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Allergy Mar 2022This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations...
This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA²LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), the European Dermatology Forum (EDF; EuroGuiDerm), and the Asia Pacific Association of Allergy, Asthma and Clinical Immunology with the participation of 64 delegates of 50 national and international societies and from 31 countries. The consensus conference was held on 3 December 2020. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease that presents with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous or inducible urticaria is disabling, impairs quality of life, and affects performance at work and school. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert-guided and evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria.
Topics: Angioedema; Asthma; Chronic Disease; Humans; Prevalence; Quality of Life; Urticaria
PubMed: 34536239
DOI: 10.1111/all.15090 -
Advances in Therapy Mar 2023Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a mutation in the C1 esterase inhibitor gene. HAE affects 1/50,000 people worldwide. Three main... (Review)
Review
Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a mutation in the C1 esterase inhibitor gene. HAE affects 1/50,000 people worldwide. Three main types of HAE exist: type I, type II, and type III. Type I is characterized by a deficiency in C1-INH. C1-INH is important in the coagulation complement, contact systems, and fibrinolysis. Most HAE cases are type I. Type I and II HAE result from a mutation in the SERPING1 gene, which encodes C1-INH. Formally known as type III HAE is typically an estrogen-dependent or hereditary angioedema with normal C1-INH activity. Current guidelines now recommend subdividing hereditary angioedema with normal C1 esterase inhibitor gene (HAE-nl-C1-INH formerly known as HAE type III) based on underlying mutations such as in kininogen-1 (HAE-KNG1), plasminogen gene (PLG-HAE), myoferlin gene mutation (MYOF-HAE), heparan sulfate-glucosamine 3-sulfotransferase 6 (HS3ST6), mutation in Hageman factor (factor XII), and in angiopoietin-1 (HAE-ANGPT-1). The clinical presentation of HAE varies between patients, but it usually presents with nonpitting angioedema and occasionally abdominal pain. Young children are typically asymptomatic. Those affected by HAE usually present with symptoms in their early 20s. Symptoms can arise as a result of stress, infection, or trauma. Laboratory testing shows abnormal levels of C1-INH and high levels of bradykinin. C4 and D-dimer levels can also be monitored if an acute HAE attack is suspected. Acute treatment of HAE can include IV infusions of C1-INH, receptor antagonists, and kallikrein inhibitors. Short- and long-term prophylaxis can also be administered to patients with HAE. First-line therapies for long-term prophylaxis also include IV infusion of C1-INH. This review aims to thoroughly understand HAE, its clinical presentation, and how to treat it.
Topics: Child; Humans; Child, Preschool; Angioedemas, Hereditary; Complement C1 Inhibitor Protein; Mutation
PubMed: 36609679
DOI: 10.1007/s12325-022-02401-0 -
WMJ : Official Publication of the State... Apr 2022Hereditary angioedema (HAE) is a rare and disabling disorder wherein there is excessive bradykinin production, with subsequent increased vascular permeability in the... (Review)
Review
Hereditary angioedema (HAE) is a rare and disabling disorder wherein there is excessive bradykinin production, with subsequent increased vascular permeability in the superficial tissues and gastrointestinal and respiratory mucosa. This article serves as a review of the pathogenesis of the disease, as well as an update of the evidence-based new treatment recommendations to help clinicians with the diagnosis and management of HAE.
Topics: Angioedemas, Hereditary; Blindness; Bradykinin; Complement C1 Inhibitor Protein; Humans
PubMed: 35442579
DOI: No ID Found -
Blood May 2023
Topics: Humans; Angioedemas, Hereditary; Complement C1 Inhibitor Protein; Thrombosis; Venous Thrombosis; Blood Coagulation
PubMed: 37166925
DOI: 10.1182/blood.2023019861 -
The Journal of Allergy and Clinical... Jan 2021Scientific and clinical progress together with the development of effective novel therapeutic options has engendered multiple important changes in the diagnosis and...
Scientific and clinical progress together with the development of effective novel therapeutic options has engendered multiple important changes in the diagnosis and management of hereditary angioedema (HAE). We now update and extend the 2013 United States Hereditary Angioedema Association Medical Advisory Board guidelines for the treatment and management of HAE. The guidelines are based on a comprehensive literature review with recommendations indicating both the strength of our recommendation and the quality of the underlying evidence. Guidelines are provided regarding the classification, diagnosis, on-demand treatment, prophylactic treatment, special considerations for women and children, development of a comprehensive management and monitoring plan, and assessment of burden of illness for both HAE due to C1 inhibitor deficiency and HAE with normal C1 inhibitor. Advances in HAE treatment now allow the development of management plans that can help many patients with HAE lead a normal life. Achieving this goal requires that physicians be familiar with the diagnostic and therapeutic transformations that have occurred in recent years.
Topics: Advisory Committees; Angioedemas, Hereditary; Child; Complement C1 Inhibitor Protein; Female; Humans; Physicians; United States
PubMed: 32898710
DOI: 10.1016/j.jaip.2020.08.046 -
Polskie Archiwum Medycyny Wewnetrznej 2016Angioedema and urticaria often constitute a challenge in daily clinical practice. They may either co- -occur or present as independent conditions. They are characterized... (Review)
Review
Angioedema and urticaria often constitute a challenge in daily clinical practice. They may either co- -occur or present as independent conditions. They are characterized by a complex pathomechanism, and their symptoms may be triggered by diverse factors. These differences are crucial for developing a successful treatment regimen. Both conditions may have an allergic origin (immunoglobulin [Ig] E and non-IgE-related), usually induced by histamine, or a nonallergic one, such as bradykinin-mediated angioedema in patients with C1 inhibitor (C1-INH) deficiency or angioedema induced by certain drugs (eg, angiotensin-converting enzyme inhibitors). Currently, we distinguish 5 types of nonallergic angioedema: hereditary angioedema (HAE) due to C1-INH deficiency, acquired angioedema (AAE), and angioedema induced by the renin-angiotensin-aldosterone system, all of which are mediated by bradykinin, as well as pseudoallergic angioedema and idiopathic angioedema. Bradykinin-mediated angioedema (eg, laryngeal angioedema) may be life-threatening because of resistance to corticosteroids and antihistamine drugs. C1-INH concentrates are the drugs of choice in the treatment of HAE and AAE. In recent years, some new drugs have been introduced in the treatment of bradykinin-mediated angioedema, such as bradykinin B2-receptor antagonist, icatibant, and kallikrein inhibitor, ecallantide, which allow to improve treatment outcomes.
