Pavlina Kralickova

@lfhk.cuni.cz

Institute of Clinical Immunology and Allergy, Charles University, Faculty of Medicine in Hradec Kralove
University Hospital Hradec Kralove

Pavlina Kralickova


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https://researchid.co/pavlinakralickova

RESEARCH INTERESTS

Primary and secondary immunodeficiency
Allergy
65

Scopus Publications

Scopus Publications

  • Oral deucrictibant for on-demand treatment of hereditary angioedema attacks (RAPIDe-1): a randomised, double-blind, placebo-controlled, phase 2 trial
    Marcus Maurer, Marcin Stobiecki, Anna Valerieva, Roman Hakl, Maria T Staevska, Laurence Bouillet, Aurélie Du-Thanh, Aharon Kessel, Sorena Kiani-Alikhan, Markus Magerl, Avner Reshef, Maria Luisa Baeza, Olivier Fain, Henriette Farkas, Jens Greve, Mar Guilarte, Joshua S Jacobs, H Henry Li, Ramon Lleonart, Michael E Manning, Gordon L Sussman, John Anderson, Hugo Chapdelaine, Danny M Cohn, David Hagin, Pavlína Králíčková, Bruce Ritchie, Giuseppe Spadaro, Petra Staubach, Michael D Tarzi, William H Yang, Marie-Helene Jouvin, Rafael Crabbé, Simone van Leeuwen, Huaihou Chen, Li Zhu, Jochen Knolle, Anne Lesage, Peng Lu, Marc A Riedl, Emel Aygören-Pürsün
    Lancet Haematology, 2026
    BACKGROUND: Hereditary angioedema is a bradykinin-mediated disorder characterised by recurrent painful swelling attacks. Treatment relies on medications to prevent attacks and on-demand therapies for attack manifestations. Parenteral administration associated with most available on-demand therapies often leads to treatment being delayed or forgone. Deucrictibant is an orally bioavailable bradykinin B2 receptor antagonist under development for prophylaxis and on-demand treatment of hereditary angioedema attacks. We aimed to investigate the efficacy and safety of deucrictibant for the on-demand treatment of hereditary angioedema attacks. METHODS: RAPIDe-1 was a double-blind, randomised, placebo-controlled, crossover, dose-ranging, phase 2 trial that recruited adults aged 18-75 years with hereditary angioedema type 1 or 2 from 38 sites (eg, university hospitals and accredited angioedema centres) across North America, Europe, and Israel. Participants were required to have experienced two or more attacks within the past 2 months or three or more attacks within the past 4 months before screening. An interactive response technology system randomly assigned eligible patients (1:1:1) to receive a blinded dose of oral deucrictibant (immediate-release capsule) 10 mg, 20 mg, or 30 mg during an attack-free period to assess pharmacokinetics and safety in the part 1 of the study; and, subsequently, to receive a crossover treatment sequence of two administrations of the same deucrictibant dose (10 mg, 20 mg, or 30 mg) and one of placebo to treat three investigator-confirmed angioedema attacks in the part 2. Randomisation was stratified by whether the participant was willing to participate in full pharmacokinetic sampling. Participants, investigators, site personnel, and the sponsor were masked to treatment assignment and capsules of deucrictibant and placebo were identical. For each attack, patients self-administered the oral study drug within 3 h after at least one symptom (skin pain, skin swelling, or abdominal pain) reached a visual analog scale (VAS) individual score of 30 or more out of 100 and within 6 h from symptom onset. The primary endpoint was change in the patient-reported composite VAS-3 score measuring severity of attack manifestations from before treatment to 4 h post-treatment and assessed in the modified intention-to-treat and per-protocol populations. Safety was assessed in all patients who received any dose of study drug. RAPIDe-1 is registered with ClinicalTrials.gov (NCT04618211) and is completed. FINDINGS: Between Feb 3, 2021, and June 23, 2022, 89 patients were screened, of whom 74 were randomly assigned between Feb 23, 2021, and July 26, 2022. For the primary analysis set, the median follow-up was 130·0 (IQR 92·5 to 212·5) days for the deucrictibant 10 mg group, 166·5 (80·0 to 275·0) days for the deucrictibant 20 mg group, and 172·0 (65·0 to 242·0) days for the deucrictibant 30 mg group. The primary efficacy analysis included 147 attacks in 62 patients, of whom 42 (68%) were female and 60 (97%) were White. Least squares mean differences of change in VAS-3 score between deucrictibant and placebo was -16·75 (95% CI -21·52 to -11·97) for 10 mg, -15·02 (-20·22 to -9·81; p<0·0001) for 20 mg, and -16·28 (-21·27 to -11·29; p<0·0001) for 30 mg. In part 1, all treatment-emergent adverse events were grades 1-2, with the most common in two or more patients being headache (two [8%] of 25) and nasopharyngitis (two [8%] of 25) in the deucrictibant 30 mg group. In part 2, no single treatment-emergent adverse event was reported by two or more patients in any treatment group. Most adverse events were considered unrelated to the study drug and there were no grade 3 or worse adverse events. INTERPRETATION: Deucrictibant significantly reduced the severity of hereditary angioedema attacks compared with placebo; these results support continued investigation of antagonism of the bradykinin B2 receptor with an orally available agent as a potentially effective approach, with a safety profile similar to placebo, for on-demand treatment. FUNDING: Pharvaris.
  • Epidemiology and management of malignancies in patients with inborn errors of immunity—An ESID registry study of 19,959 patients
    Delfien J.A. Bogaert, Christina H. Wolfsberger, Andishe Attarbaschi, Jonathan Gathmann, Klaus Warnatz, Gabriele Mueller, Anna Mukhina, Stephan Rusch, Dalia Abd Elaziz, Sohilla Lofty M. Abdelkader, Avigaelle Abitbol, Hassan Abolhassani, Lalash Abrahim, Mohamed Abuzakouk, Pietro Andrea Accardo, Nabila Achir Moussouni, Veronica Afonso, Philipp Agyeman, Martina Ahlmann, Alessandro Aiuti, Abla Akl, Güzide Aksu, Kim Albers, Michael H. Albert, Meda Diana Alecsandru, Olga Aleinikova, Svetlana Aleshkevich, Radwa Salah Eldeen Youssif Alkady, Luis Allende, Mickaël Alligon, Zoe Allwood, Manrique de Lara Laia Alsina, Daniela Ambrosch-Barsoumian, Lisa Ameshofer, Kenza Amour, Evrim Anadol, Ariharan Ananthachagaran, Chantal Andriamanga, Julia Andris, Karin Andritschke, Federica Angelini, Tobias Ankermann, Katrin Apel, Siamak Arami, Ömür Ardeniz, Peter Arkwright, Karina Arnold, Rudolf Ascherl, Najla Assam, Nurit Assia-Batzir, Faranaz Atschekzei, Sybille Aumann, Volker Aumann, Magnus Aurivillius, Bernd Ausserer, Tadej Avcin, Sezin Aydemir, Emel Aygören-Pürsün, Cagdas AyvazDeniz, Chiara Azzari, Lo Babacar, Perrine Bach, Sophie Bachmann, Peter Bader, Shahrzad Bakhtiar, Renate Bancé, Catherine Bangs, Katerina Barfusz, Safa Baris, B. BarlanIsil, Michaela Bartsch, Lucia Augusta Baselli, Laura Batlle-Maso, Helge Baumann, Ulrich Baumann, Veronika Baumeister, Helen Baxendale, Suzanne Bazen, Beatrice Beaurain, Julien Beauté, Mounia Bechar-Makhloufi, Norbert Beck, Brigitta Becker, Christian Becker, Uta Behrends, Renata Beider, Rita Beier, Amel Belkacem, Luisa Belke, Sven Bellert, Bernd H. Belohradsky, Aouatef Ben-Bouzid, Vincent Benoît, Lilia BenSlama, Thamila Berdous-Sahed, Jan Bergils, Anna Berglöf, Peter Bergman, Katarzyna Bernat-Sitarz, Jolanta Bernatoniene, Ewa Bernatowska, Benedikt Bernbeck, Elena Bertolini, Claire Bethune, Kai Beuckmann, Malini Bhole, Anika-Kerstin Biegner, Stefan Bielack, Kirsten Bienemann, Arndt Bigl, Amélie Bigorgne, Marc Bijl, Nadine Binder, Michaela Bitzenhofer-Grüber, Geraldine Blanchard Rohner, Dagmar Blank, Claudia Blattmann, Julia Blau, Audra Blaziene, Stefan Blazina, Markéta Bloomfield, Roswitha Blume, Barbara Boardman, Sebastian Bode, Jaap-Jan Boelens, Christoph Boesecke, Delfien Bogaert, Johannes Bogner, Nadezda Bohynikova, Claire Booth, Victoria Bordon, Arndt Borkhardt, Melanie Börries, Michael Borte, Stephan Borte, Lukas Bossaller, Madeleine Bossard, Jeannet Botros, Soraya Boucherit, Jeannette Boutros, Oksana Boyarchuk, Onur Boyman, Kaan Boztug, Sara Branco Pereira da Silva, Thomas Braschler, Sophie Bravo, Robbert Bredius, Philip Bright, Carolina Brito de Azevedo Amaral, Nicholas Brodszki, Grit Brodt, Allan Brolund, Pauline Brosselin, Bastian Brummel, Sonja Brun-Schmid, Jürgen Brunner, Roswitha Bruns, Christina Buchta, Dietke Buck, Aileen Bücker, Matthew Buckland, Martina Bührlen, Stefan Burdach, Siobhan Burns, Janet Burton, Luis Caminal, Caterina Cancrini, Fabio Candotti, Clementina Canessa, Andrew J. Cant, Nathan Cantoni, Brindusa Capilna, Fabiola Caracseghi, Isabel Caragol, Javier Carbone, Maria Carrabba, Jean-Laurent Casanova, Latanya Chamberlain, Anita Chandra, Christophe Chantrain, Helen Chapel, Julie Chassot, Ronnie Chee, Matteo Chinello, Charu Chopra, Zita Chovancova, Martin Christmann, Krystyna Chrzanowska, Peter Ciznar, Karlien Claes, Carl Friedrich Classen, Martin Classen, Alexis-Virgil Cochino, Alison