J ALLERGY CLIN IMMUNOL

FEBRUARY 2014

AB34 AbstractsSATURDAY

123 Health-Related Quality Of Life (HRQoL) In Adult Patients WithHereditary Angioedema Due To C1 Inhibitor Deficiency (HAE-C1-INH) Assessed By SF-36v2

Dr. Teresa Caballero, MD, PhD1,2, Dr. Magdalena Julia Caminoa, MD1,

Elia P�erez-Fern�andez, MSc3, Dr. Carmen G�omez-Traseira, MD1,

Dr. Anne Aabom, MD4, Prof. Werner Aberer, MD5, Dr. Stephen D.

Betschel, MD6, Prof. Anette Bygum, MD4, Dr. Dorottya Csuka, PhD7, Hen-

riette Farkas, MD, PhD, DSc7, Dr. Maria Gomide, MD8, Dr. Adriane

Groffik, MD9, Dr. Anete S. Grumach8, Mrs. Iris Leivobich10,

Dr. Alejandro Malbran, MD11, Dr. Eniko Mihaly, MD12, Dr. Dumitru

Moldovan, MD, PhD13, Dr. Krystyna Obtulowicz, MD14, Dr. Gregor

Porebski, MD15, Mrs. Celina Rayonne16, Dr. Avner Reshef, MD10,

Dr. Petra Staubach, MD9, Dr. Michaela Wiednig, MD5, Maria Joao

Forjaz17,18, Dr. Nieves Prior, MD19; 1Allergy Department, Hospital La

Paz Institute for Health Research (IdiPaz), Madrid, Spain, 2Biomedical

Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain,3Hospital Universitario Fundaci�on Alcorc�on, Madrid, Spain, 4Denmark

HAE Centre, Department of Dermatology and Allergy Centre, Odense Uni-

versity Hospital, Odense, Denmark, 5Department of Dermatology and Vene-

reology, Medical University of Graz, Graz, Austria, 6University of Toronto,

Division of Clinical Immunology and Allergy, Toronto, ON, Canada, 7Hun-

garian HAE Center, 3rd Department of InternalMedicine, Semmelweis Uni-

versity, Budapest, Hungary, 8Department of Dermatology, School of

Medicine,University of SaoPaulo, SaoPaulo,Brazil, 9Department ofDerma-

tology, University Medical Center, University of Mainz, Mainz, Germany,10Allergy, Clinical Immunology &Angioedema Unit, Chaim ShebaMedical

Center, Tel Hashomer, Israel, 11British Hospital of Buenos Aires, Buenos

Aires, Argentina, 12Department of Allergology–Immunology, Mures County

Hospital, T�ırgu-Mures, Romania, 13Department of Allergology–Immu-

nology, Mures County Hospital, Tirgu-Mures, Romania, Tirgu-Mures,

Romania, 14Department of Clinical and EnviromentalMedicine, Jagiellonian

University, Krakow, Poland, 15Department of Clinical and Environmental

Allergology, Jagiellonian University, Krakow, Poland, 16ReSolve Research

Solutions Inc., Whitby, Ontario, Canada, 17National School of Public Health,

Carlos III Institute of Public Health, Madrid, Spain, 18REDISSEC, Madrid,

Spain, 19Hospital Universitario Severo Ochoa, Madrid, Spain.

RATIONALE: HAE-C1-INH is a rare disease with high morbidity and

life-threatening attacks, and a paucity of HRQoL studies.

METHODS: A prospective international multicenter cohort study was

performed in 11 countries. The SF-36 v2 generic HRQoL survey was self-

administered to adult patients with HAE-C1-INH as part of the pilot study

for the validation of IHAE-QoL. The recall period was 4 weeks. Subscales

scores (PF, SF, RP, RE, MH, VT, BP, GH), Physical component summary

(PCS) and Mental component Summary (MCS) were calculated. No data

were imputed. For each of the subscales and the component summary

scores, higher values indicate better HRQoL.

