Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema

Clinical Immunology : the Official Journal of the Clinical Immunology Society
Zsuzsanna KelemenLilian Varga

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.

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Citations

Apr 5, 2014·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·Ying-Yang XuHong-Yu Zhang
Jun 4, 2013·The Journal of Allergy and Clinical Immunology·Bruce L ZurawUNKNOWN American College of Allergy, Asthma and Immunology
Mar 16, 2012·Allergologia et immunopathologia·T Caballero
Sep 6, 2015·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Edimara S ReisJohn D Lambris
Dec 10, 2013·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Andrew J MacGinnitie
Feb 14, 2014·The Journal of Allergy and Clinical Immunology·Erika KajdácsiLászló Cervenak
Mar 29, 2014·International Immunopharmacology·Mustafa DemirtürkGünnur Deniz
Feb 9, 2011·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Dorottya CsukaLilian Varga
May 5, 2016·Clinical Reviews in Allergy & Immunology·Henriette FarkasLilian Varga
Jul 2, 2016·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·Ibolya CzallerHenriette Farkas
Mar 23, 2017·The New England Journal of Medicine·Hilary LonghurstUNKNOWN COMPACT Investigators
Feb 10, 2021·Clinical Reviews in Allergy & Immunology·Grzegorz PorebskiAvner Reshef
Aug 1, 2021·Journal of Immunological Methods·Clemens SchöfflWerner Aberer
Oct 3, 2018·International Archives of Allergy and Immunology·Nihal Mete GökmenAytül Zerrin Sin

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