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Original Article


Clinical Pattern and Acute and Long-term Management of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency


Gómez-Traseira C1,4, Pérez-Fernández E2,4, López-Serrano MC1,4,5, García-Ara MC1,4,5, Pedrosa M1,4, López-Trascasa M3,4,5, Caballero T1,4,5

1Allergy Department, Hospital Universitario La Paz, Madrid, Spain
2Biostatistics Section, Research Unit, Hospital Universitario La Paz, Madrid, Spain
3Immunology Unit, Hospital Universitario La Paz, Madrid, Spain
4Hospital Universitario La Paz Institute for Health Research (IdiPAZ), Madrid, Spain
5Biomedical Research Network on Rare Diseases-U754 (CIBERER)

J Investig Allergol Clin Immunol 2015; Vol. 25(5): 358-364



Background: Hereditary angioedema due to C1-esterase inhibitor deficiency (HAE-C1-INH) is a life-threatening disease.

Objectives: To describe the clinical characteristics and management of patients with HAE-C1-INH during routine clinical practice.

Methods: An observational, retrospective study was performed in patients with HAE-C1-INH. Demographic, clinical, and analytical data were collected from 2 periods: period A (October 2009-September 2010) and period B (October 2007-September 2009).

Results: We studied 112 patients with HAE-C1-INH (57.1% females). Age at onset of symptoms was 14.4 years (lower in patients who had experienced attacks in the previous year). In period B (n=87), 62.1% of patients presented at least 1 edema attack (median, 3.5 attacks/patient/2 years), and 19.1% of attacks were treated. In period A (n=77), 58.4% of patients were on maintenance therapy. Stanozolol was the most widely used drug (48.9%), with a mean weekly dose of 6.7 mg. At least 1 attack was recorded in 72.7% of patients (median, 3.0 attacks/patient/year), and 31.5% of the attacks were treated. Treatment of acute attacks increased by 12.4%.

Conclusion: Age at onset of symptoms is associated with clinical expression of disease. The higher age at onset of symptoms, the fewer number of attacks per patient and year, and the lower dose of attenuated androgens necessary to control the disease than in other series lead us to hypothesize that HAE-C1-INH could have a less severe expression in Spain. Acute attacks seem to be treated increasingly often.

Key words: Bradykinin. C1-esterase inhibitor. C1-esterase inhibitor deficiency. Hereditary angioedema. Treatment.