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C1 inhibitor deficiency: 2014 United Kingdom consensus document

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posted on 2023-06-08, 20:23 authored by H J Longhurst, Michael TarziMichael Tarzi, F Ashworth, C Bethune, C Cale, J Dempster, M Gompels, S Jolles, S Seneviratne, C Symons, A Price, D Edgar
C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organisation. This article is protected by copyright. All rights reserved.

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Publication status

  • Published

File Version

  • Published version

Journal

Clinical and Experimental Immunology

ISSN

0009-9104

Publisher

Wiley

Issue

3

Volume

180

Page range

475-483

Department affiliated with

  • BSMS Publications

Full text available

  • Yes

Peer reviewed?

  • Yes

Legacy Posted Date

2015-09-04

First Open Access (FOA) Date

2015-09-04

First Compliant Deposit (FCD) Date

2015-09-04

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