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Management and Treatment in Angioedema
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Management and Treatment in Angioedema

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Vascular Medicine".

Deadline for manuscript submissions: closed (31 October 2021) | Viewed by 25681

Special Issue Editors

Dr. Maria Bova

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Guest Editor
Department of Internal Medicine, AORN Cardarelli, Naples, Italy
Interests: hereditary angioedema with C1-inhibitor deficiency; bradykinin-mediated angioedema; angioedema with normal C1-inhibitor; orofacial granulomatosis; idiopathic angioedema
Special Issues, Collections and Topics in MDPI journals
Dr. Mar Guilarte, MD.

E-Mail
Guest Editor
1. Hospital Universitari Vall d’Hebron, Barcelona, Spain
2. Universitat Ramon Llull, Barcelona, Spain
Interests: angioedema; hereditary angioedema; bradykinin-mediated angioedema; mast cell-mediated angioedema; idiopathic angioedema; contact system

Special Issue Information

Dear Colleagues,

In the last decade, our knowledge regarding angioedema has taken a significant number of steps forward. Recurrent angioedema without wheals is now recognized as a clinical entity. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. In the last few years, the identification of new types of hereditary angioedema has required a continuous update of the classification. Recently, additional mechanisms have been involved in angioedema pathogenesis, including the uncontrolled activation of factor XII, generation of vasoactive mediators that induce dysregulation of endothelial functions, and bidirectional interactions between mast cell-derived mediators and the plasma contact system. This fervid research activity has promoted the identification of new diagnostic tools, and many new therapies, some of them available in many countries, and others progressing at different research stages.

As it often happens, each answer opens the way for a new question. Despite major advances in our understanding of recurrent angioedema, many unsolved questions remain, leaving several unmet needs for patients and caregivers.

In this Special Issue, we will more closely examine recent innovations and advancements in the knowledge of pathogenetic mechanisms, with a focus about genetics. Moreover, we will also address the management of different forms of angioedema, with special attention to idiopathic types, and their approach in clinical and emergency settings, both for diagnostic and therapeutic approaches.

Dr. Maria Bova, MD
Dr. Mar Guilarte, MD
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Bradykinin-mediated angioedema
  • Idiopathic angioedema
  • Role of endothelium in angioedema
  • Genetics of angioedema
  • Biomarkers in angioedema
  • Diagnostic test for angioedema
  • Angioedema treatment
  • Angioedema in emergency setting
  • Burden of disease of angioedema

Published Papers (6 papers)

