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Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema
  1. Sorena Kiani-Alikhan1,2,
  2. Elizabeth Walker1,
  3. Alaco Hickey1,
  4. Sofia Grigoriadou1,
  5. Matt Buckland1,
  6. Chris Scott1
  1. 1Clinical Immunology, Barts Health NHS Trust, London, UK
  2. 2Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK
  1. Correspondence to Dr Sorena Kiani-Alikhan, Clinical Immunology, Barts Health NHS Trust, London E1 1RD, UK; skiani{at}nhs.net

Abstract

The World Allergy Organisiation/European Academy of Allergy and Clinical Immunology (WAO/EAACI) 2017/2018 guidelines recommend measuring complement4 levels, followed by C1-inhibitor level and function for diagnosis of hereditary angioedema (HAE). We analysed 6 months’ worth of data generated in our laboratory which is a specialist regional immunology service and also provides laboratory service for the Barts Health immunology department, which is a GA2LEN/HAEi-Angioedema Centre of Excellence and Reference (ACARE) and hence, investigates a large number of patients for HAE. We found that an efficient and sensitive approach for laboratory diagnosis of HAE is to only test the C1-inhibitor function. This approach had a sensitivity of 100% and reduced the cost of laboratory investigations for HAE diagnosis by 45%.

  • complement system proteins
  • medical laboratory science
  • skin
  • immune system diseases
  • diagnosis

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Footnotes

  • Handling editor Stephen R A Jolles.

  • Contributors SK-A wrote the article. EW and AH performed the assays. The idea originate from SG and MB. CS pulled and analysed the data.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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