Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain
Abstract Background The Icatibant Outcome Survey (IOS) is an international registry monitoring the use of icatibant, a bradykinin B2 receptor antagonist indicated for the acute treatment of hereditary angioedema (HAE) attacks. Our goal was to assess disease characteristics and icatibant treatment ou...
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| Format: | Article |
| Language: | English |
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BMC
2021-12-01
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| Series: | Allergy, Asthma & Clinical Immunology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13223-021-00641-3 |
| _version_ | 1819173880343822336 |
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| author | Mar Guilarte Anna Sala-Cunill María Luisa Baeza Rosario Cabañas María Dolores Hernández Ethel Ibañez Carlos Hernando de Larramendi Ramon Lleonart Teófilo Lobera Luis Marqués Blanca Sáenz de San Pedro Jaco Botha Irmgard Andresen Teresa Caballero for the IOS Study Group |
| author_facet | Mar Guilarte Anna Sala-Cunill María Luisa Baeza Rosario Cabañas María Dolores Hernández Ethel Ibañez Carlos Hernando de Larramendi Ramon Lleonart Teófilo Lobera Luis Marqués Blanca Sáenz de San Pedro Jaco Botha Irmgard Andresen Teresa Caballero for the IOS Study Group |
| author_sort | Mar Guilarte |
| collection | DOAJ |
| description | Abstract Background The Icatibant Outcome Survey (IOS) is an international registry monitoring the use of icatibant, a bradykinin B2 receptor antagonist indicated for the acute treatment of hereditary angioedema (HAE) attacks. Our goal was to assess disease characteristics and icatibant treatment outcomes in patients with HAE due to C1 inhibitor deficiency (HAE type 1 or 2 (HAE-1/2)) from Spain relative to other countries participating in IOS. Methods Descriptive retrospective analyses of data are reported from 10 centers in Spain vs 51 centers in 12 other participating countries (July 2009 to January 2019). Results No meaningful differences were identified between patients in Spain (n = 119) and patients across other countries (n = 907) regarding median age at symptom onset (15.0 vs 12.0 years) or diagnosis (22.3 vs 20.5 years). Overall HAE attack rates (total attacks/total years of follow-up) were 2.66 in Spain and 1.46 across other countries. Patients in Spain reported fewer severe/very severe HAE attacks before treatment (41.0% vs 45.9%; P < 0.0001) and, for icatibant-treated attacks, longer median time to treatment (2.9 vs 1.0 h), time to attack resolution (18.0 vs 5.5 h), and total attack duration (24.6 vs 8.0 h). Use of androgens for long-term prophylaxis was higher in Spain (51.2% vs 26.7%). Conclusion Patients with HAE-1/2 in Spain reported fewer severe/very severe attacks, administered icatibant later, and had longer-lasting attacks than did patients across other countries in IOS. These differences may indicate varying disease management practices (e.g., delayed icatibant treatment) and reporting. Efforts to raise awareness on the benefits of early on-demand treatment may be warranted. Trial registration: NCT01034969. |
| first_indexed | 2024-12-22T20:30:06Z |
| format | Article |
| id | doaj.art-90ff5c111ba24797ac5c9aba507fb7be |
| institution | Directory Open Access Journal |
| issn | 1710-1492 |
| language | English |
| last_indexed | 2024-12-22T20:30:06Z |
| publishDate | 2021-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Allergy, Asthma & Clinical Immunology |
