The Neglected Older Adults in Hereditary Angioedema: Insights From the ITACA Registry.
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Perego Francesca, Marcelli Azzurra Cesoni, Senter Riccardo, Zingale Lorenza Chiara, Gidaro Antonio, Janu Valentina Popescu, Arcoleo Francesco, Accardo Pietro Andrea, Lo Pizzo Mariangela, Zanichelli Andrea, Giardino Francesco, Caruso Andrea, Longhitano Simone Giosuè, Cataudella Edoardo, Vultaggio Alessandra, Matucci Andrea, Petraroli Angelica, Gatti Roberta, Spadaro Giuseppe, Brussino Luisa, Nicola Stefania, Lo Sardo Luca, Guarino Maria Domenica, Ruin Federica, Ranucci Luca, Bignardi Donatella, Giliberti Marica, Borrelli Paolo, Triggianese Paola, Triggiani Massimo, Colangelo Caterina, Minciullo Paola Lucia, De Pasquale Tiziana Maria Angela, Cogliati Chiara Beatrice, Angeli Giada De, Firinu Davide, Montinaro Vincenzo, Cancian Mauro
Аннотация
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.