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
Department of Immunology, Royal Free London NHS Foundation Trust, London, UK.
Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
J Clin Immunol. 2024 Dec 17;45(1):57. doi: 10.1007/s10875-024-01838-y.
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.
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.
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.
This paper sets out a comprehensive set of recommendations to optimise transitional care for PID and AID.
原发性免疫缺陷病（PID）和自身炎症性疾病（AID）共同构成了大多数遗传性免疫缺陷（IEI），年轻PID和AID患者的预后有了显著改善，这使得优化向成人服务的过渡和护理移交变得必要。有效的过渡对于改善患者的健康结果和治疗依从性至关重要。对欧洲医疗中心现有过渡项目的评估发现，缺乏针对PID和AID的疾病特异性过渡指南，这对过渡过程构成了挑战。本研究旨在为PID和AID年轻患者向成人服务的过渡制定专家共识声明。
本项目采用德尔菲法就拟议的建议达成共识。在对现有过渡项目进行文献综述后，制定了一组声明草案。然后，ERN RITA过渡工作组召开会议，审查起草的建议并将其发展成一项调查。该调查在医疗专业人员中进行分发，使用五点李克特量表来确定共识，设定的同意水平为80%或更高。未达成共识的声明由工作组进行修订，并在受访者中再次分发。
初始调查收到了来自23个国家68个中心的93份回复，而概述修订建议的后续调查收到了66份回复。受访者就详细说明过渡项目的结构和管理、与社会系统的协作以及护理移交的禁忌症的建议达成了一致。
本文提出了一套全面的建议，以优化PID和AID的过渡性护理。
