Lack of increased prevalence of immunoregulatory disorders in hereditary angioedema due to C1-inhibitor deficiency

H. Farkas, Dorottya Csuka, Judit Gács, Ibolya Czaller, Zsuzsanna Zotter, G. Füst, L. Varga, Péter Gergely

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Hereditary angioedema due to deficiency of C1-INH (HAE-C1-INH) is associated with enhanced consumption of the early complement components, which may predispose for autoimmune disease.We assessed the prevalence of such disorders among HAE- C1-INH patients and their impact on the natural course of HAE-C1-INH.Clinical data and immunoserological parameters of 130 HAE-C1-INH and 174 non-C1-INH-deficient patients with angioedema were analyzed.In our study, the incidence of immunoregulatory disorders was 11.5% in the population of HAE-C1-INH patients and 5.2% among non-C1-INH-deficient controls with angioedema. Immunoserology screening revealed a greater prevalence of anticardiolipin IgM (p = 0.0118) among HAE-C1-INH patients, than in those with non-C1-INH-deficient angioedema.We did not find higher prevalence of immunoregulatory disorders among our HAE-C1-INH patients. However, in patients with confirmed immunoregulatory disorders, the latter influenced both the severity of HAE-C1-INH and the effectiveness of its long-term management. Appropriate management of the immunoregulatory disease thus identified improves the symptoms of HAE-C1-INH.

Original languageEnglish
Pages (from-to)58-66
Number of pages9
JournalClinical Immunology
Volume141
Issue number1
DOIs
Publication statusPublished - Oct 2011

Fingerprint

Hereditary Angioedemas
Angioedema
Disease Management
Autoimmune Diseases
Immunoglobulin M
Incidence

Keywords

  • Autoantibodies
  • C1-inhibitor deficiency
  • Hereditary angioedema
  • Immunoregulatory disease
  • Prevalence
  • Screening

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Lack of increased prevalence of immunoregulatory disorders in hereditary angioedema due to C1-inhibitor deficiency. / Farkas, H.; Csuka, Dorottya; Gács, Judit; Czaller, Ibolya; Zotter, Zsuzsanna; Füst, G.; Varga, L.; Gergely, Péter.

In: Clinical Immunology, Vol. 141, No. 1, 10.2011, p. 58-66.

Research output: Contribution to journalArticle

Farkas, H. ; Csuka, Dorottya ; Gács, Judit ; Czaller, Ibolya ; Zotter, Zsuzsanna ; Füst, G. ; Varga, L. ; Gergely, Péter. / Lack of increased prevalence of immunoregulatory disorders in hereditary angioedema due to C1-inhibitor deficiency. In: Clinical Immunology. 2011 ; Vol. 141, No. 1. pp. 58-66.
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