Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor

Authors: Magerl, Markus ^{a, b, ∗}, Riedl, Marc A. ^c, Arruda, Luisa Karla ^d, Bauer, Andrea ^e, Berardi, Alejandro ^f, Bernstein, Jonathan A. ^g, Bouillet, Laurence ^h, Buckland, Matthew ⁱ, Buttgereit, Thomas ^{a, b}, Cohn, Danny M. ^j, Craig, Timothy ^k, Criado, Roberta F. ^l, Du-Thanh, Aurélie ^m, Fain, Olivier ⁿ, Gonçalo, Margarida ^o, Greve, Jens ^p, Grumach, Anete Sevciovic ^q, Guilarte, Mar ^r, Katelaris, Constance ^s, Kinaciyan, Tamar ^t, Latysheva, Elena A. ^u, Lleonart, Ramon ^v, Llosa, Oscar Calderón ^w, Mansour, Eli ^x, Grivcheva-Panovska, Vesna ^y, Parisi, Claudio ^z, Rosario Filho, Nelson Augusto ^aa, Santos, Amélia Spínola ^bb, Staubach, Petra ^cc, Valerieva, Anna ^dd, Rodrigues Valle, Solange Oliveira ^ee, Danese, Sherry ^ff, Ulloa, Julie ^ff, Audhya, Paul K. ^gg, Maurer, Marcus ^{a, b}

Source: In Journal of Allergy and Clinical Immunology: Global August 2025 4(3)

Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus

Authors: Reshef, Avner, Buttgereit, Thomas, Betschel, Stephen D., Caballero, Teresa, Farkas, Henriette, Grumach, Anete S., Hide, Michihiro, Jindal, Ankur K., Longhurst, Hilary, Peter, Jonathan, Riedl, Marc A., Zhi, Yuxiang, Aberer, Werner, Abuzakouk, Mohamed, Al Farsi, Tariq, Al Sukaiti, Nashat, Al-Ahmad, Mona, Altrichter, Sabine, Aygören-Pürsün, Emel, Baeza, Maria Luisa, Bara, Noemi Anna, Bauer, Andrea, Bernstein, Jonathan A., Boccon-Gibod, Isabelle, Bonnekoh, Hanna, Bouillet, Laurence, Brzoza, Zenon, Bygum, Anette, Calderon, Oscar, de Albuquerque Campos, Regis, Campos Romero, Freya Helena, Cancian, Mauro, Chong-Neto, Herberto Jose, Christoff, George, Cimbollek, Stefan, Cohn, Danny M., Craig, Timothy, Danilycheva, Inna, Darlenski, Razvigor, Du-Thanh, Aurélie, Ensina, Luis Felipe, Fomina, Daria, Fonacier, Luz, Fukunaga, Atsushi, Gelincik, Asli, Giavina-Bianchi, Pedro, Godse, Kiran, Gompels, Mark, Goncalo, Margarida, Gotua, Maia, Guidos-Fogelbach, Guillermo, Guilarte, Mar, Kasperska-Zajac, Alicja, Katelaris, Constance H., Kinaciyan, Tamar, Kolkhir, Pavel, Kulthanan, Kanokvalai, Kurowski, Marcin, Latysheva, Elena, Lauerma, Antti, Launay, David, Lleonart, Ramon, Lumry, William, Malbran, Alejandro, Ali, Ramzy Mohammed, Nasr, Iman, Nieto-Martinez, Sandra, Parisi, Claudio, Pawankar, Ruby, Piñero-Saavedra, Macarena, Popov, Todor A., Porebski, Grzegorz, Prieto Garcia, Alicia, Pyatilova, Polina, Rudenko, Michael, Sekerel, Bulent Enis, Serpa, Faradiba Sarquis, Sheikh, Farrukh, Siebenhaar, Frank, Soria, Angèle, Staevska, Maria, Staubach, Petra, Stobiecki, Marcin, Thomsen, Simon Francis, Triggiani, Massimo, Valerieva, Anna, Valle, Solange, Van Dinh, Nguyen, Vera Ayala, Carolina Elisa, Zalewska-Janowska, Anna, Zanichelli, Andrea, Magerl, Markus, Maurer, Marcus

Source: In The Journal of Allergy and Clinical Immunology August 2024 154(2):398-411

