title: C1 inhibitor deficiency: 2014 United Kingdom consensus document.
abstract: 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 organization.
authors.affiliation: Department of Immunology, Barts Health NHS Trust and Medical Adviser HAE, UK.
authors.forename: H J
authors.initials: HJ
authors.lastname: Longhurst
authors.affiliation: Department of Medicine, Brighton and Sussex Medical School, Brighton, UK.
authors.forename: M D
authors.initials: MD
authors.lastname: Tarzi
authors.affiliation: Department of Immunology, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK.
authors.forename: F
authors.initials: F
authors.lastname: Ashworth
authors.affiliation: Department of Immunology, Plymouth Hospitals NHS Trust, Plymouth, UK.
authors.forename: C
authors.initials: C
authors.lastname: Bethune
authors.affiliation: Department of Immunology, Great Ormond Street Hospital for Children, London, UK.
authors.forename: C
authors.initials: C
authors.lastname: Cale
authors.affiliation: Department of Immunology, Barts Health NHS Trust and Medical Adviser HAE, UK.
authors.forename: J
authors.initials: J
authors.lastname: Dempster
authors.affiliation: Department of Immunology, North Bristol NHS Trust, Bristol, UK.
authors.forename: M
authors.initials: M
authors.lastname: Gompels
authors.affiliation: Department of Immunology, University Hospital of Wales, Cardiff, UK.
authors.forename: S
authors.initials: S
authors.lastname: Jolles
authors.affiliation: Department of Immunology, Royal Free London NHS Trust, London, UK.
authors.forename: S
authors.initials: S
authors.lastname: Seneviratne
authors.affiliation: Department of Immunology, Plymouth Hospitals NHS Trust, Plymouth, UK.
authors.forename: C
authors.initials: C
authors.lastname: Symons
authors.affiliation: Herditary Angioedema, UK (HAE UK).
authors.forename: A
authors.initials: A
authors.lastname: Price
authors.affiliation: UK Primary Immunodeficiency Network (UK PIN), Newcastle upon Tyne, UK.
authors.forename: D
authors.initials: D
authors.lastname: Edgar
mesh_terms: D054179:Angioedemas, Hereditary / Q000628:therapy*; D019468:Disease Management; D006801:Humans
keywords: C1 inhibitor deficiency; HAE; guidelines; hereditary angioedema