Medical Literature - 2022

Isolated angioedema: A review of classification and update on management. [Review]

Kesh S, Bernstein JA 12/2022 Annals of Allergy, Asthma, & Immunology.

129(6):692-702

OBJECTIVE: To review the various types of angioedema including diagnosis and treatment.

DATA SOURCES: PubMed search of articles in the English language of various types of angioedema.

STUDY SELECTIONS: Articles on the subject matter were selected and reviewed.

RESULTS: Herein, a case-based approach is presented for discussing the major types of angioedema, including the following: hereditary angioedema types I and II and normal complement, acquired angioedema, angiotensin-converting enzyme-induced angioedema, and histaminergic and nonhistaminergic angioedema. Emerging treatments of hereditary angioedema including targets of prekallikrein, DNA vector technology replacing C1-INH protein, and CRIPSR technology targeting prekallikrein among many others are explored. In addition, other causes and mimickers of angioedema are briefly reviewed. Finally, a novel algorithm is proposed to help guide the treating physician through the workup and management of patients with suspected idiopathic angioedema unresponsive to conventional therapy with antihistamines.

CONCLUSION: Over the years, many strides have been made in both understanding the pathophysiology of various types of angioedema and expansion of treatment options. It is important for clinicians to be aware of current and emerging treatment options. We provide a novel practical algorithm to guide clinicians in challenging cases of idiopathic angioedema refractory to antihistamines. Copyright © 2022 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Available online at: dx.doi.org/10.1016/j.anai.2022.08.003

National audit of a hereditary and acquired angioedema cohort in New Zealand.

Lindsay K, Chua I, Jordan A, Stephens S. 12/2022 Internal Medicine Journal.

52(12):2124-2129

BACKGROUND: Hereditary angioedema (HAE) leads to significant morbidity and mortality from unpredictable intermittent peripheral, abdominal and laryngeal swelling. Access to appropriate healthcare and effective therapies, which can prevent and treat attacks, reduce the suffering and greatly improve quality of life. Although treatments such as C1 inhibitor (Berinert), and Icatibant are available in New Zealand (Aotearoa), there are no published data available on their use.

AIM: To present a national audit of HAE and acquired angioedema (AAE) in 2019.

METHODS: Patients were identfied and demographical and clinical data on HAE were collected retrospectively by interview and notes review.

RESULTS: The total number of known adult (48) and children (3) HAE and AAE (3) patients is 54. Of these, 41/54 (75%) of HAE and AAE patients were recruited to the audit. Icatibant has been available for the treatment of acute HAE attacks since 2016, and is now used in 73% of HAE patients. Icatibant is also used by patients for laryngeal attacks in the community, who may not then present to hospital. Androgens are used in half of the patients as prophylaxis, but 33% of the latter were identified as not having regular liver ultrasound screening. Tranexamic acid is used as prophylaxis in one-fifth of patients. Participants have had 40 children, half of whom may be affected. Three have been diagnosed with HAE, suggesting that the majority have not yet been tested.

CONCLUSIONS: Corrective actions arising from this audit will improve our capacity to provide long-term care for HAE patients and their families. Copyright © 2021 Royal Australasian College of Physicians.

Available online at: dx.doi.org/10.1111/imj.15466 (small fee)

Long-term Prophylaxis with Androgens in the management of Hereditary Angioedema (HAE) in emerging countries. [Review]

Guo Y, Zhang H, Lai H, Wang H, Chong-Neto HJ, Valle SOR, Zhu R. 11/2022 Orphanet Journal Of Rare Diseases.

17(1):399

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterized by repetitive subcutaneous or submucosal angioedema, activation of the kinin system, and increased vascular permeability. C1-inhibitor (C1-INH) deficiency, the main mechanism of HAE pathogenesis, occurs when abnormal activation of plasma kallikrein, bradykinin, and factor XII, or mutation of genes such as SERPING1 cause quantitative or functional C1-INH defects. Although androgens are not approved for HAE treatment in many countries, they are widely used in China and Brazil to reduce the frequency and severity of HAE attacks. The long-term adverse effects of androgen treatment are concerning for both physicians and patients. Virilization, weight gain, acne, hirsutism, liver damage, headache, myalgia, hematuria, menstrual disorders, diminished libido, arterial hypertension, dyslipidemia, and anxiety/depression are commonly observed during long-term treatment with androgens. These adverse effects can affect the quality of life of HAE patients and often lead to treatment interruption, especially in women and children. In-depth studies of the pathogenesis of HAE have led to the approval of alternative treatment strategies, including plasma-derived C1 inhibitor, recombinant human C1 inhibitor, plasma Kallikrein inhibitor (ecallantide; lanadelumab), and bradykinin B2 receptor antagonist (icatibant), some of which have achieved satisfactory results with mostly non-serious side effects. Therefore, a new standard of medical care may expand possibilities for the management of HAE in emerging countries. Copyright © 2022. The Author(s).

Available online at: dx.doi.org/10.1186/s13023-022-02536-x

Funding for Canadian Hereditary Angioedema Network has been generously provided by unrestricted grants from:

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