Parameters of the classical complement pathway predict disease severity in hereditary angioedema

Dorottya Csuka, G. Füst, H. Farkas, L. Varga

Research output: Contribution to journalArticle

14 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)85-93
Number of pages9
JournalClinical Immunology
Volume139
Issue number1
DOIs
Publication statusPublished - Apr 2011

Fingerprint

Hereditary Angioedemas
Classical Complement Pathway
Danazol
Angioedema
Complement Activation
Population
Therapeutics

Keywords

  • C1-inhibitor deficiency
  • C1rC1sC1-inh
  • C3bBbP
  • Disease marker
  • Hereditary angioedema
  • SC5b-9

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Medicine(all)

Cite this

Parameters of the classical complement pathway predict disease severity in hereditary angioedema. / Csuka, Dorottya; Füst, G.; Farkas, H.; Varga, L.

In: Clinical Immunology, Vol. 139, No. 1, 04.2011, p. 85-93.

Research output: Contribution to journalArticle

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