Format

Send to

Choose Destination
Clin Transl Allergy. 2019 Jul 19;9:37. doi: 10.1186/s13601-019-0272-9. eCollection 2019.

Elderly versus younger patients with hereditary angioedema type I/II: patient characteristics and safety analysis from the Icatibant Outcome Survey.

Author information

1
1Department of Dermatology and Allergy Centre, Odense University Hospital, J.B. Winsløws Vej 4, Indgang 142, 5000 Odense C, Denmark.
2
2Department of Clinical Research, University of Southern Denmark, Odense, Denmark.
3
3Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.
4
4Faculdade de Medicina ABC, Santo Andre, SP Brazil.
5
5Department of Clinical Biochemistry and Immunology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
6
6National Reference Centre for Angioedema, Internal Medicine Department, Grenoble University Hospital, Grenoble, France.
7
7Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
8
8Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, ASST Fatebenefratelli Sacco, Milan, Italy.
9
9Shire, a Takeda company, Zug, Switzerland.
10
10Department of Dermatology and Allergy, Dermatological Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Abstract

Background:

Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is characterized by recurrent swelling in subcutaneous or submucosal tissues. Symptoms often begin by age 5-11 years and worsen during puberty, but attacks can occur at any age and recur throughout life. Disease course in elderly patients is rarely reported.

Methods:

The Icatibant Outcome Survey (IOS) is an observational study evaluating the safety, tolerability, and efficacy of icatibant. We conducted descriptive analyses in younger (age < 65 years) versus elderly patients (age ≥ 65 years). Here, we report patient characteristics and safety-related findings.

Results:

As of February 2018, 872 patients with C1-INH-HAE type I/II were enrolled, of whom 100 (11.5%) were ≥ 65 years old. Significant differences between elderly versus younger patients, respectively, were noted for median age at symptom onset (17.0 vs 12.0 years), age at diagnosis (41.0 vs 19.4 years), and delay between symptom onset and diagnosis (23.9 vs 4.8 years) (P ≤ 0.0001 for all). Median age at diagnosis was significantly higher in elderly patients regardless of family history (P < 0.0001). Throughout the study, icatibant was used to treat 6798 attacks in 574 patients, with 63 elderly patients reporting 715 (10.5%) of the icatibant-treated attacks. No serious adverse events (SAEs) in elderly patients were judged to be possibly related to icatibant, whereas two younger patients reported three possibly related SAEs. Excluding off-label use and pregnancy (evaluated for regulatory purposes), the percentage of patients with at least one possibly/probably related AE was similar for elderly (2.0%) versus younger patients (2.7%). No deaths linked to icatibant treatment were identified. All related events in elderly patients were attributed to general disorders/administration site conditions, whereas related events in younger patients occurred across various system organ class designations.

Conclusions:

Elderly patients with C1-INH-HAE were significantly older at diagnosis and had greater delay in diagnosis than younger patients. Elderly patients contributed to approximately 10% of the icatibant-treated attacks. Our analysis found similar AE rates (overall and possibly/probably related) in icatibant-treated elderly versus younger patients, despite the fact that elderly patients had significantly more comorbidities and were receiving a greater number of concomitant medications. Our analysis did not identify any new or unexpected safety concerns.

KEYWORDS:

Elderly; Hereditary angioedema; Icatibant Outcome Survey; Safety

Conflict of interest statement

Competing interestsAB has received research grant support and/or speaker/consulting fees from CSL Behring and Shire*, and participated in a clinical trial for BioCryst and Jerini AG. She is an advisor for the HAE Scandinavian Patient Organization. TC has received speaker fees from CSL Behring, Novartis, and Shire,* consulting fees from BioCryst, CSL Behring, Novartis, and Shire,* funding for travel/meeting attendance from CSL Behring, Novartis, and Shire,* and has participated in clinical trials/registries for BioCryst, CSL Behring, Novartis, Pharming, and Shire.* She is a researcher from the IdiPAZ program for promoting research activities. ASG has been a speaker/consultant for BioCryst, CSL Behring, and Shire.* HJL has received research grant support and/or speaker/consulting fees from Adverum, BioCryst, CSL Behring, Shire,* and Sobi. LB has received honoraria from BioCryst, CSL Behring, Novartis, Pharming, and Shire* and her institute has received research funding from CSL Behring, GlaxoSmithKline, Novartis, Roche, and Shire.* WA has acted as a medical advisor and speaker for BioCryst, CSL Behring, Pharming, and Shire* and has received funding to attend conferences/educational events, and donations to his departmental fund from and participated in clinical trials for Shire.* AZ has received speaker fees from CSL Behring, Shire,* and Sobi; consulting fees from CSL Behring and Shire* and has acted on the medical/advisory boards for CSL Behring and Shire.* JB and IA are full-time employees of Shire,* Zug, Switzerland. MM has received research grant support and/or speaker/consulting fees from BioCryst, CSL Behring, and Shire*. *A Takeda company.

Supplemental Content

Full text links

Icon for BioMed Central Icon for PubMed Central
Loading ...
Support Center