Development and validation of a relapsing polychondritis disease-specific quality of life instrument (ERN ReCONNET RP-QoL)
Year: 2026
Type: article
Abstract: OBJECTIVES: Relapsing polychondritis (RP) is a rare, multisystem inflammatory disease characterized by heterogeneous clinical manifestations and a substantial impact on patients' daily functioning and well-being. Health-related quality of life (HR-QoL) in RP remains insufficiently characterized, and no disease-specific measurement tool is available so far. This study aimed to develop and validate the RP-QoL, a multilingual, patient-reported outcome instrument specifically designed to assess HR-QoL in individuals with RP. METHODS: The RP-QoL was developed within the European Reference Network ReCONNET using a structured four-step approach: (1) identification of relevant HR-QoL domains through systematic literature review, an international patient survey and expert consensus; (2) item generation; (3) pilot testing including cognitive debriefing; and (4) comprehensive psychometric validation. Validation analyses included assessment of internal consistency, structural validity, construct validity, convergent validity with the SF-36, and criterion-related validity. RESULTS: Domain identification incorporated input from 274 patients across 22 countries, leading to a 31-item pilot questionnaire with a 28-day recall period and 5-point response scale. Cognitive debriefing confirmed clarity, relevance, and feasibility. Validation in 239 patients from 19 countries (median age 55 years; 80% female) demonstrated excellent internal consistency (Cronbach's α = 0.96). Exploratory factor analysis supported near-unidimensionality, with the first factor explaining 46.6% of variance. RP-QoL scores correlated strongly with disease impact (p = -0.62, P < 0.0001) and SF-36 physical (p = 0.65) and mental (p = 0.55, P < 0.0001) components (both P < 0.0001, P < 0.0001). Discriminative ability was high (AUC = 0.87). CONCLUSION: The RP-QoL is the first validated, disease-specific HR-QoL instrument for RP, with robust psychometric performance and applicability in both clinical practice and research.
Source: Lara D. Veeken
Authors: Laurent Arnaud, Oliver Sander, Laura Damian, Cristina Pamfil, Francesca Crisafulli, Raquel Faria, Sofia Silva-Ribeiro, Jean-Charles Piette, Alexis Mathian, Marcella Ferrada, Jun Shimizu, Aman Sharma, Lou Kawka, Cédric Sztejkowski, Christina Dusing, Thomas Rose, Antonio Lamas, Carlos Vasconcelos, Paolo Semeraro, Alba-Chiara Pozzi, Teodora Neagu, Mihaela Resteu, Arola Armengou, Ana del Cielo Perez Jaen, Guillem Policarpo Torres, Hervé Devilliers, Lisa Matthews, Camelia Bucsa, Simona Rednic, Philippe Mertz
Institutions: Inserm, Hôpitaux Universitaires de Strasbourg, Düsseldorf University Hospital, Iuliu Hațieganu University of Medicine and Pharmacy, Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia, University of Brescia, Universidade do Porto, Hospital de Santo António, Unidade Local de Saúde de Santo António EPE, Sorbonne Université, Assistance Publique – Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Centre d'Immunologie et des Maladies Infectieuses, University of Maryland, Baltimore, St. Marianna University School of Medicine, Post Graduate Institute of Medical Education and Research, Heinrich Heine University Düsseldorf, Charité - Universitätsmedizin Berlin, Hospital Universitari de Girona Doctor Josep Trueta, CHU Dijon Bourgogne, Centre Hospitalier de Versailles, Hôpital Tenon
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,597
Funder: European Commission
OpenAlex ID: W7166850407
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