High attack frequency in patients with angioedema due to C1-inhibitor deficiency is a major determinant in switching to home therapy: a real-life observational study.
BACKGROUND
Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent attacks of swelling that affect various body sites. Such attacks are a frequent cause of visits to the emergency department and are often treated in the hospital. In recent years, self-administration of C1-inhibitor (C1-INH) concentrates at home has become an increasingly used option, with a positive impact on patient outcomes and quality of life.


METHODS
This was an observational study of 6 months' duration in 56 patients with C1-INH-HAE referred to a HAE center in southern Italy. The patients received three types of treatment for their swelling attacks: C1-INH concentrates administered at home (n = 25); icatibant administered at home (n = 12); and C1-INH concentrates administered in the hospital (n = 19). The objectives of this observational study were to compare therapy compliance (defined as the proportion of treated attacks) and quality of life in home- and hospital-treated patients, and to identify factors associated with the decision to use home therapy.


RESULTS
Overall, 918 attacks were reported over 6 months, of which 544 (59.2 %) were treated. Total number of reported attacks and the mean (±SD) number of attacks per patient, respectively, in the three groups were: 611 and 24.4 (±26.1) for home-based C1-INH; 191 and 15.9 (±12.0) for home-based icatibant; 166 and 6.1 (±6.5) for hospital-based C1-INH. Differences in attack frequency between home- and hospital-based treatments were statistically significant (p = 0.002), while patient demographic characteristics and the disease severity score did not correlate with the use of home therapy. Compliance with therapy was significantly better with home-based therapy (71.2 % of treated attacks with C1-INH and 44.0 % with icatibant) than with hospital-based therapy (21.6 %, p = 0.003). Quality of life showed an opposite trend, with patients on hospital-based treatment reporting the highest quality of life.


CONCLUSIONS
Home-based therapy was associated with better compliance compared with hospital-based therapy. The choice to adopt home-based therapy appeared to correlate with a high attack frequency. Home-based therapy is a valid treatment option for patients with C1-INH-HAE and should be offered to all such patients, and especially to those with high attack frequency.
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
Veronica
V
Squeglia
Department of Public Health, Università degli Studi di Napoli Federico II, Naples, Italy.
Alessandro
A
Barbarino
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy. bovamaria@virgilio.it.
Maria
M
Bova
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
Carmela
C
Gravante
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
Angelica
A
Petraroli
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
Giuseppe
G
Spadaro
Department of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Massimo
M
Triggiani
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
Arturo
A
Genovese
Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy. marone@unina.it.
Gianni
G
Marone
Attack frequency; C1-inhibitor concentrates; C1-inhibitor deficiency; Hereditary angioedema; Home therapy; Icatibant; Self-administration
Int Arch Allergy Immunol. 2013;161 Suppl 1:17-20
23689240
J Allergy Clin Immunol Pract. 2015 Mar-Apr;3(2):213-9
25609333
J Infus Nurs. 2014 Jan-Feb;37(1):29-34
24384882
Eur J Dermatol. 2009 Mar-Apr;19(2):147-51
19264579
Allergy Asthma Proc. 2016 Jan-Feb;37(1):72-5
26831850
Allergy. 2012 Oct;67(10):1289-98
22913638
Int Arch Allergy Immunol. 2013;161 Suppl 1:10-6
23689239
Allergy Asthma Proc. 2011 May-Jun;32(3):248-54
21549036
Lancet. 2012 Feb 4;379(9814):474-81
22305226
J Allergy Clin Immunol. 2006 Apr;117(4):904-8
16630950
Health Qual Life Outcomes. 2015 Jul 01;13:92
26129930
Acta Derm Venereol. 2014 Jul;94(4):436-41
24202369
J Allergy Clin Immunol Pract. 2013 Sep-Oct;1(5):458-67
24565617
Int Arch Allergy Immunol. 2013;161 Suppl 1:3-9
23689238
Allergy. 2011 Nov;66(11):1397-406
21859431
Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):21
20667124
Allergy Asthma Proc. 2014 Jan-Feb;35(1):47-53
24268449
Health Qual Life Outcomes. 2012 Jul 20;10:82
22817696
Allergy Asthma Proc. 2014 May-Jun;35(3):250-4
24801468
Int Arch Allergy Immunol. 2012;157(4):417-24
22123229
Int Arch Allergy Immunol. 2015;167(1):21-8
26112099
Pharmacoeconomics. 2001;19(12):1185-97
11772154
Transfusion. 2012 Jan;52(1):100-7
21756262
Acta Derm Venereol. 2015 Jul;95(6):706-10
25394853
Ann Allergy Asthma Immunol. 2015 Aug;115(2):120-5
26118352
Allergy. 2014 May;69(5):602-16
24673465
PLoS One. 2013;8(2):e53773
23390491
Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):22
20667125
J Allergy Clin Immunol Pract. 2015 Mar-Apr;3(2):220-7
25609328
Int Arch Allergy Immunol. 2015;166(4):259-66
25924832
Allergy. 2011 Jan;66(1):76-84
20804470
Allergy. 2014 Mar;69(3):305-14
24438203
Acta Derm Venereol. 2015 Feb;95(2):225-6
24603953
World Allergy Organ J. 2012 Dec;5(12):182-99
23282420
Allergy Asthma Proc. 2015 Mar-Apr;36(2):92-9
25551795
Allergy Asthma Proc. 2010 Sep-Oct;31(5):407-14
20929608
Allergy Asthma Proc. 2014 Mar-Apr;35(2):185-90
24411585
Intern Emerg Med. 2014 Feb;9(1):85-92
24002787