Breakthroughs and challenges in conducting systematic reviews on rare diseases: implications of the ERNs Guidelines Programme

Article type
Authors
MARTÍN GÓMEZ C1, Isabel-Gómez R1, Pérez García G2, Vicente Edo M2, de Armas-Castellano A3, Trujillo-Martín M3, Godo Pla A4, Estrada Sabadell M4, Domínguez-García J5, Jausoro-Zubiaga A5, Blasco Amaro J
1Health Technology Assessment Area-AETSA, Andalusian Public Foundation Progress and Health-FPS, Sevilla, Andalucía, España; Spanish Network of Agencies for Assessing National Health System Technologies and Performance (RedETS), Spain
2Instituto Aragonés de Ciencias de la Salud, IACS, Spain; Spanish Network of Agencies for Assessing National Health System Technologies and Performance (RedETS), Spain
3Fundación Canaria Instituto de Investigación Sanitaria de Canarias (FIISC)/ Servicio de Evaluación del Servicio Canario de la Salud (SESCS), Spain; Spanish Network of Agencies for Assessing National Health System Technologies and Performance (RedETS), Spain
4Agency for Health Quality and Assessment of Catalonia (AQuAS), Ministry of Health, Government of Catalonia, Spain; Spanish Network of Agencies for Assessing National Health System Technologies and Performance (RedETS), Spain
5Osteba-Bioef, Spain; Spanish Network of Agencies for Assessing National Health System Technologies and Performance (RedETS), Spain
Abstract
"Background. The European Reference Networks (ERNs) Guidelines Programme aims to contribute to evidence-based clinical decision-making in rare diseases (RDs) through the development of clinical practice guidelines (CPGs) and other support tools. Methodologists and information specialists from 5 HTA agencies conducted systematics reviews (SRs) to address this objective. This study explores the barriers and facilitators they encountered during this process.

Methods. HTA Agencies staff contributed through an online survey. The information was collected on the number of SRs conducted, the RDs addressed, the clinical questions approached, the barriers and facilitators for each stage of the review (from defining the PICO components of the question to issuing recommendations), and the dissemination of the obtained results. A thematic analysis was conducted to identify the issues that arose at each stage of the SRs.

Results. A total of 35 RDs were addressed through 111 SRs, with clinical questions mainly focusing on diagnosis and treatment. The main barriers identified were: non-specific MesH terms associated with the conditions, non-representative abstracts and keywords, lack of relevant information reported in the articles, and data not allowing for quantitative syntheses or recommendations to be made. Facilitating aspects included having expert clinicians and patients in the working groups who were also involved in all steps of the SR, methodologists and information specialists with previous experience in RDs, and access to specific sources of RDs documents such as Orphanet's. Regarding the dissemination of results 8 SRs have been presented at international conferences, 1 SRs have already been published and 6 are under review in peer-review journals.

Conclusions. Although conducting SRs in the field of RDs is challenging, certain aspects can facilitate its accomplishment such as the collaboration with the ERNs and the use of specialised resources. Furthermore, authors of primary studies are encouraged to be more thorough when collecting data and communicating results. These actions could contribute to overcome the characteristic diagnostic and treatment delays, and to the quality of life of these patients.

Patient and public involvement. It was required that the CPG development groups within the ERNs Guidelines Programme must involve at least 2 members of the patient community."