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Research

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12 pages, 971 KiB  
Article
Clinical Characteristics and Management of Angioedema Attacks in Polish Adult Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency
by Katarzyna Piotrowicz-Wójcik, Małgorzata Bulanda, Aldona Juchacz, Joanna Jamróz-Brzeska, Jacek Gocki, Krzysztof Kuziemski, Robert Pawłowicz and Grzegorz Porebski
J. Clin. Med. 2021, 10(23), 5609; https://doi.org/10.3390/jcm10235609 - 29 Nov 2021
Cited by 5 | Viewed by 2063
Abstract
Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease characterized by recurrent swellings. This study aims to determine (i) the clinical characteristics of the HAE patient population from Poland, and (ii) real-life patients’ treatment practices. A cross-sectional study involved 138 [...] Read more.
Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease characterized by recurrent swellings. This study aims to determine (i) the clinical characteristics of the HAE patient population from Poland, and (ii) real-life patients’ treatment practices. A cross-sectional study involved 138 adult HAE patients (88 females, 50 males) treated in six regional HAE centers in Poland. Consecutive patients during routine follow-up visits underwent a structured medical interview on the clinical characteristics of the course and treatment of HAE attacks within the last six months. A total of 118 of 138 patients was symptomatic. They reported in total 2835 HAE attacks predominantly peripheral and abdominal, treated with plasma-derived C1-INH (61.4%), icatibant (36.7%) and recombinant C1-INH (1.9%). An amount of 116 patients carried the rescue medication with them while traveling, and 74 patients self-administrated on demand treatment. There were twice as many symptomatic women (n = 78) as there were men (n = 40). Women treated their HAE attacks significantly more often than men. Older patients (≥65 years) reported a longer delay in diagnosis, and practiced the self-administration of rescue medication less frequently in comparison to other patients. Clinical features of the surveyed population are similar to other European, but not Asian, HAE patient groups. Self-administration still remains an unmet medical need. Some distinct HAE patients may require special attention due to the severe course of the disease (females) or a delay in diagnosis (the elderly). Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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9 pages, 704 KiB  
Communication
Angioedema Caused by Drugs That Prevent the Degradation of Vasoactive Peptides: A Pharmacovigilance Database Study
by Yoshihiro Noguchi, Azusa Murayama, Hiroki Esaki, Mayuko Sugioka, Aisa Koyama, Tomoya Tachi and Hitomi Teramachi
J. Clin. Med. 2021, 10(23), 5507; https://doi.org/10.3390/jcm10235507 - 25 Nov 2021
Cited by 5 | Viewed by 2256
Abstract
Angioedema results from the decreased degradation of vasoactive peptides such as substance P and bradykinin. In this study, we sought to clarify whether dipeptidyl peptidase-4 (DPP-4) and angiotensin-converting enzyme (ACE) inhibitors that suppress the degradation of substance P and bradykinin are involved in [...] Read more.
Angioedema results from the decreased degradation of vasoactive peptides such as substance P and bradykinin. In this study, we sought to clarify whether dipeptidyl peptidase-4 (DPP-4) and angiotensin-converting enzyme (ACE) inhibitors that suppress the degradation of substance P and bradykinin are involved in angioedema onset. We calculated information coefficients (ICs) by performing a disproportionality analysis to evaluate DPP-4/ACE inhibitor-induced angioedema using the Japanese Adverse Drug Event Report (JADER) database. No angioedema signals were detected for DPP-4 inhibitors; however, a signal was detected for ACE inhibitors (IC: 2.42, 95% confidence interval (CI): 2.19 to 2.65). Of the patients treated with DPP-4 inhibitors, four developed drug-induced angioedema in combination with ACE inhibitors, and all were taking vildagliptin. Signals were detected for enalapril (IC: 2.39, 95% CI: 2.06 to 2.71), imidapril (IC: 2.83, 95% CI: 2.38 to 3.27), lisinopril (IC: 2.28, 95% CI: 1.55 to 3.00), temocapril (IC: 1.35, 95% CI: 0.29 to 2.40), and trandolapril (IC: 1.57, 95% CI: 0.19 to 2.95). Both inhibitors inhibited the degradation of substance P and bradykinin and were thus expected to cause angioedema. However, no signal of angioedema was detected with the DPP-4 inhibitors, in contrast to some ACE inhibitors. This study found that ACE inhibitors and DPP-4 inhibitors, which inhibit the degradation of substance P and bradykinin, tended to have different effects on the onset of angioedema in clinical practice. Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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8 pages, 1081 KiB  
Article
Deciphering the Genetics of Primary Angioedema with Normal Levels of C1 Inhibitor
by Gedeon Loules, Faidra Parsopoulou, Maria Zamanakou, Dorottya Csuka, Maria Bova, Teresa González-Quevedo, Fotis Psarros, Gregor Porebski, Matthaios Speletas, Davide Firinu, Stefano del Giacco, Chiara Suffritti, Michael Makris, Sofia Vatsiou, Andrea Zanichelli, Henriette Farkas and Anastasios E. Germenis
J. Clin. Med. 2020, 9(11), 3402; https://doi.org/10.3390/jcm9113402 - 23 Oct 2020
Cited by 18 | Viewed by 2969
Abstract
The genetic alteration underlying the great majority of primary angioedema with normal C1 inhibitor (nl-C1-INH-HAE) cases remains unknown. To search for variants associated with nl-C1-INH-HAE, we genotyped 133 unrelated nl-C1-INH-HAE patients using a custom next-generation sequencing platform targeting 55 genes possibly involved in [...] Read more.
The genetic alteration underlying the great majority of primary angioedema with normal C1 inhibitor (nl-C1-INH-HAE) cases remains unknown. To search for variants associated with nl-C1-INH-HAE, we genotyped 133 unrelated nl-C1-INH-HAE patients using a custom next-generation sequencing platform targeting 55 genes possibly involved in angioedema pathogenesis. Patients already diagnosed with F12 alterations as well as those with histaminergic acquired angioedema were excluded. A variant pathogenicity curation strategy was followed, including a comparison of the results with those of genotyping 169 patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE), and only filtered-in variants were studied further. Among the examined nl-C1-INH-HAE patients, carriers of neither the ANGPT1 p.Ala119Ser nor the KNG1 p.Met379Lys variant were found, whereas the PLG p.Lys330Glu was detected in four (3%) unrelated probands (one homozygote). In total, 182 different variants were curated, 21 of which represented novel mutations. Although the frequency of variants per gene was comparable between nl-C1-INH-HAE and C1-INH-HAE, variants of the KNG1 and XPNPEP1 genes were detected only in nl-C1-INH-HAE patients (six and three, respectively). Twenty-seven filtered variants in 23 different genes were detected in nl-C1-INH-HAE more than once, whereas 69/133 nl-C1-INH-HAE patients had compound heterozygotes of filtered variants located in the same or different genes. Pedigree analysis was performed where feasible. Our results indicate the role that alterations in some genes, like KNG1, may play in disease pathogenesis, the complex trait that is possibly underlying in some cases, and the existence of hitherto unrecognized disease endotypes. Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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Review

