The Neglected Older Adults in Hereditary Angioedema: Insights From the ITACA Registry.

Abstract

Background: Hereditary Angioedema due to C1-inhibitor deficiency (HAE-C1INH) is a rare disease that affects individuals of all ages, however, older adults have never been characterized in terms of disease severity, comorbidities, and treatments. The aim was to identify the clinical characteristics of and therapeutic approaches in HAE-C1INH patients aged 65 and older.

Methods: Data from the ITACA (Italian network for Hereditary and Acquired Angioedema) Registry were prospectively collected for 10-month.

Results: Data from 647 HAE-C1INH, patients including 343 females (53%), were collected: 114 patients (17.6%) were aged 65 and older (68 females; 58.6%). Group mean age was 74.3 ± 7.5 years, mean age at first-symptom-onset was 19 ± 15 years, and mean age at diagnosis was 45.6 ± 14.3 years. Common comorbidities were: hypertension (59.6%), dyslipidaemia (28.9%), coronary artery disease (14.0%), diabetes (14.0%), endocrinopathies (14.0%), neoplasia (12.3%) and B/C hepatitis (11.4%). Half of the older patients (49%) experienced at least one attack during the study period, and 8 patients (7%) had an attack frequency of > 0.5 attacks/month. Long-term prophylaxis (LTP) was the treatment of choice in 45 patients (39.5%) and represented 15.9% of overall LTP prescriptions: 60% were treated with lanadelumab, 22.2% with attenuated androgens, 13.3% with berotralstat, and 4.5% with sub-cutaneous C1INH concentrate. Gender differences were not detected in any of the variables analyzed.

Conclusion: Older patients with HAE-C1INH constitute a relevant subgroup, characterized by persistent disease activity and comorbidities. The availability of new therapies and guideline recommendations are driving an increase in LTP use, although shifting from older non-specific treatments, especially androgens, is still incomplete.