A Core Outcome Set for Efficacy of Acute Treatment of Hereditary Angioedema

Authors: Petersen, Remy S. ^a, Fijen, Lauré M. ^a, Apfelbacher, Christian ^b, Magerl, Markus ^{c, d}, Weller, Karsten ^{c, d}, Aberer, Werner ^e, Adatia, Adil ^f, Audhya, Paul ^g, Bara, Noémi-Anna ^h, Betschel, Stephen ⁱ, Boccon-Gibod, Isabelle ^j, Bouillet, Laurence ^{j, k}, Brodszki, Nicholas ^l, Busse, Paula J. ^m, Buttgereit, Thomas ^{c, d}, Bygum, Anette ⁿ, Cancian, Mauro ^o, Craig, Timothy ^p, Csuka, Dorottya ^q, Farkas, Henriette ^q, Fomina, Daria ^{r, s, t}, Gil-Serrano, Johana ^{u, v}, Gompels, Mark ^w, Guidos Fogelbach, Guillermo ^x, Guilarte, Mar ^{u, v}, Hide, Michihiro ^y, Kiani-Alikhan, Sorena ^z, Kinaciyan, Tamar ^aa, Lenten, Annet ^bb, lleonart, Ramon ^cc, Longhurst, Hilary ^dd, Lumry, William R. ^ee, Malbran, Alejandro ^ff, Malinauskiene, Laura ^gg, Matta Campos, Juan J. ^hh, Mendivil, Joan ⁱⁱ, Nieto-Martinez, Sandra A. ^jj, Peter, Jonathan G. ^kk, Porebski, Grzegorz ^ll, Reshef, Avner ^mm, Riedl, Marc ⁿⁿ, Valerieva, Anna ^oo, Waserman, Susan ^pp, Maurer, Marcus ^{c, d}, Cohn, Danny M. ^{a, ∗}

Source: In The Journal of Allergy and Clinical Immunology: In Practice June 2024 12(6):1614-1621

Challenging dogmas: Intravenous versus oral beta-lactam antibiotic provocation tests

Authors: Molina-Molina, Gustavo-Jorge, Gómez-Duque, Manuel, Vidal Guitart, Xavier, Agustí Escasany, Antònia, Labrador-Horrillo, Moisés, Luengo, Olga, Sala-Cunill, Anna, Galvan-Blasco, Paula, Guilarte, Mar, Cardona, Victoria

Source: In World Allergy Organization Journal June 2024 17(6)

Serum Amyloid A as a Potential Biomarker for Disease Activity in Chronic Spontaneous Urticaria

Authors: Carvallo, Alvaro, Veleiro, Beatriz, Sabaté-Brescó, Marina, Baeza, Maria Luisa, Guilarte, Mar, Herrera-Lasso, Valeria, Rodríguez-Garijo, Nuria, Diaz Donado, Carmen, Labrador-Horrillo, Moisés, Ferrer, Marta

Source: In The Journal of Allergy and Clinical Immunology: In Practice January 2024 12(1):195-200

Angioedema Due to Acquired Deficiency of C1-Inhibitor: A Cohort Study in Spain and a Comparison With Other Series

Authors: Baeza, Maria L., González-Quevedo, Teresa, Caballero, Teresa, Guilarte, Mar, Lleonart, Ramon, Varela, Susana, Castro, Monica, Díaz, Carmen, Escudero, Elena, García, Marimar G., Laffond, Elena, Muñoz-Bellido, Francisco.J., Núñez, Ramon, Prior, Nieves, Sala-Cunill, Anna, Sáenz de San Pedro, Blanca, Prieto-García, Alicia

Source: In The Journal of Allergy and Clinical Immunology: In Practice April 2022 10(4):1020-1028

One-Dilution Rapid Desensitization Protocol to Chemotherapeutic and Biological Agents: A Five-Year Experience

Authors: Sala-Cunill, Anna, Molina-Molina, Gustavo-Jorge, Verdesoto, Jenny-Tatiana, Labrador-Horrillo, Moisés, Luengo, Olga, Galvan-Blasco, Paula, Guilarte, Mar, Cardona, Victoria

Source: In The Journal of Allergy and Clinical Immunology: In Practice November 2021 9(11):4045-4054

A Comparative Study of Sex Distribution, Autoimmunity, Blood, and Inflammatory Parameters in Chronic Spontaneous Urticaria with Angioedema and Chronic Histaminergic Angioedema

Authors: Sabaté-Brescó, Marina, Rodriguez-Garijo, Núria, Azofra, Julian, Baeza, Maria Luisa, Donado, Carmen D., Gaig, Pere, Guilarte, Mar, Herrera-Lasso, Valeria, Labrador-Horrillo, Moisés, Sala-Cunill, Anna, Veleiro, Beatriz, Gil, María Pilar, Kaplan, Allen, Ferrer, Marta

Source: In The Journal of Allergy and Clinical Immunology: In Practice June 2021 9(6):2284-2292

De Novo or inherited: gonosomal mosaicism in hereditary angioedema due to C1 inhibitor deficiency.

