Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema

Andrási, Noémi; Veszeli, Nóra; Kőhalmi, Kinga Viktória; Temesszentandrási, György

doi:10.1016/j.jaip.2018.04.018

Cited by 11 publications

(9 citation statements)

References 26 publications

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“…Idiopathic nonhistaminergic acquired angioedema (InH-AAE) is a rare disease characterized by submucosal swelling without concomitant urticaria and poor response to antihistamines and corticosteroids. 1 Compared with other forms of hereditary and acquired angioedema, InH-AAE seems to have a predilection for facial and tongue swelling and is often difficult to diagnose because patients have normal laboratory values and no family history. 1 To the best of our knowledge, there have been no publications to date describing idiopathic nonhistaminergic angioedema as a complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, although nonhistaminergic angioedema has been seen in the setting of other viral infections.…”

Section: Idiopathic Nonhistaminergic Acquired Angioedema In a Patient With Coronavirus Disease 2019mentioning

confidence: 99%

“…1 Compared with other forms of hereditary and acquired angioedema, InH-AAE seems to have a predilection for facial and tongue swelling and is often difficult to diagnose because patients have normal laboratory values and no family history. 1 To the best of our knowledge, there have been no publications to date describing idiopathic nonhistaminergic angioedema as a complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, although nonhistaminergic angioedema has been seen in the setting of other viral infections. 2,3 Here, we describe a case of suspected InH-AAE in an intubated patient with coronavirus disease 2019 (COVID-19).…”

Section: Idiopathic Nonhistaminergic Acquired Angioedema In a Patient With Coronavirus Disease 2019mentioning

confidence: 99%

“…Peanut allergy affects approximately 2% of children in the United States. 1 Delayed introduction of allergenic foods was recommended by the American Academy of Pediatrics in 2000 in an effort to reduce the incidence of allergic disease. 2 This recommendation was withdrawn in 2008 owing to insufficient evidence, and no specific guidance was provided on the timing of allergenic food introduction.…”

Section: Pediatric Provider Knowledge Of Early Peanut Introduction Recommendationsmentioning

confidence: 99%

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Idiopathic nonhistaminergic acquired angioedema in a patient with coronavirus disease 2019

Azmy

Benson

Love

et al. 2020

Annals of Allergy, Asthma & Immunology

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Section: Idiopathic Nonhistaminergic Acquired Angioedema In a Patient With Coronavirus Disease 2019mentioning

confidence: 99%

Section: Idiopathic Nonhistaminergic Acquired Angioedema In a Patient With Coronavirus Disease 2019mentioning

confidence: 99%

Section: Pediatric Provider Knowledge Of Early Peanut Introduction Recommendationsmentioning

confidence: 99%

See 1 more Smart Citation

Idiopathic nonhistaminergic acquired angioedema in a patient with coronavirus disease 2019

Azmy

Benson

Love

et al. 2020

Annals of Allergy, Asthma & Immunology

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“… 47 In InH-AAE, the average age at symptom onset is 36 years, and 56% of patients have more than 12 angioedema attacks per year. 55 Attacks are most common in the face and extremities. The upper respiratory tract can be affected, and approximately 20% of cases may have abdominal and genital attacks.…”

