First case of acquired functional C1(-) INH deficiency: association with angioedema during Churg and Strauss vasculitis.
This report deals with the first case of acquired functional C1(-) INH deficiency with normal antigenic C1(-) INH level which was detected in a young girl with angioedema and Churg and Strauss vasculitis. This complement abnormality was associated with slightly depressed levels of CH50, C4 and C2, but a normal level of C3, and high levels of total IgE and IgM rheumatoid factors. Finally, most of these abnormalities disappeared after corticosteroid therapy and clinical improvement.
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000799:Angioedema / Q000150:complications*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003176:Complement C3 / Q000032:analysis; D005260:Female; D006801:Humans; D014581:Urticaria / Q000150:complications; D014657:Vasculitis / Q000150:complications*
D000305:Adrenal Cortex Hormones; D003174:Complement C1 Inactivator Proteins; D003176:Complement C3