Authors: Batlle-Masó, Laura, Perurena-Prieto, Janire, Viñas-Giménez, Laura, Aguiló-Cucurull, Aina, Fernández-Álvarez, Paula, Gil-Serrano, Johana, Guilarte, Mar, Colobran, Roger

Source: Frontiers in Immunology; 2025, p1-9, 9p

Subject Terms: GENETIC counseling, GENETIC testing, MOSAICISM, GENETIC disorders, PARENTS

Long‐term safety and efficacy of garadacimab for preventing hereditary angioedema attacks: Phase 3 open‐label extension study.

Authors: Reshef, Avner, Hsu, Connie, Katelaris, Constance H., Li, Philip H., Magerl, Markus, Yamagami, Keiko, Guilarte, Mar, Keith, Paul K., Bernstein, Jonathan A., Lawo, John‐Philip, Shetty, Harsha, Pollen, Maressa, Wieman, Lolis, Craig, Tim J.

Source: Allergy; Feb2025, Vol. 80 Issue 2, p545-556, 12p

Subject Terms: SAFETY, ANGIONEUROTIC edema, ADVERSE health care events, CLINICAL trials, PREVENTIVE medicine, TREATMENT effectiveness

Management of hereditary angioedema attacks by patients on long-term prophylaxis versus on-demand therapy only.

Authors: Lumry, William R.¹ LumryMD@AllergySpecialists.us, Grumach, Anete S.², Betschel, Stephen D.³, Radojicic, Cristine⁴, van Kooten, Sally⁵, Heckmann, Markus⁵, Danese, Sherry⁶, Malloy, Neil⁷, Goga, Ledia⁵, Guilarte, Mar⁸

Source: Allergy & Asthma Proceedings. Jan2025, Vol. 46 Issue 1, p32-37. 6p.

Subject Terms: Patients' attitudes, Standard deviations, Angioneurotic edema, Internet surveys, Everyday life

Prodromal symptoms of hereditary angioedema (HAE) attacks: A patient survey in UK and Spain.

Authors: Yong, Patrick F. K.¹ (AUTHOR), Guilarte, Mar^2,3 (AUTHOR) mar.guilarte@vallhebron.cat

Source: Allergy. Aug2024, Vol. 79 Issue 8, p2258-2262. 5p.

Subject Terms: *PATIENT experience, *MEDICAL personnel, *PATIENTS' attitudes, *SPANIARDS, *GENETIC mutation

Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.

Authors: Gil-Serrano, Johana, Horrillo, Moisés Labrador, Galvan-Blasco, Paula, Sala-Cunill, Anna, Bigas, Patricia, González, Javier Pereira, Luengo, Olga, Cardona, Victoria, Guilarte, Mar

Source: Frontiers in Immunology; 2024, p1-10, 10p

Subject Terms: ACUTE phase proteins, ANGIONEUROTIC edema, BLOOD sedimentation, LEUKOCYTES, INFLAMMATORY mediators, URTICARIA

Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain.

Authors: Guilarte, Mar^1,2,3 (AUTHOR) mguilarte@gmail.com, Sala-Cunill, Anna^1,2,3 (AUTHOR), Baeza, María Luisa^4,5 (AUTHOR), Cabañas, Rosario^6,7 (AUTHOR), Hernández, María Dolores⁸ (AUTHOR), Ibañez, Ethel⁸ (AUTHOR), de Larramendi, Carlos Hernando⁹ (AUTHOR), Lleonart, Ramon¹⁰ (AUTHOR), Lobera, Teófilo¹¹ (AUTHOR), Marqués, Luis¹² (AUTHOR), de San Pedro, Blanca Sáenz¹³ (AUTHOR), Botha, Jaco¹⁴ (AUTHOR), Andresen, Irmgard¹⁴ (AUTHOR), Caballero, Teresa^6,7 (AUTHOR)

Source: Allergy, Asthma & Clinical Immunology. 12/29/2021, Vol. 17 Issue 1, p1-11. 11p.