RESULTS: 290 patients were included with the following distribution:

Argentine 16; Austria 18; Brazil 34; Canada 21; Denmark 27; Germany 42;

Hungary 38; Israel 9; Poland 22; Romania 19; Spain 44. Surveys were

complete enough to compute PCS and MCS scales scores in 278 and 284

patients, respectively. PCS mean was 49.7 (country range: 42.5-50.4), MCS

meanwas 46.2 (country range: 37.8-53.3). The subscale means for thewhole

sample were: PF 82.5; SF 73.6; RP 72.8; RE 77.8; MH 65.8; VT 55.6; BP

59.7;GH53.5. Sixty-sixpatients referred theywere somewhatworse ormuch

worse than a year ago. PF, RP, BP, VT and PCS scores were significantly

lower in females. PF, RP, RE, MH, VT, BP, GH, PCS and MCS scores were

significantly higher in patients with higher socioeconomic level.

CONCLUSIONS: Therewere important differences in HRQoL by country.

HRQoLwasworse in females and in patientswith lower socioeconomic level.

124 The Icatibant Outcome Survey: Characteristics Of PatientsWith Hereditary Angioedema Requiring Reinjection

Dr. Hilary Longhurst, MD1, Prof. Marcus Maurer, MD2, Dr. Vincent

Fabien, PhD3, Prof. Werner Aberer, MD4, Prof. Laurence Bouillet, MD,

PhD5, Dr. Andrea Zanichelli, MD6, Dr. Teresa Caballero, MD, PhD7;

1Barts Health NHS Trust, London, United Kingdom, 2Department of

Dermatology and Allergy, Charit�e – Universit€atsmedizin, Berlin, Ger-

many, 3Shire, Eysins, Switzerland, 4Department of Dermatology and Ve-

nereology, Medical University of Graz, Graz, Austria, 5National

Reference Centre for Angioedema, Internal Medicine Department, Greno-

ble University Hospital, Grenoble, France, 6Dipartimento di Scienze Bio-

mediche e Cliniche, Ospedale Luigi Sacco, Universit�a degli Studi di

Milano, Milan, Italy, 7Allergy Department, Hospital La Paz Institute for

Health Research (IdiPaz), Madrid, Spain.

RATIONALE: Phase III icatibant trials showed most hereditary angioe-

dema (HAE) attackswere treated successfullywith one injection of icatibant,

a selective bradykinin B2 receptor antagonist. To date, there is a paucity of

real-world data regarding icatibant reinjection for HAE type I and II attacks.

METHODS: Descriptive retrospective analyses of icatibant reinjection

were performed on Icatibant Outcome Survey (IOS; Shire, Eysins,

Switzerland [NCT01034969]) data (July2009-December2012). IOS is an in-

ternational observational studymonitoring icatibant safety and effectiveness.

RESULTS: Icatibant was administered for 507 non-laryngeal attacks in

155 patients with HAE type I or II. Patients self-administered icatibant in

69.2% of attacks; a single injection was used for 93.3% of attacks. For

attacks requiring reinjection, the second injection was given a median of

12.6 hours after the first, with 95.7% of second injections administered >_6

hours after the first. Symptoms resolved a median of 4.3 hours after second

injection. Logistic regression analysis showed no relationship between

reinjection requirement and sex, weight, family history, administrator type,

attack severity, attack frequency (<20 versus >_20 attacks/year), attack

location or long-term prophylaxis usage (all p>0.05). One injection was

used in: 95.1% (n5195/205) of attacks involving only skin; 90.3%

(n5215/238) of attacks involving only abdomen; 92.7% (n5290/313) of

severe/very severe attacks; 91.2% (n5312/342) and 96.8% (n5120/124)

of attacks in patients with <20 and >_20 attacks/year, respectively.

CONCLUSIONS: In this real-world setting, most HAE attacks resolved

with one icatibant injection. There was no distinct profile for patients or

attacks requiring reinjection.