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Current Transition Practice for Primary Immunodeficiencies and Autoinflammatory Diseases in Europe: a RITA-ERN Survey
Muskan Israni1, Bethany Nicholson1, Nizar Mahlaoui2,3
1Department of Immunology, Royal Free London NHS Foundation Trust, London, UK.
Insights
Transition services for children with inborn errors of immunity (IEI) exist across Europe. However, these services for primary immunodeficiencies (PID) and autoinflammatory disorders (AID) often lack standardized guidelines for optimal patient care.
Area of Science:
- Pediatric Rheumatology and Immunology
- Healthcare Transition Services
- Chronic Disease Management
Background:
- Children with inborn errors of immunity (IEI) require lifelong care due to the absence of curative treatments.
- Effective transition from pediatric to adult healthcare services is crucial for improving treatment adherence and long-term outcomes in IEI patients.
- The availability and nature of transition services for young people with IEI in Europe are not well-documented.
Purpose of the Study:
- To investigate the prevalence and current practices of transition services for young individuals with IEI in Europe.
- To encompass both primary immunodeficiencies (PID) and systemic autoinflammatory disorders (AID) within the scope of the study.
- To identify existing transition models and potential gaps in European healthcare systems for IEI patients.
Main Methods:
- A survey was developed by the European Reference Network on immunodeficiency, autoinflammatory, and autoimmune diseases Transition Working Group.
- The survey was electronically distributed to pediatric centers across Europe that manage children with IEI.
- Responses were collected from 52 centers across 17 countries, providing data on transition practices.
Main Results:
- All surveyed services transitioned patients to adult care, primarily to specialized PID or AID centers.
- The transition process typically begins between ages 16-18, with transfer to adult care around ages 18-20.
- While most centers reported defined transition processes and joint appointments, standardized national guidelines were rarely available for PID and AID.
Conclusions:
- Transition services for children with IEI are established in many European countries.
- A significant need exists for standardized guidelines to ensure best practices in IEI transition care across Europe.
- Further development of national and international protocols is recommended to optimize the transition process for IEI patients.
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.
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