Long-term efficacy of danazol treatment in hereditary angioedema
Version of Record online: 18 OCT 2010
© 2010 The Authors. European Journal of Clinical Investigation © 2010 Stichting European Society for Clinical Investigation Journal Foundation
European Journal of Clinical Investigation
Volume 41, Issue 3, pages 256–262, March 2011
How to Cite
Füst, G., Farkas, H., Csuka, D., Varga, L. and Bork, K. (2011), Long-term efficacy of danazol treatment in hereditary angioedema. European Journal of Clinical Investigation, 41: 256–262. doi: 10.1111/j.1365-2362.2010.02402.x
- Issue online: 8 FEB 2011
- Version of Record online: 18 OCT 2010
- Received 19 March 2010; accepted 15 September 2010
- attenuated androgen;
- hereditary angioedema;
- long-term prophylaxis
Eur J Clin Invest 2011; 41 (3): 256–262
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.