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Keywords:

  • attenuated androgen;
  • hereditary angioedema;
  • long-term prophylaxis

Eur J Clin Invest 2011; 41 (3): 256–262

Abstract

Background  No systematic study has been published yet on the long-term efficacy of attenuated androgens in hereditary angioedema (HAE). Our aim was to conduct a follow-up study in two (German and Hungarian) cohorts of HAE patients (45 and 39 patients, respectively) undergoing uninterrupted treatment for 6 years with similar (starting dose 128 ± 78 mg per day and 136 ± 70 mg per day, respectively) and constant doses of danazol.

Design  The frequencies of subcutaneous, abdominal and laryngeal attacks were recorded each year.

Results  The annual frequency of all the three types of attacks was significantly lower during the first year of danazol treatment, compared to the last year before baseline. During subsequent years in Hungarian patients, the frequency of both subcutaneous and abdominal attacks – but not that of laryngeal attacks – increased significantly. In the case of abdominal attacks, a significant increase in the attack frequency was observed only in female patients. In the German cohort, by contrast, no change in the frequency of either type of attack was found during the 6-year study period.

Conclusions  The differences observed between these cohorts cannot be related to drug dose, the age or gender distribution of subjects or the age at the onset of symptoms or the length of diagnostic delay in the patients. There were, however, marked differences in the baseline pattern of attacks: significantly – 3 times – more abdominal attacks were recorded in German patients. Further studies are necessary to clarify the mechanism of these findings.