Icatibant use in Brazilian patients with hereditary angioedema (HAE) type 1 or 2 and HAE with normal C1-INH levels: findings from the Icatibant Outcome Survey Registry Study

• Published in: Anais brasileiros de dermatología 2022-07, Vol.97 (4), p.448-457
• Main Authors: Grumach, Anete S., Henriques, Marina T., Bardou, Maine L.D., Pontarolli, Daniele A., Botha, Jaco, Correa, Mariangela
• Format: Article
• Language: English
• Subjects: Angioedemas, Hereditary - diagnosis; Angioedemas, Hereditary - drug therapy; Bradykinin; Bradykinin - analogs & derivatives; Bradykinin - therapeutic use; Bradykinin receptor; Brazil; Complement C1 Inhibitor Protein - chemistry; DERMATOLOGY; Hereditary angioedema; Humans; Original; Registries; Treatment Outcome
• ISSN: 0365-0596; 1806-4841; 1806-4841
• DOI: 10.1016/j.abd.2021.09.009

Hereditary angioedema can be caused by C1-Inhibitor (C1-INH) deficiency and/or dysfunction (HAE-1/2) or can occur in patients with normal C1-INH (HAE nC1-INH). The Icatibant Outcome Survey (IOS; NCT01034969) registry monitors the safety and effectiveness of icatibant for treating acute angioedema. Present findings from Brazilian patients with HAE-1/2 and HAE nC1-INH participating in IOS. 42 patients were enrolled (HAE-1/2, n = 26; HAE nC1-INH, n = 16). Median age at symptom onset was significantly lower with HAE-1/2 vs. HAE nC1-INH (10.0 vs. 16.5y, respectively; p = 0.0105), whereas median age at diagnosis (31.1 vs. 40.9y; p = 0.1276) and the median time between symptom onset and diagnosis (15.0 vs. 23.8y; p = 0.6680) were numerically lower vs. HAE nC1-INH, respectively. One icatibant dose was used for > 95% of HAE attacks. Median (range) time-to-event outcomes were shorter for patients with HAE nC1-INH vs. HAE-1/2, including time to first administration (0.5 [0–96.0] vs. 1.0 [0–94.0]h, respectively), time from first administration to complete resolution (1.0 [0–88.0] vs. 5.5 [0–96.0]h, respectively), and total attack duration (7.0 [0.3–99.0] vs. 18.5 [0.1–100.0]h, respectively). Mean (SD) time from attack onset to resolution was significantly shorter for patients with HAE nC1-INH vs. HAE-1/2 (9.8 [18.7] vs. 19.6 [24.0]h, respectively; p = 0.0174). 83 adverse events (AEs) in 42 patients were reported; most were mild (66.3%) or moderate (13.3%) and non-serious (75.9%). The most common icatibant-related AE was injection site erythema (HAE-1/2, 34.6%; HAE nC1-INH, 18.8%). This was an observational study without a treatment comparator and that relied on patient recall. Findings demonstrate effectiveness and tolerability of icatibant in Brazilian HAE patients.