M. Condliffe, Selim Corbacioglu, Ana Isabel Cordeiro, Donatienne Cordier Wynar, Claire Core, Laurence Costes, Tanya Coulter, Virginie Courteille, Maria Cristina, Maria Cucuruz, Leonik Nel Dabrowska, Nel Dabrowska Leonik, Mandy Dähling, Mary Louise Daly, Claudia-Sabrina Daniel, Maria Giovanna Danieli, James Darroch, Graham Davies, Javier De Gracia Roldan, Narimene de Nadai, Iris de Schutter, Nathalie De Vergnes, Esther de Vries, Josine de Witte, Frans deBaets, Theo Debert, Christiane DeBoeck, Corina Defila, Judith Deimel, Diane Delaplace, Rosa Maria Dellepiane, Nelli Dellert, Laura Delliera, Anita Delor, Ulrike Demel, John Dempster, M.M. den Os, Rosmarie Dengg, Sora Asfaw Desisa, Alexandra Desta, Drahomira Detkova, Maite Dewerchin, Romina Dieli Crimi, Dagmar Dilloo, Florentia Dimitriou, Sarah Svenja Dinges, Jasmin Dinser, Nabila Dipani, Johannes Dirks, Anna-Maria Dittrich, Lylia Djermane, Yagmur Dogru, Figen Dogu Esin, Angelika Dombrowski, Julia Dominguez Escobar, Michaela Döring, Camilla Heldbjerg Drabe, Susann Drerup, Barbara Drexel, G.J.A. Driessen, Gregor Dückers, Yasmine Dudoit, Andrea Duppenthaler, Georg Ebetsberger-Dachs, Mate Barnabas Ecser, J. David Edgar, Maartje Eekman, Stephan Ehl, Rosanna Ehrat, Eva Eisl, Olov Ekwall, Rabab El Hawary, Sabine M. El-Helou, Aisha El-Marsafy, Sarina Elbe, Suzanne Elcombe, Alia Eldash, Youssif Alkady Radwa Salah Eldeen, P.M. Ellerbroek, Roland Elling, Jane Elliott, Aisha Elmarsafy, Angelika Engelhardt, Diana Ernst, Fügen Ersoy, Stefanie Esper, Isabel Esteves, Amos Etzioni, Andrew Exley, Martin Faber, Giovanna Fabio, Gaby Fahrni, Laura Eva Faletti, Claire-Michele Farber, João Farela Neves, Emilia Faria, Henriette Farkas, Evangelia Farmaki, Maria Faßhauer, Anders Fasth, Gerd Fätkenheuer, Stefanie Faustmann, Gisela Fecker, Conleth Feighery, Cornelia Feiterna-Sperling, Perez Eduardo Fernandez-Cruz, Cordeiro Ferreira Concalo, Alice Ferster, Tobias Feuchtinger, Oliver Feyen, Daniela Finke, Alain Fischer, Sigrid Fitter, Stefan Flaschberger, Lucia Fleckenstein, Dirk Föll, Adriano Fontana, Concetta Forino, Elisabeth Förster-Waldl, Nora Franchet, Dagmar Freitag, Urs P. Frey, Hannah Margarete Frick, Elisabeth Friedel, Wilhelm Friedrich, Barbara Frisch, Lukas Frischknecht, Stephanie Fritzemeyer, Alenka Gagro, Manfred Gahr, Nermeen Mouftah Galal, Eleonora Gambineri, Agnes Gamper, Franziska Gams, Astrid Ganzow, Nicolas Garcelon, Tomaz Garcez, Marina GarciaPrat, Ann Gardulf, Janine Garibay, Birgit Garwer, Jonathan Gathmann, Benjamin Gathmann, Corinna Gebauer, Linda Geberzahn, Tilman Geikowski, Ulf Geisen, Christiane Gemander, Andrew R. Gennery, Marie Gerisch, Michael Gernert, Katrin Gerrer, Stev Gerschmann, Sujal Ghosh, Carolin Giannini, Juana Gil Herrera, Noemi Gimenez Sanz, Matthias Girndt, Ramona Girrbach, Hermann Girschick, Antonios Gkantaras, Ioannis Gkougkourelas, Dominika Gładysz, Susanne Gnatowski, Elisabeth Gnodtke, Vera Goda, Sarah Goddard, Daniela Goebel, Jean-Christophe Goffard, Sigune Goldacker, Eva Maria Gollowitsch, Manuella Gomes, Mark Gompels, Míriam González, Luis Ignacio Gonzalez Granado, Pavels Gordins, Katharina Gößling, Lisa Göschl, Lucy Gossens, Ewelina Gowin, Lea Graafen, Leo Graca, Brigita Gradauskiene-Sitkauskiene, Dagmar Graf, Norbert Graf, Elliot Grange, H. Anne Grashoff, Johann Greil, Sofia Grigoriadou, Bodo Grimbacher, Helen Gronlund, Ute Groß-Wieltsch, Teresa Guerra, Kissy Guevara-Hoyer, Mor Seny Gueye, Noemie Guibert, Birgit Gülnur, Tayfun Güngör, Marina Guseva, David Guzman, Marcel Haag, Gabriele Haase, Henriette Haenicke, Filomeen Haerynck, Ines Hafsa, David Hagin, Emine Haliti, Michael Hallek, Lennart Hammarstroem, Gonca Hancioglu, Rupert Handgretinger, Leif G. Hanitsch, Susanne Hansen, Tetyana Hariyan, Thomas Harrer, Pia Hassunah, Maria Hatzistilianou, Fabian Hauck, Thomas Hauser, Margje H. Haverkamp, Grant Hayman, Paul Heath, Christian Hedrich, Maximilian Heeg, Michael Heike, Martin Heimbrodt, Sabine Heine, Ulrich Heininger, Christian Heinrich, Valerie Heinz, Andreas Heitger, Matthew Helbert, Arthur Helbling, Camilla Heldbjerg Drabe, Antje Hellige, Julya Hempel, Karen Henderson, Jörg Henes, Philipp Henneke, Christian Hennig, Karin Henrichs, Martin Herbst, Walter Hermann, Manuel Hernandez, Anja Hernández, Edyta Heropolitanska-Pliszka, Richard Herriot, Friedrich Herrmann, Archana Herwadkar, Christoph Hess, Ursula Hess, Sebastian Hesse, Sonja Higgins, Anna Hilfanova, Sophie Hilpert, Chantal Hintze, Eva Hlaváčková, Isabel Hodl, Adna Hodzic, Miriam Hoernes, Christina Hoffmann, Andreas Holbro, Christiane Höllinger, Lisa Holtsch, Ursula Holzer, Dirk Holzinger, Manfred Hönig, Andrea Hönscheid, Julia Horn, Gerd Horneff, Claire Hoyoux, Nataliya Hristova, Kai Hübel, Angela Hübner, Christian Huemer, Aarnoud Huissoon, Brigitte Hülsmann, Patrick Hundsdörfer, Hans-Iko Huppertz, Kristina Huß, Sadia Hussain, Julien Husson, Tanja Hüttner-Foehlisch, Hanna Ijspeert, Aydan Ikinciogullari, Kökçü İlknur, Ninela Irga, Alexandra Jablonka, Karina Jahnz-Rozyk, Marcus Jakob, Donate Jakoby, Donate Jakoby-Gaide, Peter Jandus, Annette Jansson, Melanie Jaquet, Helene Jardefors, Edyta Jargulinska, Barbara Jauk, Milos Jesenak, Katharina Jilka, Stephen Jolles, Alison Jones, Regina Jones, Birgit Jonkman-Berk, Göran Jönsson, Hilary J. Joyce, Pricillia Juliana, Michael Kabesch, Leo Kager, Christian Kahlert, Petra Kaiser-Labusch, Ioannis Kakkas, Dirk Kamitz, Maria Kanariou, Lothar Kanz, Elif Karakoc-Aydiner, Boris Karanovic, Mutlu Kartal-Kaess, Elisabeth Käser, Terese L. Katzenstein, Hana Kayserova, Peter Kelleher, Tessa Kerre, Sara Sebnem Kilic, Gerhard Kindle, Martina Kirchner, Simone Kiwit, Ayca Kiykim, Jessica Klasen, Maja Klaudel-Dreszler, Ariane Klein, Christoph Klein, Andreas Klein-Franke, Ilona Kleine, Stefan Kleinert, Christian Klemann, Marion Klima, Adam Klocperk, Robin Kobbe, Dilara Fatma Kocacik Uygun, Melanie Koch, Yvonne Kochler, Naschla Kohistani, Marina Kojic, Antonio Kolios, Uwe Kölsch, Sylwia Koltan, Irina Kondratenko, Christoph Königs, Julia Konoplyannikova, Peter Kopac, Jana Kopp, Dieter Körholz, Julia Körholz, Pauline Korte, Larysa Kostyuchenko, Ina Kötter, Sven Kracker, Pavlina Králícková, Christof Kramm, Philipp Kramme, Máté Krausz, Wolfhart Kreuz, Johanna Krista, Gergely Kriván, Gabriele Kropshofer, Renate Krüger, Olga Krystufkova, Maria Ktistaki, Jörn-Sven Kühl, Alexander Kühn, Taco W. Kuijpers, Wietse Kuis, Silke Kullmann, Andreas Kulozik, Dinakantha Kumararatne, Ria Kümmler, Andrea Kündgen, Magdalena Kurenko-Deptuch, Cosima KussPaula, Necil Kütükcüler, Cécile Lafoix-Mignot, Beate Lamers, Peter Lanbeck, Paul Landais, Sybille Landwehr-Kenzel, Vanessa Langemeyer, Thorsten Langer, Petra Lankisch, Nadia Lanz, Manrique de Lara, Eusebia Lara-Villacanas, Lisa Laubenthal, Hans-Jürgen Laws, Ronan Leahy, Jae-Yun Lee, Andrea Lehmann, Kai Lehmberg, Patricia Lehner, Hans Leibfrit, Leoni Leistner, Loic LeMignot, Petra Lesch, Simon Leutner, Manolis Liatsis, Linda Libai Véghová, Johanna Liebel, Johannes G. Liese, Kotryna Linauskiene, Richard Linde, Martina Linßner, Conrad Ferdinand Lippert, Jiri Litzman, Pilar Llobet, Babacar Lo, Tariq Lodin, Jindrich Lokaj, David Longhino, Hilary Longhurst, Susana Lopes da Silva, Catharina Lorenzen, Vassilios Lougaris, Doris Löw, Annelie Lubatschofski, Mary Lucas, Verena Lutz-Wiegers, Sabine Maaß, Maria Elena Maccari, Anna Macura-Biegun, Vanessa Maerz, Paraskevi Maggina, Nizar Mahlaoui, Hannah Mahrenholz, Sarah Maier, Mounia Makhlouf, Anne Malfroot, Laura Malinauskiene, Wilma Mannhardt-Laakmann, Ania Manson, Felicia Mantkowski, Petra Manzey, Carolina Marasco, Nufar Marcus-Mandelblit, Wolfgang Marg, Gasper Markelj, Laszlo Marodi, José Goncalo Marques, Laura Marques, Karin Marschall, Natalia Martínez, Rafael Martinez de la Ossa Saenz-Lopez, Inmaculada Martinez-Saguer, Baldassarre Martire, Antonio Marzollo, Refiloe Masekela, Katja Masjosthusmann, Tania Nicole Masmas, Nuria Matamoros, Jutta Mattern, Pearl Mau-Asam, Elizabeth McDermott, Nichole McIntosh, Karmen Mesko Meglic, Ruben Meijer, Andrea Meinhardt, Safa Meshaal, Yasmina Messaoud, Björn Meyer, Dirk Meyer-Olson, Isabelle Meyts, Romain Micol, Bozena Micoloc, Gudrun Mielke, Cinzia Milito, Tomas Milota, Siraj Misbah, Carolin Mödden, Michael Mohr, Karina Mohrmann, Mostafa Moin, Luis Molinos, Jana Möller, Sarah Möller-Nehring, Henner Morbach, Viviana Moschese, Olga Moser, Despina Moshous, Radoslaw Motkowski, Jayashree Motwani, Nermeen MouftahGalal, Carmen Müglich, Anna Mukhina, Eva Muller, Christiane Müller, Gabriele Müller, Hedi Müller, Ingo Müller, Thomas Müller, Zoe Müller, Ulf Müller-Ladner, Sarah Müller-Stöver, Esther Münstermann, Núria Murtra Garrell, Moritz Muschaweck, Miriam Mutert, Zohreh Nademi, Paru Naik, Andrea Näke, Andrea Martin Nalda, Gulnara Nasrullayeva, Nora Naumann-Bartsch, Verena Nemitz, Olaf Neth, Andreas Neubauer, Jennifer Neubert, Carla Neumann, Tim Niehues, Mara Niemuth, Alexandra Nieters, Chris