[Bradykinin mediated angioedema].
Bradykinin angioedema (AE) are characterized by acute recurrent episodes of localized swelling. They are not associated with pruritus or erythema, and are short-lived (24 to 72 hours), disappearing without any sequelae. Corticosteroids are useless. Skin or mucous membranes (upper respiratory and intestinal) could be affected. Bradykinin AE can be secondary to: (1) AE associated with C1 inhibitor deficiency (hereditary or acquired); (2) drug-induced AE (converting enzyme inhibitors…); (3) type III AE type (oestrogen dependant) without C1 inhibitor deficiency. These type III AE can be associated with a gain of function mutation that markedly increases factor XII activity. Prognosis depends on the laryngeal attacks (resulting in 25 % of death in the absence of specific treatment). In case of severe attacks, icatibant (bradykinin receptor antagonist) or C1 inhibitor concentrate can be used. In case of frequent attacks, long-term therapy with danazol or tranexamic acid is effective.
Bouillet
L
L
Centre de référence sur les angiœdèmes bradykiniques (CREAK), clinique universitaire de médecine interne, BP 217, 38043 Grenoble cedex 09, France. LBouillet@chu-grenoble.fr
Boccon-Gibod
I
I
Massot
C
C
D000799:Angioedema / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology; D054179:Angioedemas, Hereditary / Q000235:genetics; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001920:Bradykinin / Q000235:genetics*; D050718:Complement C1 Inhibitor Protein / Q000235:genetics; D051056:Complement Inactivating Agents / Q000378:metabolism; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005819:Genetic Markers / Q000235:genetics; D006801:Humans; D011379:Prognosis; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000378:metabolism*
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000933:Antifibrinolytic Agents; D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents; D004965:Estrogen Antagonists; D005819:Genetic Markers; D014665:Vasodilator Agents; D001920:Bradykinin