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REVIEW

A Review of Randomized Controlled Trials of Hereditary Angioedema Long-Term Prophylaxis with C1 Inhibitor Replacement Therapy: Alleviation of Disease Symptoms Is Achievable

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Pages 269-277 | Received 05 Nov 2022, Accepted 01 Feb 2023, Published online: 09 Mar 2023

Figures & data

Figure 1 Production of bradykinin through kallikrein-dependent reactions. Reprinted from J Allergy Clin Immunol, Vol 126, no 5, Kaplan AP, Enzymatic pathways in the pathogenesis of hereditary angioedema: the role of C1 inhibitor therapy, pp918-925, Copyright 2023, with permission from Elsevier.Citation17

Abbreviation: C1-INH, C1 esterase inhibitor.
Figure 1 Production of bradykinin through kallikrein-dependent reactions. Reprinted from J Allergy Clin Immunol, Vol 126, no 5, Kaplan AP, Enzymatic pathways in the pathogenesis of hereditary angioedema: the role of C1 inhibitor therapy, pp918-925, Copyright 2023, with permission from Elsevier.Citation17

Table 1 Study Design Elements in Randomized Controlled Trials of Long-Term Prophylaxis with C1-INH Replacement Therapies in Adult Patients with HAE

Table 2 Primary Efficacy and Safety Outcomes in Randomized Controlled Trials of Long-Term HAE Prophylaxis with C1-INH Replacement Therapy

Table 3 Additional Efficacy Endpoints in Randomized Controlled Trials of Long-Term HAE Prophylaxis with C1-INH Replacement Therapy

Figure 2 Mean percentage reductions in acute hereditary angioedema attack rates with C1-INH replacement therapies, by dose.

Notes: *Based on HAE attack severity (severity scores: 1 [mild], 2 [moderate], 3 [severe]) during placebo treatment period in patients ≥12 years of age.Citation18,Citation21†Average HAE attack severity during 3-month period before screening for patients ≥6 and ≤12 years of age was moderate (75% [n = 9]) or severe (25% [n = 3]).Citation25 Data from Longhurst H et al. N Engl J Med. 2017:376(12):1131–40Citation18 and Aygören-Pürsün E, at al. Pediatr Allergy Immunol. 2019:30(5):553–561,Citation25 with additional study data.
Abbreviations: C1-INH, C1 esterase inhibitor; HAE, hereditary angioedema, IV, intravenous; pdC1-INH, plasma-derived C1-INH; rhC1-INH, recombinant human C1-INH; SC, subcutaneous.
Figure 2 Mean percentage reductions in acute hereditary angioedema attack rates with C1-INH replacement therapies, by dose.