Nieuwhof, Daniela Nolkemper, Sadia Noorani, Budde Adya Noorlander, Luigi D. Notarangelo, Gundula Notheis, Sanam Amelie Nowatsh, Gaelle Obenga, Suheyla Ocak, Mehmet Oker, Peter Olbrich, Anna Olipra, Heymut Omran, Prasad Oommen, Linda Opitz, Jaroslava Orosova, Solveig Oskarsdottir, Hülya Özsahin, Malgorzata Pac, Jana Pachlopnik-Schmid, Franco Pandolfi, Efimia Papadopoulou-Alataki, Theodora Papastamatiou, Anna Papatriantafillou-Schmieder, Alba Parra-Martinez, Olga Paschenko, Srdjan Pašić, Jarek Pasnik, Smita Patel, Martin Pavlík, Estela Paz Artal, Anouk Peeters, Sara Branco Pereira da Silva, Ruy Perez-Becker, Marc Perez-Guzman, Martine Pergent, Markus Perlhagen, Hans-Hartmut Peter, Marin Petrić, Michael Pfreundschuh, Pierre Philippet, Capucine Picard, Barbara Pietrucha, Leonora Pietzsch, Claudio Pignata, Monica Piquer Gibert, Kerstin Pirolt, Alessandro Plebani, Daniel E. Pleguezuelo, Katrin Pollok, Helena Pommerening, Daniela Popihn, Aleksandra Poplonek, Marina Popp, Fulvio Porta, Jan Portegys, Klara Posfay-Barbe, Judith Potjewijd, Sebastian Poulheim, Seraina Prader, Petra Prämassing-Scherzer, Martina Prelog, Johan Prevot, Arthur Price, Timothy Price, Marijke Proesmans, Johan Provot, Federica Pulvirenti, Isabella Quinti, Anna Raab, Anita Rack, Stefan Raffac, Eduardo Ramos Oviedo, Philippe Randrianomenjanahary, Anja Ranohavimparany, Maria Raptaki, Roonaka Rashidzadeh, Margit Rathwallner, Shereen Reda, Nahida Redouane, Frederico S. Regateiro, Janine Reichenbach, Bianca Reimers, Cornelia Reinhardt, Dirk Reinhardt, Anne Reinprecht, Tamara Reiß, Ismail Reisli, Eleonore Renner, Nima Rezaei, Alex Richter, Darko Richter, Nikolaus Peter Rieber, Nadja Rieckehr, Marion Riedel, Heidi Riescher, Johannes Rischewski, Nicole Ristl, Henrike Ritterbusch, Tanja Ritz, Francois Rivier, Jacques G. Riviere, Peter Robinson, Jürgen K. Rockstroh, Joachim Roesler, Himatur Rofiah, Elizabeth Rogerson, Elisabeth Rolfes, Beate Roller, Yaryna Romanyshyn, Roberto Rondelli, Fien Roosens, Angela Rösen-Wolff, Valentina Rösler, Johannes Roth, Tobias Rothoeft, Agathe Roubertie, Gesa Rübsam, Stephan Rusch, Abraham Rutgers, Paul Ryan, Gudrun Sach, Kambis Sadeghi, Ulrike Sahrbacher, Angelika Saidi, Özden Sanal Tezcan, Silvia Sanchez-Ramon, Juan Luis Santos, Ravishankar Sargur, Ihor Savchak, Sinisa Savic, Bernhard Schaaf, Marzena Schaefer, Christina Schäfe, Eva Scharbatke, Ellen Schatorje, Uwe Schauer, Carmen Scheibenbogen, Raphael Scheible, Clemens Scheinecker, Romana Schiller, Beatrice Schilling, Freimut Schilling, Steffi Schlieben, Thilo Schmalbach, Marc Thomas Schmalzing, Pirmin Schmid, Nadine Schmidt, Reinhold Ernst Schmidt, Monika Schmitz, Dominik Schneider, Dominik T. Schneider, Reinhard Schneppenheim, Cathy Scholtes, E.H. Schölvinck, Stefan Schönberger, Stefan Schreiber, Rik Schrijvers, Andrea Schroll, Simone Schruhl, Johanna Schrum, Ralf Schubert, Catharina Schuetz, Sebastian Schuh, Ansgar Schulz, Claudia Schulz, Ilka Schulze, Hendrik Schulze-Koops, Ulf Schulze-Sturm, Eva-Maria Schumacher, Elvira Schürmann, Gesine Schürmann, Volker Schuster, Eva Schwaneck, Klaus Schwarz, Tobias Schwarz, Carolynne Schwarze-Zander, Lothar Schweigerer, Anna Sediva, Jörg Seebach, Reinhard Seger, Florian Segerer, Markus G. Seidel, Barbara Selle, Suranjith Seneviratne, Mikko Seppänen, Hatidje Shabanaj, Svetlana Sharapova, Anna Shcherbina, Benjamin Shillitoe, Anne Siepelmeyer, Kathrin Siepermann, Anna Simon, Arne Simon, Katja Simon-Klingenstein, Marija Simonovic, Merlin Simsen-Baratault, Elena Sindram, Alla Skapenko, Maiken Skarke, Malgorzata Skomska-Pawliszak, Mary Slatter, Julie Smet, C.I. Edvard Smith, Ali Sobh, Bettina Sobik, Georgios Sogkas, Michael Sohm, Xavier Solanich, Xavier Solanich-Moreno, Pere Soler Palacín, Franz Sollinger, Raz Somech, Anja Sonnenschein, Annarosa Soresina, Marijke Sornsakrin, Stavrieta Soura, Sabrina Spaccarotella, Guiseppe Spadaro, Monika Sparber-Sauer, Christof Specker, Carsten Speckmann, Lisa Speidel, Matthaios Speletas, Klaus-Daniel Stachel, Catherine Stadon, Aurélie Stanislas, Cynthia Stapornwongkul, Paulina Staus, Regina Steck, Cathal Steele, Herbert Steffin, Urs Steiner, Sandra Steinmann, Wim Stevens, Martina Stiefel, Sarah Stieger, Sophie Stiehler, Hermann Stimm, Silvia Stojanov, Matthias Stoll, Dominique Stoppa-Lyonnet, Tobias Strapatsas, Gabriele Strauß, Monika Streiter, Riet Strik-Albers, Gaby Strotmann, Héctor SuárezCasado, Jose Luis Subiza, Mikael Sundin, Birgit Süß, Fabienne Sutter, Anna Szaflarska, Monika Szemkus, Hannah Tamary, Sofia Tantou, Michael D. Tarzi, Helga Taschner, Ulf Tedgard, Carla Teixeira, R.J.M. ten Berge, Klaus Tenbrock, Sabine Tester, Ilhan Tezcan, Sonja Thalguter, Julian Thalhammer, Katharina Thoma, Moira Thomas, Fabian Thomczyk, Vojtech Thon, Adrian Thrasher, Patricia Tierney, Nadine Tietsch, Alberto Tommasini, Beate Tönnes, Hans-Peter Tony, Maria Trachana, Carmen Trapp, Lourdes Tricas, Maria Conceicao Trindade Neves, Jordis Trischler, Hugo Ubieto, Anett Uelzen, Annette Uhlmann, Jan Ullrich, Kurt Ullrich, Ekrem Ünal, Gerhard Urbanski, Simon Urschel, Aleksandra Uszynska, Angelo Vacca, N.N. Vaganov, Daniel Vagedes, Stefanie Valicevic, Florence Vallelian, Rachel T. van Beem, Charlotte van Damme, Annick van de Ven, J. Merlijn van den Berg, Michiel van der Flier, J.T. van Dissel, P.M. van Hagen, J.M. van Montfrans, Geoffrey van Ogtrop, Jacqui van Rens, Christel A.M.P. van Riel, Annet van Royen-Kerkhof, G.Th.J. van Well, Antari Vasiliki, Sirje Velbri, Jo Vencken, Alessandro Ventura, François Vermeulen, Christiane Vermylen, Dorothee Viemann, Anja Viereck, Anna Villa, Jeroen Vincke, Lisa Vinnemeier-Laubenthal, Kim Duy Vo Thi, Mirjam Voeller, Kristina Vollbach, Alla Volokha, Horst von Bernuth, Philipp von Bismarck, Rebecca Voss, Sandra Voß, Yüksel Vural, Heike Wachuga, Norbert Wagner, Per Wagström, Matthias Wahle, Volker Wahn, Nadine Wapp, Klaus Warnatz, Monika Warneke, Adilia Warris, Jan-Christian Wasmuth, Jean-Blaise Wasserfallen, Angela Wawer, Manfred Weber, Corrie M.R. Weemaes, Lisa Wege, Julius Wehrle, Stephan Weidinger, Michael Weiß, Elisabeth Weißbarth-Riedel, Antje Werner, Sara Wessel, Marco Westkemper, Monika Wicher, Lutz Wickmann, Sabine Wiegert, Monique Wiehe, Katharina Wiehler, Lydia Wiesböck, Ewa Wiesik-Szewczyk, Anthony Williams, Beate Winkler, Christel Winkler, Martina Winkler, Melanie Winkler, Uwe Wintergerst, Lukas Wisgrill, Torsten Witte, Helmut Wittkowski, Barbara Wolf, Sandra Wölke, Christina Wolschner, Beata Wolska-Kusnierz, Philip Wood, Sarita Workman, Austen Worth, Michaela Wortmann, Walter Alfred Wuillemin, Nico M. Wulffraat, Katharina Wustrau, Chloé Wyndham-Thomas, Olcay Yegin, Alisan Yildiran, Denise Yilmaz, Patrick Young, Esra Yucel, Milica Zečević, Erić Želimir, Fred Zepp, Klaus Zetzsche, Rainald Zeuner, Stefan Zielen, Martina Zimmermann, Gerhard Kindle, Joris M. van Montfrans, Markus G. Seidel
    Journal of Allergy and Clinical Immunology, 2026
    BACKGROUND: Inborn errors of immunity (IEI), or primary immune disorders (PIDs), predispose individuals to infections, autoimmunity, inflammation, allergy, and malignancy. Malignancies are a major cause of morbidity and mortality in patients with IEI/PIDs, with poorer outcomes compared with the general population. OBJECTIVE: We sought to determine the frequency and types of malignancies in patients with IEI/PIDs and to assess clinical management approaches across Europe. METHODS: Descriptive analyses were performed on malignancy data within each IEI category. In addition, a European Society for Immunodeficiencies Registry survey (05/2022-03/2024) collected data on management strategies and challenges. RESULTS: Of 19,959 patients with IEI/PIDs, 1783 (8.9%) developed malignancies, of whom 27.1% presented malignancy as first manifestation of IEI/PIDs. A total of 1210 malignancies were specified; B-cell non-Hodgkin lymphoma was most common (24.2%). Detailed malignancy-IEI/PID association maps are provided. Predominantly antibody deficiencies accounted for 59.1% of malignancy cases, with a higher median age at first malignancy (43.6 years) compared with other IEI/PID categories, for example, combined immunodeficiencies with syndromic or associated features (11.7 years). Survey findings revealed that oncological treatment was modified because of IEI/PIDs in 21.5% of cases, with assumed negative impacts of IEI/PIDs on complications and outcomes (in 27.4% and 30.7%, respectively). IEI/PIDs influenced transplant decisions in 16.5% of cases. Management practices such as interdisciplinary decision finding and guideline availability were recorded. CONCLUSIONS: This study provides comprehensive epidemiological data on malignancies in IEI/PIDs, highlighting the need for tailored screening and management. Survey results emphasize the real-world challenges and support the development of IEI/PID-specific oncological surveillance guidelines and treatment strategies.
