title: Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema.
abstract: The diagnosis of hereditary angioedema (HAE) is based on complement tests. We studied for the first time the possible association between complement parameters measured at the time of diagnosis and disease severity in 115 patients with HAE. Serum levels of functional C1-inhibitor (C1-INH(f)), antigenic C1-inhibitor (C1-INH(a)), C4 and hemolytic activity of the classical pathway (CH50) were determined at the time of diagnosis. We found a significant correlation between severity scores and baseline C1-INH(f) levels, as determined by ELISA assay (p=0.0003). On the other hand, there was no correlation between severity scores and other complement parameters (C1-INH(a), C4, and CH50). We consider the correlation between severity scores and baseline C1-INH(f) levels an important pathophysiological observation. Our findings underlie the potential significance of monitoring functional C1-INH levels in relation to clinical disease course.
authors: Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
authors: Zsuzsanna
authors: Z
authors: Kelemen
authors: Dumitru
authors: D
authors: Moldovan
authors: Eniko
authors: E
authors: Mihály
authors: Beáta
authors: B
authors: Visy
authors: Gábor
authors: G
authors: Széplaki
authors: Dorottya
authors: D
authors: Csuka
authors: George
authors: G
authors: Füst
authors: Henriette
authors: H
authors: Farkas
authors: Lilian
authors: L
authors: Varga
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003172:Complement C1 / Q000276:immunology; D050718:Complement C1 Inhibitor Protein / Q000032:analysis / Q000276:immunology*; D003181:Complement C4 / Q000276:immunology; D015941:Complement Hemolytic Activity Assay; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D055815:Young Adult
chemical_list: D003172:Complement C1; D050718:Complement C1 Inhibitor Protein; D003181:Complement C4