[Hereditary angioedema. Effect of danazol on C4 and functional C1INH].
Hereditary angioedema (HAE) is an inherited deficiency of the inhibitor of C1 esterase (C1 inh). Two types of genetic C1 inh deficiency have been described, type I: quantitative, and type II: functional. For the purpose of the present study, 4 out of 51 HAE patients were selected. None of them had received any previous androgenic therapy. The group was integrated by two type I and one type II cases. All patients that entered in the protocol received 400 mg/day of danazol over 14 days. The complement system was evaluated by monitoring C4, Hemolytic complement 50% (CH50), Circulating Immune Complexes (CIC), and antigenic and functional C1 INH during the study. The level of the complement factors at the beginning and the end of this period demonstrated a statistically significant increase of C4 and CH50 and the disappearance of CIC, while C1INH remained unmodified. These results suggest that the therapeutic effect of Danazol may have two mechanisms of action: i. promotion of C4 synthesis by anabolic effect resulting in an improvement of the complement system with the disappearance of CIC, and ii, a minor increase of C1 inh level primarily due to the lack of its consumption.
Fabiani
J E
JE
Paulin
P
P
Simkin
G
G
Leoni
J
J
Palombarani
S
S
Squiquera
L
L
D000328:Adult; D000799:Angioedema / Q000145:classification / Q000188:drug therapy* / Q000276:immunology; D000936:Antigen-Antibody Complex / Q000032:analysis; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000378:metabolism; D003181:Complement C4 / Q000032:analysis*; D003613:Danazol / Q000494:pharmacology* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011245:Pregnadienes / Q000494:pharmacology*
D000936:Antigen-Antibody Complex; D003174:Complement C1 Inactivator Proteins; D003181:Complement C4; D011245:Pregnadienes; D003613:Danazol
Angioedema hereditario (AEH). Acción del danazol sobre el C4 y C1INH funcional.