  • Machine learning–assisted diagnosis classification of primary immune dysregulation using IDDA2.1 phenotype profiling
    Malte Schwitzkowski, Sai Pavan Kumar Veeranki, Benedikt N. Seidel, Gerhard Kindle, Stephan Rusch, Dalia Abd Elaziz, Sohilla Lofty M. Abdelkader, Avigaelle Abitbol, Hassan Abolhassani, Lalash Abrahim, Mohamed Abuzakouk, Pietro Andrea Accardo, Nabila Achir Moussouni, Veronica Afonso, Philipp Agyeman, Martina Ahlmann, Alessandro Aiuti, Abla Akl, Güzide Aksu, Kim Albers, Michael H. Albert, Meda Diana Alecsandru, Olga Aleinikova, Svetlana Aleshkevich, Radwa Salah Eldeen Youssif Alkady, Luis Allende, Mickaël Alligon, Zoe Allwood, Manrique de Lara Laia Alsina, Daniela Ambrosch-Barsoumian, Lisa Ameshofer, Kenza Amour, Evrim Anadol, Ariharan Ananthachagaran, Chantal Andriamanga, Julia Andris, Karin Andritschke, Federica Angelini, Tobias Ankermann, Katrin Apel, Siamak Arami, Ömür Ardeniz, Peter Arkwright, Karina Arnold, Rudolf Ascherl, Najla Assam, Nurit Assia-Batzir, Faranaz Atschekzei, Sybille Aumann, Volker Aumann, Magnus Aurivillius, Bernd Ausserer, Tadej Avcin, Sezin Aydemir, Emel Aygören-Pürsün, Cagdas AyvazDeniz, Chiara Azzari, Lo Babacar, Perrine Bach, Sophie Bachmann, Peter Bader, Shahrzad Bakhtiar, Renate Bancé, Catherine Bangs, Katerina Barfusz, Safa Baris, B. BarlanIsil, Michaela Bartsch, Lucia Augusta Baselli, Laura Batlle-Maso, Helge Baumann, Ulrich Baumann, Veronika Baumeister, Helen Baxendale, Suzanne Bazen, Beatrice Beaurain, Julien Beauté, Mounia Bechar-Makhloufi, Norbert Beck, Brigitta Becker, Christian Becker, Uta Behrends, Renata Beider, Rita Beier, Amel Belkacem, Luisa Belke, Sven Bellert, Bernd H. 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Cant, Nathan Cantoni, Brindusa Capilna, Fabiola Caracseghi, Isabel Caragol, Javier Carbone, Maria Carrabba, Jean-Laurent Casanova, Latanya Chamberlain, Anita Chandra, Christophe Chantrain, Helen Chapel, Julie Chassot, Ronnie Chee, Matteo Chinello, Charu Chopra, Zita Chovancova, Martin Christmann, Krystyna Chrzanowska, Peter Ciznar, Karlien Claes, Carl Friedrich Classen, Martin Classen, Alexis-Virgil Cochino, Alison M. Condliffe, Selim Corbacioglu, Ana Isabel Cordeiro, Donatienne Cordier Wynar, Claire Core, Laurence Costes, Tanya Coulter, Virginie Courteille, Maria Cristina, Maria Cucuruz, Leonik Nel Dabrowska, Nel Dabrowska Leonik, Mandy Dähling, Mary Louise Daly, Claudia-Sabrina Daniel, Maria Giovanna Danieli, James Darroch, Graham Davies, Javier De Gracia Roldan, Narimene de Nadai, Iris de Schutter, Nathalie De Vergnes, Esther de Vries, Josine de Witte, Frans deBaets, Theo Debert, Christiane DeBoeck, Corina Defila, Judith Deimel, Diane Delaplace, Rosa Maria Dellepiane, Nelli Dellert, Laura Delliera, Anita Delor, Ulrike Demel, John Dempster, M.M. den Os, Rosmarie Dengg, Sora Asfaw Desisa, Alexandra Desta, Drahomira Detkova, Maite Dewerchin, Romina Dieli Crimi, Dagmar Dilloo, Florentia Dimitriou, Sarah Svenja Dinges, Jasmin Dinser, Nabila Dipani, Johannes Dirks, Anna-Maria Dittrich, Lylia Djermane, Yagmur Dogru, Figen Dogu Esin, Angelika Dombrowski, Julia Dominguez Escobar, Michaela Döring, Camilla Heldbjerg Drabe, Susann Drerup, Barbara Drexel, G.J.A. Driessen, Gregor Dückers, Yasmine Dudoit, Andrea Duppenthaler, Georg Ebetsberger-Dachs, Mate Barnabas Ecser, J. David Edgar, Maartje Eekman, Stephan Ehl, Rosanna Ehrat, Eva Eisl, Olov Ekwall, Rabab El Hawary, Sabine M. El-Helou, Aisha El-Marsafy, Sarina Elbe, Suzanne Elcombe, Alia Eldash, Youssif Alkady Radwa Salah Eldeen, P.M. Ellerbroek, Roland Elling, Jane Elliott, Aisha Elmarsafy, Angelika Engelhardt, Diana Ernst, Fügen Ersoy, Stefanie Esper, Isabel Esteves, Amos Etzioni, Andrew Exley, Martin Faber, Giovanna Fabio, Gaby Fahrni, Laura Eva Faletti, Laura Eva Faletti, Claire-Michele Farber, João Farela Neves, Emilia Faria, Henriette Farkas, Evangelia Farmaki, Maria Faßhauer, Anders Fasth, Gerd Fätkenheuer, Stefanie Faustmann, Gisela Fecker, Conleth Feighery, Cornelia Feiterna-Sperling, Perez Eduardo Fernandez-Cruz, Cordeiro Ferreira Concalo, Alice Ferster, Tobias Feuchtinger, Oliver Feyen, Daniela Finke, Alain Fischer, Sigrid Fitter, Stefan Flaschberger, Lucia Fleckenstein, Dirk Föll, Adriano Fontana, Concetta Forino, Elisabeth Förster-Waldl, Nora Franchet, Dagmar Freitag, Urs P. Frey, Hannah Margarete Frick, Elisabeth Friedel, Wilhelm Friedrich, Barbara Frisch, Lukas Frischknecht, Stephanie Fritzemeyer, Alenka Gagro, Manfred Gahr, Nermeen Mouftah Galal, Eleonora Gambineri, Agnes Gamper, Franziska Gams, Astrid Ganzow, Nicolas Garcelon, Tomaz Garcez, Marina GarciaPrat, Ann Gardulf, Janine Garibay, Birgit Garwer, Jonathan Gathmann, Benjamin Gathmann, Corinna Gebauer, Linda Geberzahn, Tilman Geikowski, Ulf Geisen, Christiane Gemander, Andrew R. Gennery, Marie Gerisch, Michael Gernert, Katrin Gerrer, Stev Gerschmann, Sujal Ghosh, Carolin Giannini, Juana Gil Herrera, Noemi Gimenez Sanz, Matthias Girndt, Ramona Girrbach, Hermann Girschick, Antonios Gkantaras, Ioannis Gkougkourelas, Dominika Gładysz, Susanne Gnatowski, Elisabeth Gnodtke, Vera Goda, Sarah Goddard, Daniela Goebel, Jean-Christophe Goffard, Sigune Goldacker, Eva Maria Gollowitsch, Manuella Gomes, Mark Gompels, Míriam González, Luis Ignacio Gonzalez Granado, Pavels Gordins, Katharina Gößling, Lisa Göschl, Lucy Gossens, Ewelina Gowin, Lea Graafen, Leo Graca, Brigita Gradauskiene-Sitkauskiene, Dagmar Graf, Norbert Graf, Elliot Grange, H.Anne Grashoff, Johann Greil, Sofia Grigoriadou, Bodo Grimbacher, Helen Gronlund, Ute Groß-Wieltsch, Teresa Guerra, Kissy Guevara-Hoyer, Mor Seny Gueye, Seny GueyeMor, Noemie Guibert, Birgit Gülnur, Tayfun Güngör, Marina Guseva, David Guzman, Marcel Haag, Gabriele Haase, Henriette Haenicke, Filomeen Haerynck, Ines Hafsa, David Hagin, Emine Haliti, Michael Hallek, Lennart Hammarstroem, Gonca Hancioglu, Rupert Handgretinger, Leif G. Hanitsch, Susanne Hansen, Tetyana Hariyan, Thomas Harrer, Pia Hassunah, Maria Hatzistilianou, Fabian Hauck, Thomas Hauser, Margje H. Haverkamp, Grant Hayman, Paul Heath, Christian Hedrich, Maximilian Heeg, Michael Heike, Martin Heimbrodt, Sabine Heine, Ulrich Heininger, Christian Heinrich, Valerie Heinz, Andreas Heitger, Matthew Helbert, Arthur Helbling, Camilla Heldbjerg Drabe, Antje Hellige, Julya Hempel, Karen Henderson, Jörg Henes, Philipp Henneke, Christian Hennig, Karin Henrichs, Martin Herbst, Walter Hermann, Manuel Hernandez, Anja Hernández, Edyta Heropolitanska-Pliszka, Richard Herriot, Friedrich Herrmann, Archana Herwadkar, Christoph Hess, Ursula Hess, Sebastian Hesse, Sonja Higgins, Anna Hilfanova, Sophie Hilpert, Chantal Hintze, Eva Hlaváčková, Isabel Hodl, Adna Hodzic, Miriam Hoernes, Christina Hoffmann, Andreas Holbro, Christiane Höllinger, Lisa Holtsch, Ursula Holzer, Dirk Holzinger, Manfred Hönig, Andrea Hönscheid, Julia Horn, Gerd Horneff, Claire Hoyoux, Nataliya Hristova, Kai Hübel, Angela Hübner, Christian Huemer, Aarnoud Huissoon, Brigitte Hülsmann, Patrick Hundsdörfer, Hans-Iko Huppertz, Kristina Huß, Sadia Hussain, Julien Husson, Tanja Hüttner-Foehlisch, Hanna Ijspeert, Aydan Ikinciogullari, Kökçü İlknur, Ninela Irga, Alexandra Jablonka, Karina Jahnz-Rozyk, Marcus Jakob, Donate Jakoby, Donate Jakoby-Gaide, Peter Jandus, Annette Jansson, Melanie Jaquet, Helene Jardefors, Edyta Jargulinska, Barbara Jauk, Milos Jesenak, Katharina Jilka, Stephen Jolles, Alison Jones, Regina Jones, Birgit Jonkman-Berk, Göran Jönsson, Hilary J. Joyce, Pricillia Juliana, Michael Kabesch, Leo Kager, Christian Kahlert, Petra Kaiser-Labusch, Ioannis Kakkas, Dirk Kamitz, Maria Kanariou, Lothar Kanz, Elif Karakoc-Aydiner, Boris Karanovic, Mutlu Kartal-Kaess, Elisabeth Käser, Terese L. Katzenstein, Hana Kayserova, Peter Kelleher, Tessa Kerre, Sara Sebnem Kilic, Gerhard Kindle, Martina Kirchner, Simone Kiwit, Ayca Kiykim, Jessica Klasen, Maja Klaudel-Dreszler, Ariane Klein, Christoph Klein, Andreas Klein-Franke, Ilona Kleine, Stefan Kleinert, Christian Klemann, Marion Klima, Adam Klocperk, Robin Kobbe, Dilara Fatma Kocacik Uygun, Melanie Koch, Yvonne Kochler, Naschla Kohistani, Marina Kojic, Antonio Kolios, Uwe Kölsch, Sylwia Koltan, Irina Kondratenko, Christoph Königs, Julia Konoplyannikova, Peter Kopac, Jana Kopp, Dieter