Current Transition Practice for Primary Immunodeficiencies and Autoinflammatory Diseases in Europe: a RITA-ERN Survey
Muskan Israni, Bethany Nicholson, Nizar Mahlaoui, Laura Obici, Linda Rossi‐Semerano, Helen J. Lachmann, Georgia Hayward, Mojca Zajc Avramovič, Aurélien Guffroy, Virgil A. S. H. Dalm, Rachel Rimmer, Leire Solís, Carlotta Villar, Andrew R. Gennery, Stephanie Skeffington, Julia Nordin, Klaus Warnatz, Anne‐Sophie Korganow, Jordi Antón, Marco Cattalini, Tania Amin, Stephan Berg, Pere Soler‐Palacín, Siobhan O. Burns, Mari Campbell, Carine Wouters, Isabelle Meyts, Jutte E. van der Werff ten Bosch, L. Goffin, Benson Ogunjimi, O. Gilliaux, J. Kelečić, Marija Jelušić, Š. Fingerhutová, Anna Šedivá, Troels Herlin, R. J. Seppänen Mikko, Kristiina Aalto, H. Ritterbusch, Antonella Insalaco, Viviana Moschese, Alessandro Plebani, R. Cimaz, Clementina Canessa, Rosa Maria Dellepiane, Maria Carrabba, Federica Barzaghi, Jan van Laar, Nico Wulffraat, L. Marques, C. Carreras, Judith Sánchez-Manubens, Laia Alsina, Elena Seoane-Reula, A. Mendez-Echevarria, Luis I. Gonzales-Granado, M. Santamaria, Olaf Neth, Olov Ekwall, O. Brodszki, H. Hague, Liverpool Devlin, Paul Brogan, Peter D. Arkwright, A. Riordan, Liza McCann, E. McDermott, Saul N. Faust, E. Carne
Abstract
BACKGROUND: Due to the absence of curative treatments for inborn errors of immunity (IEI), children born with IEI require long-term follow-up for disease manifestations and related complications that occur over the lifespan. Effective transition from pediatric to adult services is known to significantly improve adherence to treatment and long-term outcomes. It is currently not known what transition services are available for young people with IEI in Europe. OBJECTIVE: To understand the prevalence and practice of transition services in Europe for young people with IEI, encompassing both primary immunodeficiencies (PID) and systemic autoinflammatory disorders (AID). METHODS: A survey was generated by the European Reference Network on immunodeficiency, autoinflammatory, and autoimmune diseases Transition Working Group and electronically circulated, through professional networks, to pediatric centers across Europe looking after children with IEI. RESULTS: Seventy-six responses were received from 52 centers, in 45 cities across 17 different countries. All services transitioned patients to adult services, mainly to specialist PID or AID centers, typically transferring up to ten patients to adult care each year. The transition process started at a median age of 16-18 years with transfer to the adult center occurring at a median age of 18-20 years. 75% of PID and 68% of AID centers held at least one joint appointment with pediatric and adult services prior to the transfer of care. Approximately 75% of PID and AID services reported having a defined transition process, but few centers reported national disease-specific transition guidelines to refer to. CONCLUSIONS: Transition services for children with IEI in Europe are available in many countries but lack standardized guidelines to promote best practice.