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. 2024 Dec 17;45(1):57.
doi: 10.1007/s10875-024-01838-y.

Recommendations for Transitioning Young People with Primary Immunodeficiency Disorders and Autoinflammatory Diseases to Adult Care

Muskan Israni  1 , Eliska Alderson  1 , Nizar Mahlaoui  2   3 , Laura Obici  4 , Linda Rossi-Semerano  5 , Helen Lachmann  6 , Mojca Zajc Avramovič  7 , Aurelien Guffroy  8   9 , Virgil Dalm  10   11 , Rachel Rimmer  12 , Leire Solis  13 , Carlota Villar  14 , Andrew R Gennery  15   16 , Stephanie Skeffington  17 , Julia Nordin  13 , Klaus Warnatz  18   19 , Anne-Sophie Korganow  8   9 , Jordi Antón  20 , Marco Cattalini  21 , Stefan Berg  22   23 , Pere Soler-Palacin  24   25 , Mari Campbell #  26   27 , Siobhan O Burns #  28   29 ; ESID Clinical Working Party; ERN RITA Transition Working Group Consortium
Collaborators, Affiliations

Recommendations for Transitioning Young People with Primary Immunodeficiency Disorders and Autoinflammatory Diseases to Adult Care

Muskan Israni et al. J Clin Immunol. .

Abstract

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.

Keywords: Transition; autoinflammatory diseases; inborn errors of immunity; primary immunodeficiency disorders; recommendations.

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Conflict of interest statement

Declarations. Competing Interests: MC1 has received grant support from the National Institute of Health Research, the Royal Free Charity, Primary Immunodeficiency Association, PIDUK, and IDUK, financial support to attend symposia from CSL Behring, Grifols, BPL, and Biotest, a service development grant from CSL Behring, and honoraria for speaking engagements from BioCryst, Shire and Takeda; VD has received grant support from ZonMw for studies on immunogenicity of COVID-19 vaccines in IEI patients, equipment from Pharming NV for in vitro studies, and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Pharming NV, Takeda, CSL Behring, AstraZeneca, and Pfizer; LS is employed at the International Patient Organisation for Primary Immunodeficiencies (IPOPI) who regularly receive support from a broad range of companies involved in the manufacture of immunoglobulin therapies and the field of primary immunodeficiencies (updated list available on www.ipopi.org ), and has received grants from Takeda for an intervention during the ID Masterclass on 6–7 November 2023 in Rotterdam (The Netherlands) and for a virtual presentation on Strategic Advocacy, on 13 December 2023; SB1 and PS-P have received speaking fees from Sobi to co-chair two Nordic Autoinflammatory meetings. The rest of the authors declare that they have no relevant conflicts of interest.

Figures

Fig. 1
Fig. 1
European map of the participants in the surveys
See this image and copyright information in PMC

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