Körholz, Julia Körholz, Pauline Korte, Larysa Kostyuchenko, Ina Kötter, Sven Kracker, Pavlina Králícková, Christof Kramm, Philipp Kramme, Máté Krausz, Wolfhart Kreuz, Johanna Krista, Gergely Kriván, Gabriele Kropshofer, Renate Krüger, Olga Krystufkova, Maria Ktistaki, Jörn-Sven Kühl, Alexander Kühn, Taco W. Kuijpers, Wietse Kuis, Silke Kullmann, Andreas Kulozik, Dinakantha Kumararatne, Ria Kümmler, Andrea Kündgen, Magdalena Kurenko-Deptuch, Cosima KussPaula, Necil Kütükcüler, Cécile Lafoix-Mignot, Beate Lamers, Peter Lanbeck, Paul Landais, Sybille Landwehr-Kenzel, Vanessa Langemeyer, Thorsten Langer, Petra Lankisch, Nadia Lanz, Manrique de Lara, Eusebia Lara-Villacanas, Lisa Laubenthal, Hans-Jürgen Laws, Ronan Leahy, Jae-Yun Lee, Andrea Lehmann, Kai Lehmberg, Patricia Lehner, Hans Leibfrit, Leoni Leistner, Loic LeMignot, Petra Lesch, Simon Leutner, Manolis Liatsis, Linda Libai Véghová, Johanna Liebel, Johannes G. Liese, Kotryna Linauskiene, Richard Linde, Martina Linßner, Conrad Ferdinand Lippert, Jiri Litzman, Pilar Llobet, Babacar Lo, Tariq Lodin, Jindrich Lokaj, David Longhino, Hilary Longhurst, Susana Lopes da Silva, Catharina Lorenzen, Vassilios Lougaris, Doris Löw, Annelie Lubatschofski, Mary Lucas, Verena Lutz-Wiegers, Sabine Maaß, Maria Elena Maccari, Anna Macura-Biegun, Vanessa Maerz, Paraskevi Maggina, Nizar Mahlaoui, Hannah Mahrenholz, Sarah Maier, Mounia Makhlouf, Anne Malfroot, Laura Malinauskiene, Wilma Mannhardt-Laakmann, Ania Manson, Felicia Mantkowski, Petra Manzey, Carolina Marasco, Nufar Marcus-Mandelblit, Wolfgang Marg, Gasper Markelj, Laszlo Marodi, José Goncalo Marques, Laura Marques, Karin Marschall, Natalia Martínez, Rafael Martinez de la Ossa Saenz-Lopez, Inmaculada Martinez-Saguer, Baldassarre Martire, Antonio Marzollo, Refiloe Masekela, Katja Masjosthusmann, Tania Nicole Masmas, Nuria Matamoros, Jutta Mattern, Pearl Mau-Asam, Elizabeth McDermott, Nichole McIntosh, Karmen Mesko Meglic, Ruben Meijer, Andrea Meinhardt, Safa Meshaal, Yasmina Messaoud, Björn Meyer, Dirk Meyer-Olson, Isabelle Meyts, Romain Micol, Bozena Micoloc, Gudrun Mielke, Cinzia Milito, Tomas Milota, Siraj Misbah, Carolin Mödden, Michael Mohr, Karina Mohrmann, Mostafa Moin, Luis Molinos, Jana Möller, Sarah Möller-Nehring, Henner Morbach, Viviana Moschese, Olga Moser, Despina Moshous, Radoslaw Motkowski, Jayashree Motwani, Nermeen MouftahGalal, Carmen Müglich, Anna Mukhina, Eva Muller, Christiane Müller, Gabriele Müller, Hedi Müller, Ingo Müller, Thomas Müller, Zoe Müller, Ulf Müller-Ladner, Sarah Müller-Stöver, Esther Münstermann, Núria Murtra Garrell, Moritz Muschaweck, Miriam Mutert, Zohreh Nademi, Paru Naik, Andrea Näke, Andrea Martin Nalda, Gulnara Nasrullayeva, Nora Naumann-Bartsch, Verena Nemitz, Olaf Neth, Andreas Neubauer, Jennifer Neubert, Carla Neumann, Tim Niehues, Mara Niemuth, Alexandra Nieters, Chris Nieuwhof, Daniela Nolkemper, Sadia Noorani, Budde Adya Noorlander, Luigi D. Notarangelo, Gundula Notheis, Sanam Amelie Nowatsh, Gaelle Obenga, Suheyla Ocak, Mehmet Oker, Peter Olbrich, Anna Olipra, Heymut Omran, Prasad Oommen, Linda Opitz, Jaroslava Orosova, Solveig Oskarsdottir, Hülya Özsahin, Malgorzata Pac, Jana Pachlopnik-Schmid, Franco Pandolfi, Efimia Papadopoulou-Alataki, Theodora Papastamatiou, Anna Papatriantafillou-Schmieder, Alba Parra-Martinez, Olga Paschenko, Srdjan Pašić, Jarek Pasnik, Smita Patel, Martin Pavlík, Estela Paz Artal, Anouk Peeters, Sara Branco Pereira da Silva, Ruy Perez-Becker, Marc Perez-Guzman, Martine Pergent, Markus Perlhagen, Hans-Hartmut Peter, Marin Petrić, Michael Pfreundschuh, Pierre Philippet, Capucine Picard, Barbara Pietrucha, Leonora Pietzsch, Claudio Pignata, Monica Piquer Gibert, Kerstin Pirolt, Alessandro Plebani, Daniel E. Pleguezuelo, Katrin Pollok, Helena Pommerening, Daniela Popihn, Aleksandra Poplonek, Marina Popp, Fulvio Porta, Jan Portegys, Klara Posfay-Barbe, Judith Potjewijd, Sebastian Poulheim, Seraina Prader, Petra Prämassing-Scherzer, Martina Prelog, Johan Prevot, Arthur Price, Timothy Price, Marijke Proesmans, Johan Provot, Federica Pulvirenti, Isabella Quinti, Anna Raab, Anita Rack, Stefan Raffac, Eduardo Ramos Oviedo, Philippe Randrianomenjanahary, Anja Ranohavimparany, Maria Raptaki, Roonaka Rashidzadeh, Margit Rathwallner, Shereen Reda, Nahida Redouane, Frederico S. Regateiro, Janine Reichenbach, Bianca Reimers, Cornelia Reinhardt, Dirk Reinhardt, Anne Reinprecht, Tamara Reiß, Ismail Reisli, Eleonore Renner, Nima Rezaei, Alex Richter, Darko Richter, Nikolaus Peter Rieber, Nadja Rieckehr, Marion Riedel, Heidi Riescher, Johannes Rischewski, Nicole Ristl, Henrike Ritterbusch, Tanja Ritz, Francois Rivier, Peter Robinson, Jürgen K. Rockstroh, Joachim Roesler, Himatur Rofiah, Elizabeth Rogerson, Elisabeth Rolfes, Beate Roller, Yaryna Romanyshyn, Roberto Rondelli, Fien Roosens, Angela Rösen-Wolff, Valentina Rösler, Johannes Roth, Tobias Rothoeft, Agathe Roubertie, Gesa Rübsam, Stephan Rusch, Abraham Rutgers, Paul Ryan, Gudrun Sach, Kambis Sadeghi, Ulrike Sahrbacher, Angelika Saidi, Özden Sanal Tezcan, Silvia Sanchez-Ramon, Juan Luis Santos, Ravishankar Sargur, Ihor Savchak, Sinisa Savic, Bernhard Schaaf, Marzena Schaefer, Christina Schäfe, Eva Scharbatke, Ellen Schatorje, Uwe Schauer, Carmen Scheibenbogen, Raphael Scheible, Clemens Scheinecker, Romana Schiller, Beatrice Schilling, Freimut Schilling, Steffi Schlieben, Thilo Schmalbach, Marc Thomas Schmalzing, Pirmin Schmid, Nadine Schmidt, Reinhold Ernst Schmidt, Monika Schmitz, Dominik Schneider, Dominik T. Schneider, Reinhard Schneppenheim, Cathy Scholtes, E.H. Schölvinck, Stefan Schönberger, Stefan Schreiber, Rik Schrijvers, Andrea Schroll, Simone Schruhl, Johanna Schrum, Ralf Schubert, Catharina Schuetz, Sebastian Schuh, Ansgar Schulz, Claudia Schulz, Ilka Schulze, Hendrik Schulze-Koops, Ulf Schulze-Sturm, Eva-Maria Schumacher, Elvira Schürmann, Gesine Schürmann, Volker Schuster, Eva Schwaneck, Klaus Schwarz, Tobias Schwarz, Carolynne Schwarze-Zander, Lothar Schweigerer, Anna Sediva, Jörg Seebach, Reinhard Seger, Florian Segerer, Markus G. Seidel, Barbara Selle, Suranjith Seneviratne, Mikko Seppänen, Hatidje Shabanaj, Svetlana Sharapova, Anna Shcherbina, Benjamin Shillitoe, Anne Siepelmeyer, Kathrin Siepermann, Anna Simon, Arne Simon, Katja Simon-Klingenstein, Marija Simonovic, Merlin Simsen-Baratault, Elena Sindram, Alla Skapenko, Maiken Skarke, Malgorzata Skomska-Pawliszak, Mary Slatter, Julie Smet, C. I. Edvard Smith, Ali Sobh, Bettina Sobik, Georgios Sogkas, Michael Sohm, Xavier Solanich, Xavier Solanich-Moreno, Pere Soler Palacín, Franz Sollinger, Raz Somech, Anja Sonnenschein, Annarosa Soresina, Marijke Sornsakrin, Stavrieta Soura, Sabrina Spaccarotella, Guiseppe Spadaro, Monika Sparber-Sauer, Christof Specker, Carsten Speckmann, Lisa Speidel, Matthaios Speletas, Klaus-Daniel Stachel, Catherine Stadon, Aurélie Stanislas, Cynthia Stapornwongkul, Paulina Staus, Regina Steck, Cathal Steele, Herbert Steffin, Urs Steiner, Sandra Steinmann, Wim Stevens, Martina Stiefel, Sarah Stieger, Sophie Stiehler, Hermann Stimm, Silvia Stojanov, Matthias Stoll, Dominique Stoppa-Lyonnet, Tobias Strapatsas, Gabriele Strauß, Monika Streiter, Riet Strik-Albers, Gaby Strotmann, Héctor SuárezCasado, Jose Luis Subiza, Mikael Sundin, Birgit Süß, Fabienne Sutter, Anna Szaflarska, Monika Szemkus, Hannah Tamary, Sofia Tantou, Michael D. Tarzi, Helga Taschner, Ulf Tedgard, Carla Teixeira, RJM ten Berge, Klaus Tenbrock, Sabine Tester, Ilhan Tezcan, Sonja Thalguter, Julian Thalhammer, Katharina Thoma, Moira Thomas, Fabian Thomczyk, Vojtech Thon, Adrian Thrasher, Patricia Tierney, Nadine Tietsch, Alberto Tommasini, Beate Tönnes, Hans-Peter Tony, Maria Trachana, Carmen Trapp, Lourdes Tricas, Maria Conceicao Trindade Neves, Jordis Trischler, Hugo Ubieto, Anett Uelzen, Annette Uhlmann, Jan Ullrich, Kurt Ullrich, Ekrem Ünal, Gerhard Urbanski, Simon Urschel, Aleksandra Uszynska, Angelo Vacca, N.N. Vaganov, Daniel Vagedes, Stefanie Valicevic, Florence Vallelian, Rachel T. van Beem, Charlotte van Damme, Annick van de Ven, J. Merlijn van den Berg, Michiel van der Flier, J.T. van Dissel, P.M. van Hagen, J.M. van Montfrans, Geoffrey van Ogtrop, Jacqui van Rens, Christel A.M.P. van