Subject Terms: *ANGIONEUROTIC edema, *BRADYKININ receptors, *DISEASE management, *TREATMENT effectiveness, *AGE of onset, *CLUSTER headache

Geographic Terms: SPAIN

Hereditary angioedema in Spain: medical care and patient journey.

Authors: Caballero, Teresa, Alonso, Carmen, Luisa Baeza, María, Baynova, Krasimira, Cabeza, José, Cortés, Isabel, Escobar Oblitas, Danilo, Guilarte, Mar, Joral, Alejandro, Jurado Palomo, Jesús, Lara Jiménez, María Ángeles, Martínez Virto, Ana, Medrano, Laura, Monte Boquet, Emilio, Navarro, Montserrat, Pérez, Diego, Plá Martí, María José, Smith Foltz, Sara L., Suero, Coral, Zamora, Carolina

Source: Orphanet Journal of Rare Diseases; 5/21/2024, Vol. 19 Issue 1, p1-10, 10p, 4 Diagrams, 1 Chart

Plasma contact system activation drives anaphylaxis in severe mast cell–mediated allergic reactions

Authors: Sala-Cunill, Anna, Björkqvist, Jenny, Senter, Riccardo, Guilarte, Mar, Cardona, Victoria, Labrador, Moises, Nickel, Katrin F., Butler, Lynn, Luengo, Olga, Kumar, Parvin, Labberton, Linda, Long, Andy, Di Gennaro, Antonio, Kenne, Ellinor, Jämsä, Anne, Krieger, Thorsten, Schlüter, Hartmut, Fuchs, Tobias, Flohr, Stefanie, Hassiepen, Ulrich, Cumin, Frederic, McCrae, Keith, Maas, Coen, Stavrou, Evi, Renné, Thomas

Source: In The Journal of Allergy and Clinical Immunology April 2015 135(4):1031-1043

Identification and characterization of a novel splice site mutation in the SERPING1 gene in a family with hereditary angioedema

Authors: Colobran, Roger, Lois, Sergio, de la Cruz, Xavier, Pujol-Borrell, Ricardo, Hernández-González, Manuel, Guilarte, Mar

Source: In Clinical Immunology February 2014 150(2):143-148

HARBOR: An Ongoing Phase 2/3 Study of Elenestinib in Patients With Indolent Systemic Mastocytosis

Authors: Castells, Mariana ¹, Livideanu, Cristina Bulai ², Hermine, Olivier ³, Sabato, Vito ⁴, Maurer, Marcus ⁵, Ruëff, Franziska ⁶, Barete, Stephane ⁷, Bouillet, Laurence ⁸, Panse, Jens ⁹, Twose-Alvarez, Ivan ¹⁰, González-De-Olano, David ¹¹, Cabral, Renata ¹², Bird, Robert ¹³, Hartmann, Karin ¹⁴, Rego, T.J. ¹⁵, He, Kevin ¹⁵, Muñoz-González, Javier ¹⁵, Scherber, Robyn ¹⁶, Tashi, Tsewang ¹⁷, Guilarte, Mar ¹⁸

Source: In The Journal of Allergy and Clinical Immunology February 2025 155(2) Supplement:AB170-AB170

Angioedema severity and impact on quality of life: Chronic histaminergic angioedema versus chronic spontaneous urticaria

Authors: Rodríguez-Garijo, Nuria, Sabaté-Brescó, Marina, Azofra, Julián, Baeza, Maria Luisa, Donado, Camen Diaz, Gaig, Pere, Guilarte, Mar, Herrera-Lasso, Valeria, Labrador-Horrillo, Moisés, Sala-Cunill, Anna, Veleiro, Beatriz, Gil, Maria Pilar, Ferrer, Marta

Source: In The Journal of Allergy and Clinical Immunology: In Practice November 2022 10(11):3039-3043

Chronic psychosocial stress induces reversible mitochondrial damage and corticotropin-releasing factor receptor type-1 upregulation in the rat intestine and IBS-like gut dysfunction

Authors: Vicario, María, Alonso, Carmen, Guilarte, Mar, Serra, Jordi, Martínez, Cristina, González-Castro, Ana M., Lobo, Beatriz, Antolín, María, Andreu, Antoni L., García-Arumí, Elena, Casellas, Montserrat, Saperas, Esteban, Malagelada, Juan Ramón, Azpiroz, Fernando, Santos, Javier

Source: In Psychoneuroendocrinology January 2012 37(1):65-77