Section: Acquired Angioedemamentioning

confidence: 99%

Angioedema without wheals: a clinical update

Gülbahar

2021

Balkan Med J

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Angioedema without wheals (urticaria) represents a heterogeneous group of clinically indistinguishable diseases of hereditary or acquired etiology. Hereditary angioedema is a rare inherited condition leading to recurrent, sometimes life-threatening angioedema attacks in subcutaneous tissues and gastrointestinal and oropharyngeal mucosa dating back to childhood or adolescence. Most of these patients have mutations in the SERPING1 gene, causing either low C1 inhibitor production (hereditary angioedema with C1 inhibitor deficiency type I) or the production of dysfunctional C1 inhibitor (hereditary angioedema with C1 inhibitor deficiency type II). Hereditary angioedema with normal C1 inhibitor has been defined later. Although C1 inhibitor concentration and function are in the normal range, it leads to typical hereditary angioedema symptoms owing to mutations in FXII, PLG, ANGPT1, KNG1 , and MYOF genes. Patients who exhibit none of these genetic mutations despite having a similar clinical presentation are classified as having unknown hereditary angioedema. Fewer than 1 in 10 patients with C1 inhibitor deficiency have acquired angioedema with C1 inhibitor deficiency. The clinical presentation is very similar to that of hereditary angioedema, making it difficult to distinguish these 2 conditions clinically. Unlike hereditary angioedema, there are no genetic mutations, and family history and symptoms tend to appear later in life. Acquired angioedema with C1 inhibitor deficiency is commonly associated with lymphoproliferative and autoimmune diseases. Angioedema attacks might start 1 year before the underlying disease in acquired angioedema with C1 inhibitor deficiency. Approximately half of the patients admitted to the hospital for acute angioedema are patients receiving angiotensin-converting enzyme (ACE) inhibitor therapy. Angioedema typically occurs on the lips, tongue, mouth, pharynx, and subglottic regions. Patients may require hospitalization and intensive care monitoring owing to airway involvement. Idiopathic histaminergic acquired angioedema may be diagnosed only when any possible causes of histaminergic angioedema are excluded (foods, drugs, animal dander, aeroallergens, insect stings, latex, and others), and the symptoms respond well to antihistamine treatment. Idiopathic nonhistaminergic acquired angioedema should be considered when all other types of recurrent angioedema have been ruled out and patients do not respond to high-dose antihistamines. The lack of a standard biochemical laboratory test for patients with idiopathic histaminergic acquired angioedema, idiopathic nonhistaminergic acquired angioedema, angiotensin-converting enzyme inhibitor-induced acquired angioedema, and hereditary angioedema with normal C1 inhibitor makes the diagnosis more challenging. Future efforts should focus on increasing awareness of all the rare types of angioedema among physicians and developing more straightforward and more accessible diagnostic meth...

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“…In rare cases not accompanied by classical wheals of urticaria, co-existing pruritus, flushing, flare and a positive response to antihistamines and corticosteroids also helps to distinguish mast-cell/histamine-mediated isolated idiopathic angioedema from that mediated by bradykinin. [ 15 ] As histaminergic angioedema is much more common than HAE, it is prudent to give a trial of high-dose antihistamines (double of the typical dosages) to see if the angioedema responds to antihistamines, especially in patients who have not received antihistamines before. As HAE would not respond to antihistamines, but rare causes of isolated recurrent histaminergic angioedema would, this can help to avoid extensive investigations in patients having histaminergic angioedema.…”

Section: Differential Diagnosismentioning

confidence: 99%

Hereditary Angioedema

Jindal

Bishnoi

Dogra

2021

Indian Dermatology Online Journal

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Hereditary angioedema (HAE) is an uncommon disorder with a global prevalence of approximately 1 in 10,000 to 1 in 50,000 population. This disease is grossly underrecognized in India because of lack of awareness and/or lack of diagnostic facilities. Clinical manifestations include swelling over face, eyes, lips, hands, feet, and genitals, abdominal pain, and life-threatening laryngeal edema. HAE should be suspected in all patients who present with angioedema without wheals and who do not respond to antihistamines and/or steroids. C1 levels, C1-INH levels, and C1-INH function should be checked in all patients suspected to have HAE. C1q levels should be assessed in patients with suspected autoimmune-mediated acquired angioedema. Management of HAE constitutes the treatment of acute attack and short-term and long-term prophylaxis. Because of lack of all first-line recommended medications, the management of HAE in India is a challenging task. Patients are managed using fresh frozen plasma (acute treatment), tranexamic acid, and attenuated androgens (prophylaxis). Even though attenuated androgens have been shown to be effective in the prevention of attacks of HAE, the side effect profile especially in children and in females is a serious concern. Hence, the treatment needs to be individualized considering the risk-benefit ratio of long-term prophylaxis. In this review, we provide an overview of diagnostic strategy for patients with HAE and the current treatment concepts with emphasis on currently available treatment options in resource-constrained settings.

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Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema

Cited by 11 publications

References 26 publications

Idiopathic nonhistaminergic acquired angioedema in a patient with coronavirus disease 2019

Idiopathic nonhistaminergic acquired angioedema in a patient with coronavirus disease 2019

Angioedema without wheals: a clinical update

Hereditary Angioedema

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