title: Parameters of the classical complement pathway predict disease severity in hereditary angioedema.
abstract: Functional C1-inhibitor (C1-inh) and C4 are potential severity markers of hereditary angioedema due to deficiency of C1-inh (HAE-C1-inh), and the complexes generated through complement activation may be relevant. We studied the association between disease severity and complement parameters in 105 HAE-C1-inh patients. Disease severity was characterized by the number of angioedema attacks or alternatively, by the number of C1-inh concentrate ampoules (C1-inh-amp) used for the treatment of attacks. Median C1rC1sC1-inh level was higher (32.8 U/ml vs. 3.4 U/ml; p<0.0001) in patients, compared to controls. C1rC1sC1-inh and C1-inh strongly correlated with attack number and C1-inh-amp, both in the whole patient population and in the subgroup on danazol prophylaxis. Both C1rC1sC1-inh and C1-inh are suitable for predicting disease severity based on attack frequency and C1-inh-amp (OR=4.38[1.43-13.43], p=0.010 and 11.78[2.54-54.67], p=0.002, respectively). We presume that both C1rC1sC1-inh and C1-inh might prove sensitive predictors of the severity of HAE-C1-inh.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University Budapest, Kútvölgyi Str 4, Budapest, Hungary.
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.forename: George
authors.initials: G
authors.lastname: Füst
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000188:drug therapy / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003171:Complement Pathway, Classical / Q000235:genetics* / Q000502:physiology*; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D005819:Genetic Markers; D006801:Humans; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D004965:Estrogen Antagonists; D005819:Genetic Markers; D003613:Danazol