Noémi Andrási1, Nóra Veszeli2, Ágnes Holdonner3, György Temesszentandrási4, Kinga Viktória Kőhalmi5, Lilian Varga3, Henriette Farkas6. 1. Hungarian Angioedema Reference Center, 3(rd) Department of Internal Medicine, Semmelweis University, Hungary; 2(nd) Department of Pediatrics, Semmelweis University, Hungary. 2. Hungarian Angioedema Reference Center, 3(rd) Department of Internal Medicine, Semmelweis University, Hungary; MTA-SE Immunology & Hematology Research Group, Semmelweis University and Hungarian Academy of Sciences, Hungary. 3. Hungarian Angioedema Reference Center, 3(rd) Department of Internal Medicine, Semmelweis University, Hungary. 4. 3(rd) Department of Internal Medicine, Semmelweis University, Hungary. 5. Hungarian Angioedema Reference Center, 3(rd) Department of Internal Medicine, Semmelweis University, Hungary; Hospital of Hospitaller Brothers of St. John of God, Department of Rheumatology, Hungary. 6. Hungarian Angioedema Reference Center, 3(rd) Department of Internal Medicine, Semmelweis University, Hungary. Electronic address: farkas.henriette@med.semmelweis-univ.hu.
Abstract
OBJECTIVE: Conestat alpha, a C1-inhibitor produced by recombinant technology (rhC1-INH) is an acute treatment for edematous attacks occurring in hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE). Our study evaluated the efficacy and safety of rhC1-INH administered during HAE attacks, and for short-term prophylaxis (STP). MATERIALS & METHOD: Our prospective study analyzed the course of 544 HAE attacks experienced by the 21 C1-INH-HAE patients treated, as well as the outcome of 97 instances of STP implemented with rhC1-INH. Using a purpose-designed questionnaire, the patients recorded relevant, treatment-related information. RESULTS: Time to the administration of rhC1-INH was 90.0 min (median) after the onset of HAE attacks. The symptoms started to improve as early as 60 min after the injection of rhC1-INH, and the attack resolved 730.0 min after treatment. The interval between the onset of the HAE attack and the administration of rhC1-INH correlated with time until the onset of improvement (R = 0.2053 p < 0.0001), and with time to the complete resolution of symptoms (R = 0.2805, p < 0.0001). Nine patients received STP with rhC1-INH in 97 instances. STP successfully prevented the HAE attack within 72 h of the event on 93/97 occasions. No local and serious systemic adverse events/effects were observed. CONCLUSIONS: Treatment with rhC1-INH is effective and safe both for acute management, and for STP. Following the onset of an HAE attack, early administration of rhC1-INH may reduce time to the improvement and to the complete resolution of symptoms. Repeated administration of rhC1-INH does not impair its efficacy.
OBJECTIVE: Conestat alpha, a C1-inhibitor produced by recombinant technology (rhC1-INH) is an acute treatment for edematous attacks occurring in hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE). Our study evaluated the efficacy and safety of rhC1-INH administered during HAE attacks, and for short-term prophylaxis (STP). MATERIALS & METHOD: Our prospective study analyzed the course of 544 HAE attacks experienced by the 21 C1-INH-HAEpatients treated, as well as the outcome of 97 instances of STP implemented with rhC1-INH. Using a purpose-designed questionnaire, the patients recorded relevant, treatment-related information. RESULTS: Time to the administration of rhC1-INH was 90.0 min (median) after the onset of HAE attacks. The symptoms started to improve as early as 60 min after the injection of rhC1-INH, and the attack resolved 730.0 min after treatment. The interval between the onset of the HAE attack and the administration of rhC1-INH correlated with time until the onset of improvement (R = 0.2053 p < 0.0001), and with time to the complete resolution of symptoms (R = 0.2805, p < 0.0001). Nine patients received STP with rhC1-INH in 97 instances. STP successfully prevented the HAE attack within 72 h of the event on 93/97 occasions. No local and serious systemic adverse events/effects were observed. CONCLUSIONS: Treatment with rhC1-INH is effective and safe both for acute management, and for STP. Following the onset of an HAE attack, early administration of rhC1-INH may reduce time to the improvement and to the complete resolution of symptoms. Repeated administration of rhC1-INH does not impair its efficacy.
Authors: Anna Valerieva; Maria T Staevska; Vesna Grivcheva-Panovska; Milos Jesenak; Kinga Viktória Kőhalmi; Katarina Hrubiskova; Andrea Zanichelli; Luca Bellizzi; Anurag Relan; Roman Hakl; Henriette Farkas Journal: World Allergy Organ J Date: 2021-04-22 Impact factor: 4.084