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.