AI Article Synopsis

  • Hereditary Angioedema (HAE) is a rare autosomal dominant disorder characterized by low levels or dysfunctional C1 inhibitor, leading to recurrent episodes of swelling and abdominal pain.
  • A reported case involves a 53-year-old man with a longstanding history of recurrent edema and family history, who showed low levels of plasma proteins but faced treatment challenges despite various therapies, including a trial of Lanadelumab for prophylaxis.
  • The patient's experience highlights issues in managing HAE, revealing an adverse reaction to Lanadelumab that is not commonly documented, emphasizing the need for better-informed treatment strategies.

Article Abstract

Background: Hereditary Angioedema is an autosomal dominant disorder caused by a lack or decrease in the function of the C1 inhibitor. It is a rare disease with low prevalence. Treatment focuses on symptom relief and short- and long-term prevention of acute attacks.

Case Report: 53-year-old male patient, with recurrent edema in the face, feet, scrotum, associated with abdominal pain since he was 20 years old. Patient has a history of hereditary angioedema in first-degree relatives of consanguinity. Laboratory tests showed low levels of plasma protein (antigenic), functional C1-INH and C4. The patient received several medical treatments. Given the recurrence of symptoms, prophylactic management with Lanadelumab was indicated. However, the patient presented erythema and induration at the application site with subsequent secondary generalized pruritus. For this reason, prophylaxis was suspended.

Conclusion: This case of hereditary angioedema type 1 presents the difficulty in the treatment and the relapse of the patient after trying various therapies, as well as the finding of the adverse effect presented to the prophylactic medication with Lanadelumab, which has been poorly documented in the literature medical worldwide.

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http://dx.doi.org/10.29262/ram.v71i4.1323DOI Listing

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Article Synopsis
  • Hereditary Angioedema (HAE) is a rare autosomal dominant disorder characterized by low levels or dysfunctional C1 inhibitor, leading to recurrent episodes of swelling and abdominal pain.
  • A reported case involves a 53-year-old man with a longstanding history of recurrent edema and family history, who showed low levels of plasma proteins but faced treatment challenges despite various therapies, including a trial of Lanadelumab for prophylaxis.
  • The patient's experience highlights issues in managing HAE, revealing an adverse reaction to Lanadelumab that is not commonly documented, emphasizing the need for better-informed treatment strategies.
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Idiopathic non-mast cell angioedema: Treatment insights from global experts.

Allergy Asthma Proc

January 2025

From the Division of Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, California and.

Idiopathic non-mast cell angioedema (INMA) is a rare disease typified by recurrent attacks of cutaneous and subcutaneous swelling. Every attack carries the potential for severe morbidity and, in the case of laryngeal involvement, mortality. Whereas therapies approved for hereditary angioedema (HAE) have been used in the care of patients with INMA, little is known with regard to their efficacy for the treatment of this disease.

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Background Hereditary angioedema (HAE) is a rare disorder in India, and while prevalence data is limited, it is believed that a significant number of individuals may be affected. Due to restricted access to first-line treatments, older therapies like danazol are commonly used despite associated risks in resource-constrained settings. This study aimed to assess the efficacy of danazol as an affordable long-term prophylaxis (LTP) for HAE in a three-generation family.

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Over the past two decades, guidelines for the on-demand treatment of hereditary angioedema (HAE) attacks have undergone significant evolution. Early treatment guidelines, such as the Canadian 2003 International Consensus Algorithm, often gated on-demand treatment by attack location and/or severity. Pivotal trials for on-demand injectable treatments (plasma-derived C1 esterase inhibitor [C1INH], icatibant, ecallantide [US only], recombinant C1INH), which were approved in the US and EU between 2008-2014, were designed accordingly.

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