Comparison of Chromogenic and ELISA Functional C1 Inhibitor Tests in Diagnosing Hereditary Angioedema

H. Henry Li, Paula Busse, William R. Lumry, Ashley Frazer-Abel, Howard Levy, Tamara Steele, Jeffrey Dayno, Marc Riedl

Research output: Contribution to journalArticle

Abstract

Introduction: Measuring functional C1 inhibitor (C1 INH) with chromogenic or ELISA methods can confirm a diagnosis of hereditary angioedema (HAE) due to C1 INH deficiency. Previous studies found differences in the agreement of these assays. Objective: To evaluate the agreement between chromogenic or ELISA methods in the context of an observational study. Methods: Patients with previously confirmed HAE underwent functional C1 INH testing. These patients contacted first-degree relatives (parents, siblings, or offspring) not previously evaluated for HAE, who underwent a panel of complement testing, including for functional C1 INH. Results: Overall, 31 patients with HAE and 46 untested relatives enrolled. Of 46 relatives, 14 (30.4%) were newly diagnosed with HAE based on their laboratory results. Among the 31 patients previously confirmed with HAE, all had low functional C1 INH according to the chromogenic method, whereas 22 (71.0%) had low, 7 (22.6%) had equivocal, and 2 (6.5%) had normal functional C1 INH according to the ELISA method. In the 14 newly diagnosed relatives, all had low functional C1 INH according to the chromogenic method, whereas 11 (78.5%) had low and 3 (21.4%) had equivocal results according to the ELISA method. Conclusion: Despite the apparent discordance in the ELISA and chromogenic assays in a small number of patients, both were useful in measuring functional C1 INH. To establish the diagnosis of HAE due to C1 INH deficiency, functional C1 INH results should be interpreted in combination with family and clinical history, and with other complement tests.

Original languageEnglish (US)
Pages (from-to)200-205
Number of pages6
JournalJournal of Allergy and Clinical Immunology: In Practice
Volume3
Issue number2
DOIs
StatePublished - Mar 1 2015
Externally publishedYes

Keywords

  • C1 inhibitor
  • Complement testing
  • Hereditary angioedema

ASJC Scopus subject areas

  • Immunology and Allergy

Fingerprint Dive into the research topics of 'Comparison of Chromogenic and ELISA Functional C1 Inhibitor Tests in Diagnosing Hereditary Angioedema'. Together they form a unique fingerprint.

  • Cite this