Riel, Annet van Royen-Kerkhof, G.Th.J. van Well, Antari Vasiliki, Sirje Velbri, Jo Vencken, Alessandro Ventura, François Vermeulen, Christiane Vermylen, Dorothee Viemann, Anja Viereck, Anna Villa, Jeroen Vincke, Lisa Vinnemeier-Laubenthal, Kim Duy Vo Thi, Mirjam Voeller, Kristina Vollbach, Alla Volokha, Horst von Bernuth, Philipp von Bismarck, Rebecca Voss, Sandra Voß, Yüksel Vural, Heike Wachuga, Norbert Wagner, Per Wagström, Matthias Wahle, Volker Wahn, Nadine Wapp, Klaus Warnatz, Monika Warneke, Adilia Warris, Jan-Christian Wasmuth, Jean-Blaise Wasserfallen, Angela Wawer, Manfred Weber, Corrie MR. Weemaes, Lisa Wege, Julius Wehrle, Stephan Weidinger, Michael Weiß, Elisabeth Weißbarth-Riedel, Antje Werner, Sara Wessel, Marco Westkemper, Monika Wicher, Lutz Wickmann, Sabine Wiegert, Monique Wiehe, Katharina Wiehler, Lydia Wiesböck, Ewa Wiesik-Szewczyk, Anthony Williams, Beate Winkler, Christel Winkler, Martina Winkler, Melanie Winkler, Uwe Wintergerst, Lukas Wisgrill, Torsten Witte, Helmut Wittkowski, Barbara Wolf, Sandra Wölke, Christina Wolschner, Beata Wolska-Kusnierz, Philip Wood, Sarita Workman, Austen Worth, Michaela Wortmann, Walter Alfred Wuillemin, Nico M. Wulffraat, Katharina Wustrau, Chloé Wyndham-Thomas, Olcay Yegin, Alisan Yildiran, Denise Yilmaz, Patrick Young, Esra Yucel, Milica Zečević, Erić Želimir, Fred Zepp, Klaus Zetzsche, Rainald Zeuner, Stefan Zielen, Martina Zimmermann, Diether Kramer, Markus G. Seidel
    Journal of Allergy and Clinical Immunology, 2026
    BACKGROUND: Immune dysregulation, including autoimmunity, autoinflammation, allergy, and malignancy predisposition, adds significant disease burden in primary immune disorders (PID) and inborn errors of immunity (IEIs). OBJECTIVE: We evaluated whether the 5-graded immune deficiency and dysregulation activity (IDDA2.1) score, encompassing 21 organ involvement and disease burden parameters, supports diagnosis across a wide spectrum of IEIs. METHODS: From April 2022 to November 2024, collaborators from 84 centers collected 1,043 IDDA score datasets from 825 patients across 89 IEIs (17 disorders with ≥10 patients each; range, 1-196 per IEI), including 177 scores from 141 treated patients. Supervised machine learning models (k-nearest neighbors, support vector machine, logistic regression, random forest) classified patients into disease groups and ranked corresponding predictive features, while unsupervised uniform manifold approximation and projection (UMAP) visualized disease-specific clustering. RESULTS: Feature analysis reflected clinicians' recognition of IEI patterns and confirmed internal IDDA score consistency. Phenotype profiles in treated patients remained informative, inversely reflecting anticipated treatment-dependent phenotype amelioration. UMAP effectively distinguished IEIs by IDDA2.1 profiles. Genetic disorder prediction achieved 73% overall accuracy, 70% for the correct monogenic IEI, and 93% within the top 3 predictions; classification reached 43% for IEI-International Union of Immunological Society categories and 59% for 12 "cardinal" IEIs (25 genes). CONCLUSIONS: Random forest feature importance analysis can inform targeted clinical screening for key disease manifestations. The top 3 prediction approach demonstrates diagnostic potential, but improved accuracy will require larger, globally shared datasets. Small sample sizes for rare diseases highlight the necessity of broader collaboration to enhance AI-assisted clinical decision-making in the future.
  • International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema
    Henriette Farkas, Inmaculada Martinez‐Saguer, Konrad Bork, Anastasios E. Germenis, Anete S. Grumach, Hanga Réka Horváth, Andrea Luczay, Andrea Zanichelli, Markus Magerl, Stephen Betschel, Emel Aygören‐Pürsün, Jonathan A. Bernstein, Isabelle Boccon‐Gibod, Teresa Caballero, Mauro Cancian, Sandra Christiansen, Danny M. Cohn, Francisco Contreras, Sansanee Craig, Camelia Isaic, Ankur Jindal, Constance H. Katelaris, Hilary J. Longhurst, Andrew MacGinnitie, Jonny Peter, Grzegorz Porebski, Avner Reshef, Dinh Van Nguyen, Bruce Zuraw, Anthony J. Castaldo, Henrik Balle Boysen, Timothy Craig, and
    Allergy European Journal of Allergy and Clinical Immunology, 2026
    Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life‐threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age‐specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence‐based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up‐to‐date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.
  • Bronchiectasis, Low IgG Levels and Lack of Vaccination are Risk Factors for Covid-19 Hospitalization in X-linked Agammaglobulinemia – A Retrospective Multicenter Study
    Caroline Stenlander, Hannes Lindahl, Emelie Wahren-Borgström, Christoph B. Geier, Anna Sediva, Børre Fevang, Cinzia Milito, Cláudia Varandas, Cristina Roca-Oporto, Federica Pulvirenti, Isabel Hodl, Ivana Malkusova, Jacques G. Rivière, Jiri Litzman, Jolan E. Walter, Leif G. Hanitsch, Olaf Neth, Pavlina Kralickova, Rahim Miller, Serena Shaffren, Susana L. Silva, Terese Katzenstein, Timi Martelius, Urs C. Steiner, C. I. Edvard Smith, Klaus Warnatz, Peter Bergman
    Journal of Clinical Immunology, 2025
    X-linked agammaglobulinemia (XLA) is caused by loss-of-function variants in Bruton’s tyrosine kinase, leading to absence of circulating B lymphocytes and inability to produce antibodies. Despite the fear that patients with XLA would be at high risk for severe infection when the novel virus SARS-CoV-2 emerged in the society with low pre-existing immunity, most patients with XLA did not suffer from severe disease. However, some patients were critically affected. Factors associated with hospitalization in patients with XLA remain poorly described. Thus, we designed a study to determine risk factors associated with hospitalization due to Covid-19 in patients with XLA. Data was collected from 17 sites in Europe and the US, comprising n = 81 patients, with hospitalization due to SARS-CoV-2 infection in 14 patients. Nearly 17% of patients with XLA required hospitalization due to Covid-19, but only 3 patients had ventilatory support. After correcting for the effect of the date of infection during the early pandemic, univariate and multiple logistic regression analysis showed that preexisting bronchiectasis and lower IgG serum trough levels (&lt; 8 g/L) before infection were associated with an increased risk for hospitalization, with a high rate of superinfection. The lack of vaccination seemed to contribute to this risk, and ambulatory patients had higher amounts of CD4 + T cells before infection compared to hospitalized patients. Thus, our data suggests a need for IgG trough levels above 8 g/L, especially in patients with bronchiectasis, to protect patients with XLA during viral infections such as Covid-19 and reduce morbidity due to superinfections.
  • Correction to: Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis (Journal of Clinical Immunology, (2025), 45, 1, (125), 10.1007/s10875-025-01904-z)
    Maartje Blom, Annelotte J. Duintjer, Mahnaz Jamee, Melanie de Gier, Markéta Bloomfield, Adam Klocperk, Pavlina Kralickova, Neslihan E. Karaca, Oksana Boyarchuk, Peter Čižnár, Miloš Jeseňák, Svetlana Sharapova, Ekaterina Skopovets, Luis I. Gonzalez-Granado, Serena Palmeri, Stefano Volpi, Andrea Martin Nalda, Sonia Rodriguez Tello, Pere Soler-Palacín, Hassan Abolhassani, Federica Pulvirenti, Bianca Cinicola, Uwe Wintergerst, Godelieve J. de Bree, J. Merlijn van den Berg, Helen L. Leavis, Clementien Vermont, Virgil A.S.H. Dalm, Koen van Aerde, Stefanie Henriet, Hetty Jolink, Judith Potjewijd, Arjan Lankester, Chandoshi Rhea Mukherjee, Dagmar Berghuis, Małgorzata Pac, Benjamin M.J. Shillitoe, Andrew R. Gennery, Mirjam van der Burg
    Journal of Clinical Immunology, 2025
    The original version of this article had Pere Soler-Palacín’s name formatted incorrectly. It should be “Soler-Palacín P.” instead of “Palacin P.S.“. Correct Formatting: From: &lt; GivenName &gt; Pere &lt; GivenName &gt; Soler &lt; FamilyName &gt; Palacín To: &lt; GivenName &gt; Pere &lt; FamilyName &gt; Soler-Palacín The original version has been corrected.
  • Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis
    Maartje Blom, Annelotte J. Duintjer, Mahnaz Jamee, Melanie de Gier, Markéta Bloomfield, Adam Klocperk, Pavlina Kralickova, Neslihan E. Karaca, Oksana Boyarchuk, Peter Čižnár, Miloš Jeseňák, Svetlana Sharapova, Ekaterina Skopovets, Luis I. Gonzalez-Granado, Serena Palmeri, Stefano Volpi, Andrea Martin Nalda, Sonia Rodriguez Tello, Pere Soler Palacín, Hassan Abolhassani, Federica Pulvirenti, Bianca Cinicola, Uwe Wintergerst, Godelieve J. de Bree, J. Merlijn van den Berg, Helen L. Leavis, Clementien Vermont, Virgil A.S.H. Dalm, Koen van Aerde, Stefanie Henriet, Hetty Jolink, Judith Potjewijd, Arjan Lankester, Chandoshi Rhea Mukherjee, Dagmar Berghuis, Małgorzata Pac, Benjamin M.J. Shillitoe, Andrew R. Gennery, Mirjam van der Burg
    Journal of Clinical Immunology, 2025
    Purpose Patients with (X-linked) agammaglobulinemia (XLA) suffer from severe, recurrent infections potentially leading to life-threatening complications such as sepsis, meningoencephalitis and chronic lung disease. Early diagnosis and timely treatment can prevent infections and secondary complications, emphasizing a role for early detection of XLA via newborn screening (NBS). Our international multicenter survey study aimed to evaluate self-reported outcomes and parental perspectives in XLA patients to determine whether an early diagnosis is associated with better quality of life (QoL). Methods QoL-questionnaires included the PedsQL for children and SF-36, CVID_QOL, PADQOL-16 for adults. A new questionnaire was specifically developed for parents about an early diagnosis of XLA. Results In total, 88 adult and 65 pediatric XLA patients, and 69 parents from 14 countries completed the survey. Patients with an early diagnosis reported less severe, recurrent infections and less hospitalization (p &lt; 0.05). QoL was significantly lower in multiple health domains for pediatric and adult patients with a late diagnosis compared to the general population. Patients with an early diagnosis reported similar QoL outcomes compared to the general population. Parents showed immense support for NBS for XLA stating that an early diagnosis prevents emotional insecurity, health damage, unnecessary diagnostics and allows early access to medical care and informed family planning. Conclusion Our study has shown supportive evidence to pursue an early diagnosis of XLA from both a self-reported clinical, health related QoL and parental perspective. The main plea from patients and parents is to achieve an early diagnosis for XLA and severe B-lymphocyte deficiencies with NBS.
  • Recommendations for Transitioning Young People with Primary Immunodeficiency Disorders and Autoinflammatory Diseases to Adult Care
    Muskan Israni, Eliska Alderson, Nizar Mahlaoui, Laura Obici, Linda Rossi-Semerano, Helen Lachmann, Mojca Zajc Avramovič, Aurelien Guffroy, Virgil Dalm, Rachel Rimmer, Leire Solis, Carlota Villar, Andrew R. Gennery, Stephanie Skeffington, Julia Nordin, Klaus Warnatz, Anne-Sophie Korganow, Jordi Antón, Marco Cattalini, Stefan Berg, Pere Soler-Palacin, Mari Campbell, Siobhan O. Burns, , Ann Gardulf, Anna Sediva, Pere Soler-Palacín, Fabian Hauck, Isabelle Meyts, Filomeen Haerynck, Malgorzata Pac, Margarita Velcheva, Peter Jandus, Martine Pergent, Maria Carrabba, Bénédicte Neven, , Luis Ignacio Gonzalez-Granado, Isabelle Meyts, Efimia Alataki-Papadopoulou, Reem Elfeky, Jadranka Kelečić, Jutte van der Werff ten Bosch, Zahir Amoura, Vanda Friman, Carsten Heilmann, Niall Conlan, Kristiina Aalto, Judith Sanchez-Manubens, Vito Sabato, Mikko Seppänen, Xavier Solanich, Antonella Insalaco, Rainald Zeuner, Alberto Tommasini, Elissaveta Naumova, Claudia Blattmann, Mary Slatter, Jiří Litzman, Pierre Philippet, Fabio Candotti, Olov Ekwall, Pavlina Kralickova, Markus Seidel, Clementina Canessa, Rik Schrijvers, Natasha Prescott, Ursula Holzer, Carmen Carreras, Maria Kanariou, Miloš Jeseňák, Constantinos Pitsios, Lisa Devlin, Peter Arkwright, Nico Wulffraat, Charalampia Papadopoulou, Bénédicte Neven, Lucia Baselli, Rosa Maria Dellepiane, Sinisa Savic, Nicholas Brodszki, Laia Alsina, Felipe Suarez, Viviana Moschese, Peter Jandus, Trine Hyrup Mogensen, Bjorn Runar Ludviksson, Elizabeth McDermott, Ruth Fritsch-Stork, Federica Barzaghi, Brindusa Capilna, Marina Folkers, Mary Keogan, Tim Niehues, Benson Ogunjimi, Maria Koliou, Guillaume Lefèvre, Stephen Jolles, Ana Méndez-Echevarría, Ewa Bernatowska, Rosie Hague, Jean-Christophe Goffard, Olivier Gilliaux, Saul Faust, Patricia Luck, Carine Wouters, Suzanne Elcombe, Dirk Holzinger, Maria Carrabba, Manuel Santamaria, Leif Hanitsch, Paul Brogan, Troels Herlin, Horst von Bernuth, Giovanna Fabio, Véronique Hentgen, Hanne Marquart, Susana Lopes da Silva, Terese Katzenstein, Tania Nicole Masmas, Olaf Neth, Filomeen Haerynck, Joke Dehoorne, Tania Amin, Georgia Hayward
    Journal of Clinical Immunology, 2025
    Purpose Significant improvements in the prognosis for young patients with Primary Immunodeficiency Diseases (PID) and Autoinflammatory Disorders (AID), which together make up the majority of Inborn Errors of Immunity (IEI), have resulted in the need for optimisation of transition and transfer of care to adult services. Effective transition is crucial to improve health outcomes and treatment compliance among patients. Evaluations of existing transition programmes in European health centres identified the absence of disease-specific transition guidelines for PID and AID, as a challenge to the transition process. This research aimed to establish expert consensus statements for the transition of young patients with PID and AID to adult services. Methods This project used the Delphi method to establish mutual agreement for the proposed recommendations. A draft set of statements was developed following a literature review of existing transition programmes. Then the ERN RITA Transition Working Group convened to review the drafted recommendations and develop them into a survey. This survey was circulated among healthcare professionals to determine consensus using a five-point Likert scale, with the level of agreement set to 80% or greater. Statements that did not reach consensus were revised by the Working Group and recirculated among respondents. Results The initial survey received 93 responses from 68 centres across 23 countries, while the following survey outlining revised recommendations received 66 responses. The respondents agreed upon recommendations detailing the structure and administration of transition programmes, collaborative working with social systems, and contraindications to transfer of care. Conclusion This paper sets out a comprehensive set of recommendations to optimise transitional care for PID and AID.
  • Gastrointestinal disorders in patients with common variable immunodeficiency
    Pavlína Králíčková, Štěpán Šembera, Kateřina Kamarádová, Ilja Tachecí
    Gastroenterologie A Hepatologie, 2025
    Summary: Common variable immunodefi ciency (CVID) is a group of inherited antibody immune disorders. Patients often suff er from recurrent bacterial infections, autoimmune or dysregulatory complications, and have an increased risk of developing cancer (e. g., lymphoma, gastric cancer). The most common gastrointestinal pathogens include Helicobacter pylori, Campylobacter jejuni, and Giardia lamblia; in more severe cases, norovirus or cytomegalovirus (CMV) may cause signifi cant diarrhoea. Bacterial overgrowth syndrome is also common. CVID is associated with alterations in the microbio me, which contributes to complications in distant organs such as the lungs and liver. Non-infectious complications can aff ect the entire gastrointestinal tract. Notable examples include intestinal metaplasia of the gastric mucosa with a risk of carcinoma, enteropathy mimicking celiac dis ease, infl ammatory bowel dis ease-like colitis, nodular regenerative hyperplasia, and pre-portal hypertension. These complications typically develop slowly and subtly over time. Once malnutrition sets in, irreversible changes to the intestinal mucosa are often present. Managing CVID requires a multidisciplinary approach and regular screening. Endoscopic examinations, including repeated and multiple bio psies, are essential for accurate dia gnosis. Elastography is suitable for detecting hepatopathies. Serological blood tests are generally not helpful. Treatment is challenging and should be individualized. Immunoglobulin replacement therapy reduces the frequency of infections only. In cases of non-infectious complications, immunomodulatory therapy is required and, tailored to the patient’s genetic background, if possible. Key words: common variable immunodeficiency – endoscopy – intestinal dis eases – malabsorption syndrome – liver dis ease – microbio ta – immunomodulation
  • First report from the Czech national registry of inborn errors of immunity (2012–2025)
    Zita Chovancova, Eva Hlavackova, Roman Hakl, Tomas Milota, Pavlina Kralickova, Ivana Malkusova, Beata Hutyrova, Michaela Safarova, Jana Vydlakova, Dalibor Jilek, Jiri Novak, Helena Schneiderova, Petra Kralova, Alena Zimulova, Vitezslav Novak, Jaromir Bystron, Dita Zaveska, Vendula Latalova, Frantisek Kopriva, Milan Kasl, Vladimir Kracik, Renata Formankova, Petr Sedlacek, Karolina Vytiskova, Michal Svoboda, Hana Novakova, Jiri Litzman
    Frontiers in Immunology, 2025
    IntroductionCongenital immune system defects represent an ever-growing group of diseases characterized by increased susceptibility to infections and association with autoimmune, autoinflammatory, allergic and malignant complications. Here, we provide the first comprehensive report on inborn errors of immunity (IEIs) in Czechia based on the analysis of patient data from the Czech national registry (CzNR) of IEIs.Material and methodsThe online platform of CzNR of IEIs was established in 2012, compiling data about epidemiology, type of diagnosis, clinical and laboratory parameters, as well as the treatment of patients diagnosed with IEIs since 1981.ResultsThe total of 1,443 registered patients includes 697 males (48.3%) and 746 females (51.7%). The median age at diagnosis was 21.0 (0–86) years. The most represented group of patients was those with antibody deficiencies (788 patients; 54.6%). This was followed by complement deficiencies (242; 16.8%), combined immunodeficiencies with syndromic features (250; 17.3%), combined immunodeficiencies (55; 3.8%), congenital defects of phagocyte number, function, or both (31; 2.1%), autoinflammatory disorders (28; 1.9%), immune dysregulation diseases (24; 1.7%), intrinsic and innate immunity defects (21; 1.5%), primary immunodeficiency phenocopies (3; 0.2%), and bone marrow failure disorders (1; 0.1%). Common variable immunodeficiency (504; 34.9%), hereditary angioedema (222; 15.4%), and DiGeorge syndrome (182; 12.6%) were the most frequent diagnoses.ConclusionIn this article, we report the epidemiology of IEIs in the Czech Republic for the first time based on the CzNR of IEIs data. The prevalence of IEIs is approximately 13.2 patients per 100000 inhabitants of the Czech Republic.