Topics: Angioedema; Angioedemas, Hereditary; Angiotensin-Converting Enzyme Inhibitors; Bradykinin; Humans
PubMed: 26842379
DOI: 10.20452/pamw.3273 -
Deutsches Arzteblatt International Jul 2017Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated... (Review)
Review
BACKGROUND
Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different.
METHODS
This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. The authors draw on their own clinical experience in their assessment of the literature.
RESULTS
In the emergency clinical situation, the most important information comes from accompanying manifestations such as itching and urticaria and from the patient's drug history and family history. When angioedema affects the head and neck, securing the upper airways is the highest priority. Angioedema is most commonly caused by mast-cell mediators, such as histamine. This type of angioedema is sometimes accompanied by urticaria and can be effectively treated with antihistamines or glucocorticoids. In case of a severe allergic reaction or anaphylaxis, epinephrine is given intramuscularly in a dose that is adapted to the patient's weight (150 μg for body weight >10 kg, 300 μg for body weight >30 kg). Bradykinin-mediated angioedema may arise as either a hereditary or an acquired tendency. Acquired angioedema can be caused by angiotensin converting enzyme (ACE) inhibitors and by angiotensin II receptor blockers. Bradykinin-mediated angioedema should be treated specifically with C1-esterase inhibitor concentrates or bradykinin-2 receptor antagonists.
CONCLUSION
Angioedema of the upper airways requires a well-coordinated diagnostic and therapeutic approach. Steroids and antihistamines are very effective against mast-cell-mediated angioedema, but nearly useless against bradykinin-mediated angioedema. For angioedema induced by ACE inhibitors, no causally directed treatment has yet been approved.
Topics: Anaphylaxis; Angioedema; Angiotensin-Converting Enzyme Inhibitors; Bradykinin; Drug Hypersensitivity; Glucocorticoids; Histamine Antagonists; Humans
PubMed: 28818177
DOI: 10.3238/arztebl.2017.0489 -
International Journal of Molecular... Aug 2021Angioedema is a life-threatening emergency event that is associated with bradykinin and histamine-mediated cascades. Although bradykinin-mediated angioedema currently... (Review)
Review
Angioedema is a life-threatening emergency event that is associated with bradykinin and histamine-mediated cascades. Although bradykinin-mediated angioedema currently has specific therapeutic options, angioedema is sometimes intractable with current treatments, especially histamine-mediated angioedema, suggesting that some other mediators might contribute to the development of angioedema. Fatty acids are an essential fuel and cell component, and act as a mediator in physiological and pathological human diseases. Recent updates of studies revealed that these fatty acids are involved in vascular permeability and vasodilation, in addition to bradykinin and histamine-mediated reactions. This review summarizes each fatty acid's function and the specific receptor signaling responses in blood vessels, and focuses on the possible pathogenetic role of fatty acids in angioedema.
Topics: Angioedema; Bradykinin; Capillary Permeability; Fatty Acids; Histamine; Humans; Prostaglandins
PubMed: 34445711
DOI: 10.3390/ijms22169000 -
Tidsskrift For Den Norske Laegeforening... Nov 2012Angioedema has numerous hereditary, acquired and iatrogenic causes. A number of studies show that angioedema is inadequately assessed and treated during its acute phase... (Review)
Review
BACKGROUND
Angioedema has numerous hereditary, acquired and iatrogenic causes. A number of studies show that angioedema is inadequately assessed and treated during its acute phase as well as in the follow-up period. We present an algorithm for the assessment and treatment of patients with angioedema. KNOWLEDGE BASE: The article is based on a literature search in PubMed, a review of bibliographies and the authors' clinical experience and research.
RESULTS
The majority of angioedema patients have accompanying urticaria. Pathophysiologically, angioedemas are divided into histaminergic and non-histaminergic forms. In a large group of patients no positive trigger is identified. On assessment in hospital the most frequently identified cause is drug intake, normally angiotensin-converting-enzyme inhibitors and NSAIDs , while allergic/pseudoallergic and idiopathic reactions are more commonly seen in general practice. There are a number of rare causes of angioedema, all of which are important to keep in mind. The acute and prophylactic treatment will depend on the subtype of angioedema and is best provided through cross-disciplinary collaboration.
INTERPRETATION
Angioedema is a potentially life-threatening condition and should be assessed and treated systematically. It is important to remember that angioedema is either histaminergic or non-histaminergic, as the treatment of the two types is different.
Topics: Algorithms; Angioedema; Critical Pathways; Diagnosis, Differential; Humans; Urticaria
PubMed: 23160589
DOI: 10.4045/tidsskr.12.0470 -
Allergy and Asthma Proceedings Sep 2018
Topics: Angioedemas, Hereditary; Humans
PubMed: 30153885
DOI: 10.2500/aap.2018.39.4169