| spelling | doaj.art-90ff5c111ba24797ac5c9aba507fb7be2022-12-21T18:13:37ZengBMCAllergy, Asthma & Clinical Immunology1710-14922021-12-0117111110.1186/s13223-021-00641-3Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in SpainMar Guilarte0Anna Sala-Cunill1María Luisa Baeza2Rosario Cabañas3María Dolores Hernández4Ethel Ibañez5Carlos Hernando de Larramendi6Ramon Lleonart7Teófilo Lobera8Luis Marqués9Blanca Sáenz de San Pedro10Jaco Botha11Irmgard Andresen12Teresa Caballero13for the IOS Study GroupAllergy Section, Medicine Department, Hospital Universitari Vall d’HebronAllergy Section, Medicine Department, Hospital Universitari Vall d’HebronAllergy Department, Hospital General Universitario Gregorio MarañónAllergy Department, Hospital Universitario La Paz, Hospital La Paz Institute for Health Research (IdiPaz)Allergy Department, IIS-Hospital Universitari I Politècnic La FeAllergy Department, IIS-Hospital Universitari I Politècnic La FeAllergy Section, Hospital Marina Baixa, Villajoyosa and Centro de Especialidades FoietesAllergy Unit, Medicine Department, IDIBELL, Hospital Universitari de Bellvitge, L’Hospitalet de LlobregatAllergy Department, Hospital San PedroAllergy Section, IRBLleida, Hospital Universitari Santa Maria and Hospital Universitari Arnau de VilanovaAllergy Section, Hospital Universitario de JaénTakeda Pharmaceuticals International AGTakeda Pharmaceuticals International AGAllergy Department, Hospital Universitario La Paz, Hospital La Paz Institute for Health Research (IdiPaz)Abstract Background The Icatibant Outcome Survey (IOS) is an international registry monitoring the use of icatibant, a bradykinin B2 receptor antagonist indicated for the acute treatment of hereditary angioedema (HAE) attacks. Our goal was to assess disease characteristics and icatibant treatment outcomes in patients with HAE due to C1 inhibitor deficiency (HAE type 1 or 2 (HAE-1/2)) from Spain relative to other countries participating in IOS. Methods Descriptive retrospective analyses of data are reported from 10 centers in Spain vs 51 centers in 12 other participating countries (July 2009 to January 2019). Results No meaningful differences were identified between patients in Spain (n = 119) and patients across other countries (n = 907) regarding median age at symptom onset (15.0 vs 12.0 years) or diagnosis (22.3 vs 20.5 years). Overall HAE attack rates (total attacks/total years of follow-up) were 2.66 in Spain and 1.46 across other countries. Patients in Spain reported fewer severe/very severe HAE attacks before treatment (41.0% vs 45.9%; P < 0.0001) and, for icatibant-treated attacks, longer median time to treatment (2.9 vs 1.0 h), time to attack resolution (18.0 vs 5.5 h), and total attack duration (24.6 vs 8.0 h). Use of androgens for long-term prophylaxis was higher in Spain (51.2% vs 26.7%). Conclusion Patients with HAE-1/2 in Spain reported fewer severe/very severe attacks, administered icatibant later, and had longer-lasting attacks than did patients across other countries in IOS. These differences may indicate varying disease management practices (e.g., delayed icatibant treatment) and reporting. Efforts to raise awareness on the benefits of early on-demand treatment may be warranted. Trial registration: NCT01034969.https://doi.org/10.1186/s13223-021-00641-3Hereditary angioedemaRegistriesSpainBradykininBradykinin B2 receptor antagonistsIcatibant |
| spellingShingle | Mar Guilarte Anna Sala-Cunill María Luisa Baeza Rosario Cabañas María Dolores Hernández Ethel Ibañez Carlos Hernando de Larramendi Ramon Lleonart Teófilo Lobera Luis Marqués Blanca Sáenz de San Pedro Jaco Botha Irmgard Andresen Teresa Caballero for the IOS Study Group Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain Allergy, Asthma & Clinical Immunology Hereditary angioedema Registries Spain Bradykinin Bradykinin B2 receptor antagonists Icatibant |
| title | Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain |
| title_full | Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain |
| title_fullStr | Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain |
| title_full_unstemmed | Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain |
| title_short | Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain |
| title_sort | hereditary angioedema due to c1 inhibitor deficiency real world experience from the icatibant outcome survey in spain |
| topic | Hereditary angioedema Registries Spain Bradykinin Bradykinin B2 receptor antagonists Icatibant |
| url | https://doi.org/10.1186/s13223-021-00641-3 |
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