Current Transition Practice for Primary Immunodeficiencies and Autoinflammatory Diseases in Europe: a RITA-ERN Survey.

Adult Humans Child Adolescent Young Adult Europe / epidemiology Immunologic Deficiency Syndromes / diagnosis Autoimmune Diseases / diagnosis Prevalence Hereditary Autoinflammatory Diseases

Autoinflammatory diseases Network Primary immunodeficiencies Transition

Journal

Journal of clinical immunology

ISSN: 1573-2592

Titre abrégé: J Clin Immunol

Pays: Netherlands

ID NLM: 8102137

Informations de publication

Date de publication:
01 2023

Historique:

received: 07 03 2022

accepted: 07 08 2022

pubmed: 13 10 2022

medline: 18 1 2023

entrez: 12 10 2022

Statut: ppublish

Résumé

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. 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). 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. 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. Transition services for children with IEI in Europe are available in many countries but lack standardized guidelines to promote best practice.

Sections du résumé

BACKGROUND

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.

Identifiants

DOI: 10.1007/s10875-022-01345-y PMID: 36222999 PMC: PMC9840587

pubmed: 36222999

doi: 10.1007/s10875-022-01345-y

pii: 10.1007/s10875-022-01345-y

pmc: PMC9840587

doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

Pagination

206-216

Investigateurs

C Wouters (C)

I Meyts (I)

J E van der Werff Ten Bosch (JE)

L Goffin (L)

B Ogunjimi (B)

O Gilliaux (O)

J Kelecic (J)

M Jelusic (M)

Š Fingerhutová (Š)

A Sediva (A)

T Herlin (T)

R J Seppänen Mikko (RJ)

K Aalto (K)

H Ritterbusch (H)

A Insalaco (A)

V Moschese (V)

A Plebani (A)

R Cimaz (R)

C Canessa (C)

R M Dellepiane (RM)

M Carrabba (M)

F Barzaghi (F)

J A M van Laar (JAM)

N M Wulffraat (NM)

L Marques (L)

C Carreras (C)

J Sánchez-Manubens (J)

L Alsina (L)

M E Seoane Reula (ME)

A Mendez-Echevarria (A)

L I Gonzales-Granado (LI)

M Santamaria (M)

O Neth (O)

O Ekwall (O)

O Brodszki (O)

H Hague (H)

L A Devlin (LA)

P Brogan (P)

P D Arkwright (PD)

A Riordan (A)

L McCann (L)

E McDermott (E)

S N Faust (SN)

E Carne (E)

Informations de copyright

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