The acquired C1-INH deficiencies with autoantibodies (AAE type II).
A new type of acquired C1-inhibitor (C1-INH) deficiency has been recognized (AAE type II) which is characterized by the presence of autoantibodies to C1-INH and by a circulating 96 KD C1-INH molecule. The clinical manifestations and biochemical abnormalities of this novel autoimmune disease resemble those found in the other forms of acquired C1-INH deficiency (AAE type I), including recurrent angioedema and low serum levels of C2, C4, C1, C1q and C1-INH activity. However, in contrast to AAE type I, AAE type II is not associated to other diseases. Evidence has been provided that the anti-C1-INH antibodies play a major role in the development and maintenance of AAE type II. These autoantibodies seem to impede C1-INH activity, thus allowing unopposed activation of the complement and/or contact system and to induce the generation of the 96 KD C1-INH species in the patients' plasma.
Alsenz
J
J
Basel Institute for Immunology, Switzerland.
Loos
M
M
D000799:Angioedema / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D000915:Antibody Affinity; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000276:immunology / Q000378:metabolism; D006801:Humans; D013329:Structure-Activity Relationship
D001323:Autoantibodies; D003174:Complement C1 Inactivator Proteins