  • Clinical efficacy of SARS-CoV-2 Omicron-neutralizing antibodies in immunoglobulin preparations for the treatment of agammaglobulinemia in patients with primary antibody deficiency
    Michael Karbiener, Gerhard Kindle, Isabelle Meyts, Mikko R. J. Seppänen, Fabio Candotti, Marta Kamieniak, Reinhard Ilk, Thomas R. Kreil, Markus G. Seidel, and
    Journal of Medical Virology, 2024
  • Systematic Approach Revealed SERPING1 Splicing-Affecting Variants to be Highly Represented in the Czech National HAE Cohort
    Hana Grombirikova, Viktor Bily, Premysl Soucek, Michal Kramarek, Roman Hakl, Lucie Ballonova, Barbora Ravcukova, Dita Ricna, Karolina Kozena, Lucie Kratochvilova, Marta Sobotkova, Radana Zachova, Pavel Kuklinek, Pavlina Kralickova, Irena Krcmova, Jana Hanzlikova, Martina Vachova, Olga Krystufkova, Eva Dankova, Milos Jesenak, Martina Novackova, Michal Svoboda, Jiri Litzman, Tomas Freiberger
    Journal of Clinical Immunology, 2023
  • Activated phosphoinositide 3-kinase δ syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity
    Maria Elena Maccari, Martin Wolkewitz, Charlotte Schwab, Tiziana Lorenzini, Jennifer W. Leiding, Nathalie Aladjdi, Hassan Abolhassani, Wadih Abou-Chahla, Alessandro Aiuti, Saba Azarnoush, Safa Baris, Vincent Barlogis, Federica Barzaghi, Ulrich Baumann, Marketa Bloomfield, Nadezda Bohynikova, Damien Bodet, David Boutboul, Giorgia Bucciol, Matthew S. Buckland, Siobhan O. Burns, Caterina Cancrini, Pascal Cathébras, Marina Cavazzana, Morgane Cheminant, Matteo Chinello, Peter Ciznar, Tanya I. Coulter, Maud D’Aveni, Olov Ekwall, Zelimir Eric, Efrem Eren, Anders Fasth, Pierre Frange, Benjamin Fournier, Marina Garcia-Prat, Martine Gardembas, Christoph Geier, Sujal Ghosh, Vera Goda, Lennart Hammarström, Fabian Hauck, Maximilian Heeg, Edyta Heropolitanska-Pliszka, Anna Hilfanova, Stephen Jolles, Elif Karakoc-Aydiner, Gerhard R. Kindle, Ayca Kiykim, Christian Klemann, Patra Koletsi, Sylwia Koltan, Irina Kondratenko, Julia Körholz, Renate Krüger, Eric Jeziorski, Romain Levy, Guillaume Le Guenno, Guillaume Lefevre, Vassilios Lougaris, Antonio Marzollo, Nizar Mahlaoui, Marion Malphettes, Andrea Meinhardt, Etienne Merlin, Isabelle Meyts, Tomas Milota, Fernando Moreira, Despina Moshous, Anna Mukhina, Olaf Neth, Jennifer Neubert, Benedicte Neven, Alexandra Nieters, Raphaele Nove-Josserand, Eric Oksenhendler, Ahmet Ozen, Peter Olbrich, Antoinette Perlat, Malgorzata Pac, Jana Pachlopnik Schmid, Lucia Pacillo, Alba Parra-Martinez, Olga Paschenko, Isabelle Pellier, Asena Pinar Sefer, Alessandro Plebani, Dominique Plantaz, Seraina Prader, Loic Raffray, Henrike Ritterbusch, Jacques G. Riviere, Beatrice Rivalta, Stephan Rusch, Inga Sakovich, Sinisa Savic, Raphael Scheible, Nicolas Schleinitz, Catharina Schuetz, Ansgar Schulz, Anna Sediva, Michaela Semeraro, Svetlana O. Sharapova, Anna Shcherbina, Mary A. Slatter, Georgios Sogkas, Pere Soler-Palacin, Carsten Speckmann, Jean-Louis Stephan, Felipe Suarez, Alberto Tommasini, Johannes Trück, Annette Uhlmann, Koen J. van Aerde, Joris van Montfrans, Horst von Bernuth, Klaus Warnatz, Tony Williams, Austen J.J. Worth, Winnie Ip, Capucine Picard, Emilie Catherinot, Zohreh Nademi, Bodo Grimbacher, Lisa R. Forbes Satter, Sven Kracker, Anita Chandra, Alison M. Condliffe, Stephan Ehl, Markus G. Seidel, Mikko R.J. Seppänen, Andrew Gennery, Maria G. Kanariou, Sofia Tantou, Sofia Grigoriadou, Gabriella Cericola, Leif G. Hanitsch, Carmen Scheibenbogen, Eva O. Hlaváčková, Gergely Krivan, Frances K. McGuire, Timothy Ronan Leahy, John David M. Edgar, Shahrzad Bakhtiar, Peter Bader, Geraldine Blanchard Rohner, Filomeen Haerynck, Karlien Claes, Kai Lehmberg, Ingo Müller, Susan Farmand, Maria Fasshauer, Dagmar Graf, Joao Farela Neves, Larysa Kostyuchenko, Luis Ignacio Gonzalez-Granado, Miloš Jeseňák, Maria Carrabba, Giovanna Fabio, Claudio Pignata, Giuliana Giardino, Ilknur Kökçü Karadağ, Alişan Yıldıran, Gonca Hancioglu, Pavlína Králíčková, Sandra Steinmann, Barbara Maria Pietrucha, Michael Gernert, Maarja Soomann, Torsten Witte, Adam Markocsy, Beata Wolska-Kusnierz, Philippe Randrianomenjanahary, Jérémie Rouger, Stavroula Kostaridou, Dariia V. Zabara, Yulia A. Rodina, Oksana A. Shvets
    Journal of Allergy and Clinical Immunology, 2023
  • Subsets of Eosinophils in Asthma, a Challenge for Precise Treatment
    Jakub Novosad, Irena Krčmová, Ondřej Souček, Marcela Drahošová, Vratislav Sedlák, Martina Kulířová, Pavlína Králíčková
    International Journal of Molecular Sciences, 2023
  • Severe combined immunodeficiencies
    Dita Záveská, Ilona Pospíšilová, Karin Bánszka, Tomáš Freiberger, Hana Grombiříková, Pavlína Králíčková
    Cesko Slovenska Pediatrie, 2023
  • Myeloid lineage cells evince distinct steady-state level of certain gene groups in dependence on hereditary angioedema severity
    Lucie Ballonová, Přemysl Souček, Peter Slanina, Kamila Réblová, Ondřej Zapletal, Marcela Vlková, Roman Hakl, Viktor Bíly, Hana Grombiříková, Eliška Svobodová, Petra Kulíšková, Julie Štíchová, Marta Sobotková, Radana Zachová, Jana Hanzlíková, Martina Vachová, Pavlína Králíčková, Irena Krčmová, Miloš Jeseňák, Tomáš Freiberger
    Frontiers in Genetics, 2023
  • Immunogenicity and Safety of the Spikevax® (Moderna) mRNA SARS-CoV-2 Vaccine in Patients with Primary Humoral Immunodeficiency
    Pavlina Kralickova, Karolina Jankovicova, Ilona Sejkorova, Ondrej Soucek, Katerina Koprivova, Marcela Drahosova, Ctirad Andrys, Jan Krejsek
    International Archives of Allergy and Immunology, 2022
  • Registry-based analysis of Icatibant and C1-inhibitor use in treatment of laryngeal attacks of hereditary angioedema
    Roman Hakl, Pavel Kuklínek, Marta Sobotková, Irena Krčmová, Pavlína Králíčková, Martina Vachová, Jana Hanzlíková, Martina Nováčková, Michal Svoboda, Ingrid Kováčová, Jiří Litzman
    Clinical and Experimental Allergy, 2022
  • A Multi‑Center, Open‑Label, Single‑Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency
    Manuel Santamaria, Olaf Neth, Jo A. Douglass, Gergely Krivan, Robin Kobbe, Ewa Bernatowska, Sofia Grigoriadou, Claire Bethune, Anita Chandra, Gerd Horneff, Michael Borte, Anja Sonnenschein, Pavlina Kralickova, Silvia Sánchez Ramón, Daman Langguth, Luis Ignacio Gonzalez-Granado, Laia Alsina, Montse Querolt, Rhonda Griffin, Carrie Hames, Elsa Mondou, Jeffrey Price, Ana Sanz, Jiang Lin
    Journal of Clinical Immunology, 2022
  • Correction to: A Multi-center, Open-Label, Single-Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency (Journal of Clinical Immunology, (2022), 42, 3, (500-511), 10.1007/s10875-021-01181-6)
    Manuel Santamaria, Olaf Neth, Jo A. Douglass, Gergely Krivan, Robin Kobbe, Ewa Bernatowska, Sofia Grigoriadou, Claire Bethune, Anita Chandra, Gerd Horneff, Michael Borte, Anja Sonnenschein, Pavlina Kralickova, Silvia Sánchez Ramón, Daman D. Langguth, Luis Ignacio Gonzalez‑Granado, Laia Alsina, Montse Querolt, Rhonda Griffin, Carrie Hames, Elsa Mondou, Jeffrey Price, Ana Sanz, Jiang Lin
    Journal of Clinical Immunology, 2022
  • Risk Factors for Severe COVID-19 and Hospital Admission in Patients With Inborn Errors of Immunity - Results From a Multicenter Nationwide Study
    Tomas Milota, Marta Sobotkova, Jitka Smetanova, Marketa Bloomfield, Jana Vydlakova, Zita Chovancova, Jiri Litzman, Roman Hakl, Jiri Novak, Ivana Malkusova, Jana Hanzlikova, Dalibor Jilek, Beata Hutyrova, Vitezslav Novak, Irena Krcmova, Anna Sediva, Pavlina Kralickova
    Frontiers in Immunology, 2022
  • Therapeutic options for CTLA-4 insufficiency
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