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.
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 HAEpatients (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.
Authors: Marcus Maurer; Markus Magerl; Stephen Betschel; Werner Aberer; Ignacio J Ansotegui; Emel Aygören-Pürsün; Aleena Banerji; Noémi-Anna Bara; Isabelle Boccon-Gibod; Konrad Bork; Laurence Bouillet; Henrik Balle Boysen; Nicholas Brodszki; Paula J Busse; Anette Bygum; Teresa Caballero; Mauro Cancian; Anthony J Castaldo; Danny M Cohn; Dorottya Csuka; Henriette Farkas; Mark Gompels; Richard Gower; Anete S Grumach; Guillermo Guidos-Fogelbach; Michihiro Hide; Hye-Ryun Kang; Allen P Kaplan; Constance H Katelaris; Sorena Kiani-Alikhan; Wei-Te Lei; Richard F Lockey; Hilary Longhurst; William Lumry; Andrew MacGinnitie; Alejandro Malbran; Inmaculada Martinez Saguer; Juan José Matta Campos; Alexander Nast; Dinh Nguyen; Sandra A Nieto-Martinez; Ruby Pawankar; Jonathan Peter; Grzegorz Porebski; Nieves Prior; Avner Reshef; Marc Riedl; Bruce Ritchie; Farrukh Rafique Sheikh; William B Smith; Peter J Spaeth; Marcin Stobiecki; Elias Toubi; Lilian Agnes Varga; Karsten Weller; Andrea Zanichelli; Yuxiang Zhi; Bruce Zuraw; Timothy Craig Journal: World Allergy Organ J Date: 2022-04-07 Impact factor: 5.516
Authors: Stephen Betschel; Jacquie Badiou; Karen Binkley; Jacques Hébert; Amin Kanani; Paul Keith; Gina Lacuesta; Bill Yang; Emel Aygören-Pürsün; Jonathan Bernstein; Konrad Bork; Teresa Caballero; Marco Cicardi; Timothy Craig; Henriette Farkas; Hilary Longhurst; Bruce Zuraw; Henrik Boysen; Rozita Borici-Mazi; Tom Bowen; Karen Dallas; John Dean; Kelly Lang-Robertson; Benoît Laramée; Eric Leith; Sean Mace; Christine McCusker; Bill Moote; Man-Chiu Poon; Bruce Ritchie; Donald Stark; Gordon Sussman; Susan Waserman Journal: Allergy Asthma Clin Immunol Date: 2014-10-24 Impact factor: 3.406
Authors: A L Manson; A Price; J Dempster; P Clinton-Tarestad; C Greening; R Enti; S Hill; S Grigoriadou; M S Buckland; H J Longhurst Journal: Clin Exp Immunol Date: 2013-07 Impact factor: 4.330
Authors: Richard G Gower; Paula J Busse; Emel Aygören-Pürsün; Amin J Barakat; Teresa Caballero; Mark Davis-Lorton; Henriette Farkas; David S Hurewitz; Joshua S Jacobs; Douglas T Johnston; William Lumry; Marcus Maurer Journal: World Allergy Organ J Date: 2011-02 Impact factor: 4.084