RESUMEN
J Rheumatol 2024; doi: 10.3899/jrheum.2024-0097 There were incorrect footnotes in Table 2. All instances of footnote "a" in the Possible Score Range column should be an asterisk. The correct footnote should be, "Only participants with ≤ 1 missing or * additional answer category (ie, "does not currently apply to me") per domain are included for the sum score of the corresponding scale." We apologize for this error. This correction applies only to the August 15 2024 First Release. The correct table appears in the print and online issues.
RESUMEN
OBJECTIVE: The aim of this study was to develop and psychometrically validate a self-report instrument to assess (1) competencies, (2) needs, and (3) satisfaction among youth transitioning from pediatric to adult rheumatology. METHODS: The Transition-KompAZ was developed in several steps with conceptual and psychometric analyses. To test its psychometric properties, the instrument was administered to adolescents and young adults (AYAs; 16-25 years) with inflammatory rheumatic diseases before (group 1) or after (group 2) transfer to adult rheumatology. A 2-factor, higher-order confirmatory factor analysis (CFA) model was applied to test the hypothesized factor structure. Internal consistency was estimated using the approach of Raykov with the factor loadings and error variances estimated in the CFA. Spearman rank correlation coefficients were used to assess construct validity. RESULTS: The Transition-KompAZ includes the following modules: (1) competencies in transition (knowledge, self-management), (2) needs (healthcare services, information), and (3) satisfaction (general, transitional care). A total of 173 AYAs (group 1: n = 86; group 2: n = 87) from 12 rheumatology sites completed the Transition-KompAZ. It showed good model fit (comparative fit index > 0.9; Tucker-Lewis index > 0.9; weighted root mean square residual < 0.9) with good internal consistency. The instrument demonstrated moderate-to-good construct validity and good test-retest reliability. CONCLUSION: The Transition-KompAZ appears to be a reliable tool for assessing important dimensions of transition. It may support a structured and individualized transition, as well as the evaluation of transition services. However, further studies are required to assess its predictive value in terms of transfer readiness and successful transition.
RESUMEN
BACKGROUND: Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hyper-IgD Syndrome (HIDS) are genetically defined and characterized by recurrent fever episodes and inflammatory organ manifestations. Early diagnosis and early start of effective therapies control the inflammation and prevent organ damage. The PRO-KIND initiative of the German Society of Pediatric Rheumatology (GKJR) aims to harmonize the diagnosis and management of children with rheumatic diseases nationally. The task of the PRO-KIND CAPS/TRAPS/MKD/HIDS working group was to develop evidence-based, consensus diagnosis and management protocols including the first AID treat-to-target strategies. METHODS: The national CAPS/TRAPS/MKD/HIDS expert working group was established, defined its aims and conducted a comprehensive literature review synthesising the recent (2013 to 2018) published evidence including all available recommendations for diagnosis and management. General and disease-specific statements were anchored in the 2015 SHARE recommendations. An iterative expert review process discussed, adapted and refined these statements. Ultimately the GKJR membership vetted the proposed consensus statements, agreement of 80% was mandatory for inclusion. The approved statements were integrated into three disease specific consensus treatment plans (CTPs). These were developed to enable the implementation of evidence-based, standardized care into clinical practice. RESULTS: The CAPS/TRAPS/MKD/HIDS expert working group of 12 German and Austrian paediatric rheumatologists completed the evidence synthesis and modified a total of 38 statements based on the SHARE recommendation framework. In iterative reviews 36 reached the mandatory agreement threshold of 80% in the final GKJR member survey. These included 9 overarching principles and 27 disease-specific statements (7 for CAPS, 11 TRAPS, 9 MKD/HIDS). A diagnostic algorithm was established based on the synthesized evidence. Statements were integrated into diagnosis- and disease activity specific treat-to-target CTPs for CAPS, TRAPS and MKD/HIDS. CONCLUSIONS: The PRO-KIND CAPS/TRAPS/MKD/HIDS working group established the first evidence-based, actionable treat-to-target consensus treatment plans for three rare hereditary autoinflammatory diseases. These provide a path to a rapid evaluation, effective control of disease activity and tailored adjustment of therapies. Their implementation will decrease variation in care and optimize health outcomes for children with AID.