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2015 ; 180
(3
): 475-83
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C1 inhibitor deficiency: 2014 United Kingdom consensus document
#MMPMID25605519
Longhurst HJ
; Tarzi MD
; Ashworth F
; Bethune C
; Cale C
; Dempster J
; Gompels M
; Jolles S
; Seneviratne S
; Symons C
; Price A
; Edgar D
Clin Exp Immunol
2015[Jun]; 180
(3
): 475-83
PMID25605519
show ga
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.