Prospective Analysis in Patients With HAE Under Prophylaxis With Lanadelumab: A Real-life Experience

Hahn, Janina; Trainotti, Susanne; Wigand, Marlene C.; Schuler, Patrick J.; Hoffmann, Thomas; Greve, Jens

doi:10.36849/jdd.2020.5269

Cited by 14 publications

(18 citation statements)

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“…With novel prophylactic treatment options for HAE, disease control and associated mental comorbidities are expected to improve. However, due to high costs, the availability of modern drugs will be likely limited to a selected group of patients worldwide [19,[45][46][47][48].…”

Section: Discussionmentioning

confidence: 99%

Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany

Zarnowski

Rabe

Kage

et al. 2021

Int Arch Allergy Immunol

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Background: Hereditary angioedema (HAE) is associated with relevant disease-related burden. We aimed at investigating prevalence of depression and anxiety in patients with HAE in Leipzig, Germany. Methods: Questionnaire-based evaluation of medical history, Angioedema Control Test (AECT), Angioedema Quality of Life Questionnaire (AE-QoL), Generalized Anxiety Disorder Scale-7 (GAD-7), and Hospital Anxiety and Depression Scale (HADS). Results: Thirty-seven patients with HAE were included (31 females, mean age 49.6 ± 17.5 years). A mean diagnostic delay between first symptoms and correct diagnosis of 14.2 ± 14.5 years was detected. Patients aged <50 years (n = 18) had been diagnosed significantly earlier with HAE than older patients (p = <0.001). In 6 patients (16.2%), unnecessary medical interventions were performed and 14 patients (43.8%) reported at least 1 HAE-related death of a family member. Psychological stress was the most common triggering factor (96.2%). HADS scores revealed depression in 5/37 patients (13.5%) and anxiety in 16/37 (43.2%), GAD-7 score indicated anxiety in 9/36 (25%) participants. Patients receiving long-term prophylactic treatment (n = 17, 45.9%) showed significantly better disease control (AECT; p = <0.001) and quality of life (AE-QoL; p = <0.001) compared to those with on-demand treatment only. Patients with long-term prophylactic treatment showed significantly lower scores for anxiety and depression at GAD-7 (p = 0.011) and HADS (anxiety: p = 0.021; depression: p = 0.008). In 5 patients, treatment regime was changed as AECT score indicated insufficient disease control. Subsequently, we measured significant improvement of quality of life (AE-QoL, p = 0.04) and disease control (AECT; p = 0.032). Conclusion: Anxiety was a frequent burden in our study group and showed a significant association with low disease control. Our data indicate that prophylactic HAE treatment can improve psychosocial burden of HAE.

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Section: Discussionmentioning

confidence: 99%

Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany

Zarnowski

Rabe

Kage

et al. 2021

Int Arch Allergy Immunol

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“…We, therefore, recommend evaluating patients with HAE for LTP at every visit, taking disease activity, burden, and control as well as patient preference into consideration (Recommendation 14). 182 , 194 , 195 , 196 , 197 , 198 , 199 , 200 , 201 As all of these factors can vary over time, all patients should be evaluated for LTP at least once a year. The goal of LTP is to achieve full control of disease burden while attempting to minimize treatment burden and side effects.…”

Section: Long-term Prophylactic Treatment Of Haementioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

Maurer

Magerl

Betschel

et al. 2022

World Allergy Organization Journal

120

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“…Although further evidence is necessary, these data demonstrate that lanadelumab can dramatically improve QoL in patients with C1-INH-HAE, overcoming some limits of existing long-term prophylactic drugs such as adverse effects [ 99 ], need for frequent intravenous administrations [ 76 ], and sometimes limited availability, due to a shortage in supply (particularly in the case of plasma derived products) [ 100 ]. Moreover, a gradual extension of intervals between lanadelumab injections may be achieved without losing therapeutic efficacy, reducing the still high costs and furthering the idea of an individualized therapy [ 101 ].…”

Section: Main Textmentioning

confidence: 99%

Quality of life in patients with allergic and immunologic skin diseases: in the eye of the beholder

et al. 2021

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Allergic and immunologic skin diseases negatively impact the quality of life (QoL) of affected patients with detrimental consequences. Nonetheless, in everyday clinical practice the evaluation of QoL is often overlooked. Considering the increasing prevalence of atopic dermatitis, allergic contact dermatitis, hereditary angioedema, cutaneous mastocytosis, and urticaria, it is essential to determine the effects of allergic and immunologic skin diseases on QoL. A joint meeting (GET TOGETHER 2021) of the Italian Society of Allergology, Asthma and Clinical Immunology (SIAAIC) and the Italian Society of Allergological, Occupational and Environmental Dermatology (SIDAPA) aimed to summarize the features of the main QoL tools used in these diseases and to describe the extent of QoL impairment as well as the impact of treatments on QoL, particularly biologic therapies. The assessment of QoL in patients with allergic and immunologic skin diseases relies on generic, organ-specific and disease-specific questionnaires. While generic and organ-specific questionnaires allow comparison between different diseases, disease-specific questionnaires are designed and validated for specific cohorts: the QoL Index for Atopic Dermatitis (QoLIAD) and the Childhood Atopic Dermatitis Impact Scale (CADIS) in atopic dermatitis, the ACD-11 in allergic contact dermatitis, the Angioedema QoL Questionnaire (AE-QoL) and the Hereditary Angioedema QoL questionnaire (HAE-QoL) in hereditary angioedema, the Mastocytosis QoL Questionnaires (MCQoL e MQLQ) in cutaneous mastocytosis, and the Chronic Urticaria QoL questionnaire (CU-Q2oL) in urticaria. Among the many factors that variably contribute to QoL impairment, pruritus can represent the leading cause of patient discomfort. Biologic therapies significantly ameliorate QoL in atopic dermatitis, hereditary angioedema, mastocytosis and chronic urticaria. In general, adequate management strategies are essential for improving QoL in patients with allergic and immunologic skin diseases.

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Prospective Analysis in Patients With HAE Under Prophylaxis With Lanadelumab: A Real-life Experience

Cited by 14 publications

References 0 publications

Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany

Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

Quality of life in patients with allergic and immunologic skin diseases: in the eye of the beholder

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