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9 pages, 754 KiB  
Review
The Genetics of Hereditary Angioedema: A Review
by Rosa Santacroce, Giovanna D'Andrea, Angela Bruna Maffione, Maurizio Margaglione and Maria d'Apolito
J. Clin. Med. 2021, 10(9), 2023; https://doi.org/10.3390/jcm10092023 - 9 May 2021
Cited by 46 | Viewed by 6338
Abstract
Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway. Mutations in SERPING1, the gene that encodes [...] Read more.
Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway. Mutations in SERPING1, the gene that encodes C1-INH (C1 esterase inhibitor), are responsible for the majority of cases of hereditary angioedema. C1 esterase inhibitor (C1-INH) is a major regulator of critical enzymes that are implicated in the cascades of bradykinin generation, which increases the vascular permeability and allows the flow of fluids into the extracellular space and results in angioedema. Moreover, a dominantly inherited disease has been described that has a similar clinical picture to C1-INH-HAE (Hereditary angioedema due to C1 inhibitor deficiency), but with normal C1-INH level and activity. This new type of HAE has no mutation in the SERPING1 gene and it is classified as nC1-INH-HAE (HAE with normal C1-INH). Currently mutations in six different genes have been identified as causing nC1-INH-HAE: factor XII (F12), plasminogen (PLG), angiopoietin 1 (ANGPT1), Kininogen 1 (KNG1), Myoferlin (MYOF), and heparan sulfate (HS)-glucosamine 3-O-sulfotransferase 6 (HS3ST6). In this review we aim to summarize the recent advances in genetic characterization of angioedema and possible future prospects in the identification of new genetic defects in HAE. We also provide an overview of diagnostic applications of genetic biomarkers using NGS technologies (Next Generation Sequencing). Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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14 pages, 5610 KiB  
Review
Episodic Angioedema with Hypereosinophilia (Gleich’s Syndrome): A Case Report and Extensive Review of the Literature
by Ilaria Mormile, Angelica Petraroli, Stefania Loffredo, Francesca Wanda Rossi, Mauro Mormile, Andrea Del Mastro, Giuseppe Spadaro, Amato de Paulis and Maria Bova
J. Clin. Med. 2021, 10(7), 1442; https://doi.org/10.3390/jcm10071442 - 1 Apr 2021
Cited by 11 | Viewed by 3894
Abstract
Episodic angioedema with eosinophilia (EAE) (Gleich’s syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 109 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3–4 weeks). The exact etiology of [...] Read more.
Episodic angioedema with eosinophilia (EAE) (Gleich’s syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 109 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3–4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Herein, we performed a broad literature review and report the case of a thirty-two-year-old woman with a two-year history of cyclic angioedema attacks, urticaria, periodic weight gain, and severe hypereosinophilia, diagnosed with EAE and treated with oral corticosteroids. Describing the most relevant clinical features of EAE reported so far in the literature, we aim to provide physicians with some useful tools to help them deal with this disease. In addition, we aim to raise awareness about this rare condition in which approved diagnostic classification criteria are currently missing. Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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14 pages, 1510 KiB  
Review
The Role of the Plasminogen Activation System in Angioedema: Novel Insights on the Pathogenesis
by Filomena Napolitano and Nunzia Montuori
J. Clin. Med. 2021, 10(3), 518; https://doi.org/10.3390/jcm10030518 - 1 Feb 2021
Cited by 27 | Viewed by 7159
Abstract
The main physiological functions of plasmin, the active form of its proenzyme plasminogen, are blood clot fibrinolysis and restoration of normal blood flow. The plasminogen activation (PA) system includes urokinase-type plasminogen activator (uPA), tissue-type PA (tPA), and two types of plasminogen activator inhibitors [...] Read more.
The main physiological functions of plasmin, the active form of its proenzyme plasminogen, are blood clot fibrinolysis and restoration of normal blood flow. The plasminogen activation (PA) system includes urokinase-type plasminogen activator (uPA), tissue-type PA (tPA), and two types of plasminogen activator inhibitors (PAI-1 and PAI-2). In addition to the regulation of fibrinolysis, the PA system plays an important role in other biological processes, which include degradation of extracellular matrix such as embryogenesis, cell migration, tissue remodeling, wound healing, angiogenesis, inflammation, and immune response. Recently, the link between PA system and angioedema has been a subject of scientific debate. Angioedema is defined as localized and self-limiting edema of subcutaneous and submucosal tissues, mediated by bradykinin and mast cell mediators. Different forms of angioedema are linked to uncontrolled activation of coagulation and fibrinolysis systems. Moreover, plasmin itself can induce a potentiation of bradykinin production with consequent swelling episodes. The number of studies investigating the PA system involvement in angioedema has grown in recent years, highlighting its relevance in etiopathogenesis. In this review, we present the components and diverse functions of the PA system in physiology and its importance in angioedema pathogenesis. Full article
(This article belongs to the Special Issue Management and Treatment in Angioedema)
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