (danazol). Treatment for attacks was continued for all patients during the pandemic. Patients taking danazol were switched to long-term prophylaxis using the C1-INH concentrate. Eleven (28%) patients with HAE developed COVID-19 during this study. Only one patient had severe COVID-19. Six patients (54.5%) were diagnosed with type II HAE, and five (45.5%) were diagnosed with type I HAE. The most common COVID-19 symptoms were fever (7/11; 64%) and myalgia (6/11; 55%). Mild angioedema attacks were experienced by 36% (4/11) of the HAE patients diagnosed with COVID-19. Icatibant was used in all patients.Conclusion: Agents used for HAE block the kallikrein-kinin system and may be useful in the treatment of COVID-19. Considering their beneficial effects on COVID-19, it is recommended that HAE patients should continue the use of agents blocking the kallikrein-kinin system. Keywords: COVID-19, hereditary angioedema, kallikrein-kinin system, bradykinin, C1-INH

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Section: Discussionmentioning

confidence: 99%

“…Attenuated androgens such as danazol are used for short-and long-term prophylaxis against HAE attacks (15). COVID-19 causes more severe disease, worse clinical outcomes, and a higher risk of deaths in males than in Icatibant is a synthetic B2R antagonist (21).…”

Section: Discussionmentioning

confidence: 99%

Management of Patients with Hereditary Angioedema During the COVID-19 Pandemic

Yıldız¹,

Arslan²,

Çölkesen³

et al. 2021

Asthma Allergy Immunol

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“…To date, four types of medication containing C1-INH are available [12] C1-INH concentrates are effective in treating acute HAE attacks [12,18,31]. Although evidence regarding the use of FFP to treat HAE attacks is much less abundant than the published data J Investig Allergol Clin Immunol 2023; Vol.…”

Section: On-demand Treatmentmentioning

confidence: 99%

Expert Review and Consensus on the Treat-to-Target Management of Hereditary Angioedema: From Scientific Evidence to Clinical Practice

Caballero¹,

Lleonart-Bellfill²,

Pedrosa³

et al. 2023

J Investig Allergol Clin

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Background: Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is a rare disease characterized by swelling episodes. It affects quality of life (QoL) and can be lethal, when involving upper airways. Treatment is individualized, with therapeutic options including on-demand treatment (ODT), and short- and long-term prophylaxis (STP, LTP). However, available guidelines are not always clear about treatment selection, its goals or assessment of goal achievement. Objective: To review the available evidence for the management of HAE-C1INH and build a Spanish expert consensus to steer HAE-C1INH management towards a treat-to-target (T2T) approach, while addressing some of the unclear aspects of the Spanish guidelines. Methods: We reviewed the literature on the management of HAE-C1INH with a T2T approach, focusing on: 1) treatment selection and goals; and 2) available tools to assess goal achievement. We discussed the literature based on clinical experience and drew up 45 statements on undefined management aspects. A panel of 53 HAE experts validated the statements through a two-round Delphi process. Results: The goals for ODT and STP are minimizing the morbidity and mortality of attacks, and preventing attacks caused by known triggers, respectively, while the main goal of LTP is to decrease the rate, severity and duration of attacks. Furthermore, when prescribing, clinicians should consider the reduction in adverse events, while increasing patient QoL and satisfaction. Appropriate instruments for assessing goal achievement have also been indicated. Conclusions: We provide recommendations on previously unclear aspects of HAE-C1INH management with ODT, STP and LTP, focusing particularly on clinical and patient-oriented goals.

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“…27 In parallel, various regulatory incentives in both the European Union countries and the United States that stimulated the research on rare diseases lead to the development of an abundance of medicinal products for HAE, which may not exist for any other rare disease. 28 …”

Section: Deciphering the Complexitymentioning

confidence: 99%