Prospective Study on the Efficacy and Impact of Cascade Screening and Evaluation of Hereditary Angioedema (CaSE-HAE)

Wong, Jane Chi Yan; Chiang, Valerie; Lam, Ki; Tung, Edmund; Au, Elaine; Lau, Chak Sing; Li, Philip H.

doi:10.1016/j.jaip.2022.07.035

Cited by 8 publications

(5 citation statements)

References 24 publications

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“…Family screening including parents, siblings, grandparents, and grandchildren must be carried out as soon as the diagnosis is established for the index case [ 3 ]. As has been reported in several studies, this helps in preventing mortality and also improves the quality of life of these patients [ 12 ]. In our own experience (unpublished data), the risk of death is very high in undiagnosed patients.…”

Section: Suggested Diagnostic Evaluation For Hae and Family Screening...mentioning

confidence: 70%

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Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Jindal

Sil

Aggarwal

et al. 2023

Asia Pacific Allergy

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Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., “on-demand treatment”) and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for “on-demand treatment,” STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both “on-demand treatment” and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines.

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Section: Suggested Diagnostic Evaluation For Hae and Family Screening...mentioning

confidence: 70%

“…C4 is a low-cost, easily available, and sensitive test for the diagnosis of HAE [ 12 ]. C4 levels are low in ~80% of patients [ 13 ].…”

Section: Suggested Diagnostic Evaluation For Hae and Family Screening...mentioning

confidence: 99%

Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Jindal

Sil

Aggarwal

et al. 2023

Asia Pacific Allergy

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“…Factors such as stress and anxiety in anticipation of future attacks (as well as their psychosocial consequences), individual perception of disease, side effects, and cost of medications have been shown to affect HRQoL but may not be adequately captured by the AAS alone. 14 , 19 , 20 , 21 , 22 Indeed, these 3 distinct PROMs aim to assess different, albeit related, concepts. Therefore, we advocate simultaneous use of the 3 PROMS to better reflect the overall disease burden.…”

Section: Resultsmentioning

confidence: 99%

Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema

Mak,

Wong,

et al. 2024

Journal of Allergy and Clinical Immunology: Global

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“…Specialists may offer immunosuppressive therapies or biologics for CSU, and specialized allergological or immunological tests are sometimes needed to exclude immediate-type allergies or confirm diagnoses of C1-INH deficiency, respectively ( 15 ). Although specialist-level care can significantly improve the management of severe CSU or C1-INH deficiency, these services and tests remain limited given the unmet provision of immunology and allergy (I&A) services in many parts of the world, including Hong Kong and Macau ( 10 , 16 , 17 ). Furthermore, there is a lack of clear guidance for indications or pathways for specialist management.…”

Section: Introductionmentioning

confidence: 99%

“…The prototype of bradykininmediated angioedema is HAE, an autosomal-dominant disorder characterized by recurrent angioedema due to decreased levels of functional C1 esterase inhibitor (C1-INH), which requires disease-specific medications (7,8). Erroneously missed or misdiagnosis of C1-INH deficiency may lead to delays in treating life-threatening angioedema or, conversely, inappropriate use and wastage of expensive medications and resources (9)(10)(11)(12)(13).…”

Section: Introductionmentioning

confidence: 99%

Hong Kong–Macau Severe Hives and Angioedema Referral Pathway

Li,

Au,

Cheong

et al. 2023

Front. Allergy

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BackgroundUrticaria (defined as the presence of hives, angioedema, or both) can be caused by a variety of etiologies ranging from more common conditions such as chronic spontaneous urticaria (CSU) to rarer conditions such as hereditary angioedema (HAE). Specialist referral may be necessary in cases of severe urticaria or HAE, but access to specialist services remains limited in certain regions, such as the Greater Bay Area (GBA) of China. To address this, the Hong Kong–Macau Severe Hives and Angioedema Referral Pathway (SHARP) was initiated by the Hong Kong Institute of Allergy and Macau Society of Dermatology to promote multidisciplinary collaboration and regional exchange of expertise in the diagnosis and management of severe urticaria.MethodsA nominated task force of dermatologists and immunologists who manage patients with severe urticaria formulated the consensus statements (CS) using the Delphi method. The consensus was defined a priori as an agreement of ≥80%.ResultsA total of 24 CS were formulated, including four statements on classifications and definitions, seven statements on diagnosis, and 13 statements on management and referral. The definitions for acute/chronic urticaria and severe CSU were stated. Unnecessary investigations and inappropriate medications were discouraged. The characteristics and recommended approach to suspected bradykinergic angioedema were specified. Stepwise treatment options using second-generation antihistamines, omalizumab, or cyclosporin for patients with CSU were addressed, and the importance of access to HAE-specific medications was emphasized. Furthermore, an integrated referral pathway for patients with severe hives and angioedema was constructed.ConclusionThe SHARP provides guidance for the management and specialist referral of patients with severe hives and angioedema in Hong Kong and Macau.

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Prospective Study on the Efficacy and Impact of Cascade Screening and Evaluation of Hereditary Angioedema (CaSE-HAE)

Cited by 8 publications

References 24 publications

Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema

Hong Kong–Macau Severe Hives and Angioedema Referral Pathway

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