Recommendations for Transitioning Young People with Primary Immunodeficiency Disorders and Autoinflammatory Diseases to Adult Care
Authors: Israni Muskan , Alderson Eliska , Mahlaoui Nizar , Obici Laura , Rossi-Semerano Linda , Lachmann Helen , Avramovič Mojca Zajc , Guffroy Aurelien , Dalm Virgil , Rimmer Rachel , Solis Leire , Villar Carlota , Gennery Andrew R. , Skeffington Stephanie , Nordin Julia , Warnatz Klaus , Korganow Anne-Sophie , Antón Jordi , Cattalini Marco , Berg Stefan , Soler-Palacin Pere , Campbell Mari , Burns Siobhan O. ,   on behalf of the ESID Clinical Working Party, Gardulf Ann , Sediva Anna , Soler-Palacín Pere , Hauck Fabian , Meyts Isabelle , Haerynck Filomeen , Pac Malgorzata , Velcheva Margarita , Jandus Peter , Pergent Martine , Carrabba Maria , Neven Bénédicte ,   the ERN RITA Transition Working Group Consortium, Gonzalez-Granado Luis Ignacio , Meyts Isabelle , Alataki-Papadopoulou Efimia , Elfeky Reem , Kelečić Jadranka , van der Werff ten Bosch Jutte , Amoura Zahir , Friman Vanda , Heilmann Carsten , Conlan Niall , Aalto Kristiina , Sanchez-Manubens Judith , Sabato Vito , Seppänen Mikko , Solanich Xavier , Insalaco Antonella , Zeuner Rainald , Tommasini Alberto , Naumova Elissaveta , Blattmann Claudia , Slatter Mary , Litzman Jiří , Philippet Pierre , Candotti Fabio , Ekwall Olov , Kralickova Pavlina , Seidel Markus , Canessa Clementina , Schrijvers Rik , Prescott Natasha , Holzer Ursula , Carreras Carmen , Kanariou Maria , Jeseňák Miloš , Pitsios Constantinos , Devlin Lisa , Arkwright Peter , Wulffraat Nico , Papadopoulou Charalampia , Neven Bénédicte , Baselli Lucia , Dellepiane Rosa Maria , Savic Sinisa , Brodszki Nicholas , Alsina Laia , Suarez Felipe , Moschese Viviana , Jandus Peter , Mogensen Trine Hyrup , Ludviksson Bjorn Runar , McDermott Elizabeth , Fritsch-Stork Ruth , Barzaghi Federica , Capilna Brindusa , Folkers Marina , Keogan Mary , Niehues Tim , Ogunjimi Benson , Koliou Maria , Lefèvre Guillaume , Jolles Stephen , Méndez-Echevarría Ana , Bernatowska Ewa , Hague Rosie , Goffard Jean-Christophe , Gilliaux Olivier , Faust Saul , Luck Patricia , Wouters Carine , Elcombe Suzanne , Holzinger Dirk , Carrabba Maria , Santamaria Manuel , Hanitsch Leif , Brogan Paul , Herlin Troels , von Bernuth Horst , Fabio Giovanna , Hentgen Véronique , Marquart Hanne , da Silva Susana Lopes , Katzenstein Terese , Masmas Tania Nicole , Neth Olaf , Haerynck Filomeen , Dehoorne Joke , Amin Tania , Hayward Georgia 
Published: December 2024

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.