Abstract
Background: Hereditary angioedema (HAE) is a primary immunodeficiency disorder characterized by C1 complement inhibitor deficiency and unregulated activation of complement. Aspirin hypersensitivity is related to an increase in the amount of leukotrienes with eosinophil and mast cell activation and increased levels of glandular kallikrein with upregulated local conversion of bradykinin. Both conditions can be present in the same patient. Objectives: We present five patients with HAE; they were all being treated in similar ways according to the therapeuthic options available in our institute (danazol). However, three of them had recurrent episodes of angioedema; in these cases, it was identified aspirin hypersensitivity as a cause of poor disease control. A review of the literature is included. Case Presentation: We present the cases of four females and one male (age range 21 - 58 years) with type I HAE. Subjects were all ISSSTE beneficiaries (state workers) treated at the National Medical Center “20 de Noviembre”. Aspirin hypersensitivity was identified in three patients. Elimination of NSAIDs along with dietary elimination of high salicylate-containing foods improved control of angioedema crisis (severe and/ or recurrent episodes). Discussion: Aspirin hypersensitivity was identified as a factor for poor control in our patients with HAE. Such cases improved with dietary elimination of high salicylate-containing foods and avoidance of NSAIDs. Conclusions: This is the first report of patients with HAE and aspirin hypersensitivity as a cause of poor control. We recommend a deliberate search of these comorbidities, especially in cases of poor disease control. Further studies are needed to continue the investigation on this topic.
Highlights
Hereditary angioedema (HAE) is a primary immunodeficiency with autosomal dominant transmission [OMIM 106100-SERPING1 gene located on chromosome 11 (p.11.2-q.13)] [1] [2] caused by a quantitative or qualitative deficiency (85% - 90%) of the complement component inhibitor factor (C1-Inh) and, in a lower proportion, as a result of mutations associated with coagulation factor XII [2] [3]
Hereditary history (80% positive) and clinical evolution together with low levels of complement factor C4 were the primary data for diagnostic suspicion
Four patients were hospitalized and required specialized interventions at some time during their evolution, three of which were associated with severe events of angioedema and two episodes of urticaria with NSAID ingestion
Summary
Hereditary angioedema (HAE) is a primary immunodeficiency with autosomal dominant transmission [OMIM 106100-SERPING1 gene located on chromosome 11 (p.11.2-q.13)] [1] [2] caused by a quantitative or qualitative deficiency (85% - 90%) of the complement component inhibitor factor (C1-Inh) and, in a lower proportion, as a result of mutations associated with coagulation factor XII (chromosome 5q) [2] [3]. Aspirin hypersensitivity is related to an increase in the amount of leukotrienes with eosinophil and mast cell activation and increased levels of glandular kallikrein with upregulated local conversion of bradykinin. Both conditions can be present in the same patient. Three of them had recurrent episodes of angioedema; in these cases, it was identified aspirin hypersensitivity as a cause of poor disease control. Discussion: Aspirin hypersensitivity was identified as a factor for poor control in our patients with HAE. Further studies are needed to continue the investigation on this topic
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