Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema.
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.
Kelemen
Zsuzsanna
Z
Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Moldovan
Dumitru
D
Mihály
Eniko
E
Visy
Beáta
B
Széplaki
Gábor
G
Csuka
Dorottya
D
Füst
George
G
Farkas
Henriette
H
Varga
Lilian
L
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
D003172:Complement C1; D050718:Complement C1 Inhibitor Protein; D003181:Complement C4