Data quality and patient characteristics in European ANCA associated vasculitis registries – data retrieval by federated querying

Gisslander, K. et al. (2023) Data quality and patient characteristics in European ANCA associated vasculitis registries – data retrieval by federated querying. Annals of the Rheumatic Diseases, (doi: 10.1136/ard-2023-224571) (PMID:37907255) (Early Online Publication)

Abstract

Objectives This study aims to describe the data structure and harmonisation process, explore data quality and define characteristics, treatment, and outcomes of patients across six federated antineutrophil cytoplasmic antibody-associated vasculitis (AAV) registries. Methods Through creation of the vasculitis-specific Findable, Accessible, Interoperable, Reusable, VASCulitis ontology, we harmonised the registries and enabled semantic interoperability. We assessed data quality across the domains of uniqueness, consistency, completeness and correctness. Aggregated data were retrieved using the semantic query language SPARQL Protocol and Resource Description Framework Query Language (SPARQL) and outcome rates were assessed through random effects meta-analysis. Results A total of 5282 cases of AAV were identified. Uniqueness and data-type consistency were 100% across all assessed variables. Completeness and correctness varied from 49%–100% to 60%–100%, respectively. There were 2754 (52.1%) cases classified as granulomatosis with polyangiitis (GPA), 1580 (29.9%) as microscopic polyangiitis and 937 (17.7%) as eosinophilic GPA. The pattern of organ involvement included: lung in 3281 (65.1%), ear-nose-throat in 2860 (56.7%) and kidney in 2534 (50.2%). Intravenous cyclophosphamide was used as remission induction therapy in 982 (50.7%), rituximab in 505 (17.7%) and pulsed intravenous glucocorticoid use was highly variable (11%–91%). Overall mortality and incidence rates of end-stage kidney disease were 28.8 (95% CI 19.7 to 42.2) and 24.8 (95% CI 19.7 to 31.1) per 1000 patient-years, respectively. Conclusions In the largest reported AAV cohort-study, we federated patient registries using semantic web technologies and highlighted concerns about data quality. The comparison of patient characteristics, treatment and outcomes was hampered by heterogeneous recruitment settings.

• Item Type: Articles
• Additional Information: This project has received funding from the European Union’s Horizon 2020 research and innovation programme under the EJP RD COFUND-EJP No 825575. Crafoordska stiftelsen: 20220623 (KG and AJM). Vetenskapsrådet: 2019-00263 (KG and AJM). Science Foundation Ireland 13/RC/2106_P2 and 11/Y/ B2093 (MAL). European Union’s Horizon 2020 research and innovation programme under the EJP RD: EJP-RD/I/FAIRVASC/03/2020 (K/NCB/000058) (SL, JM and KW). CSL Vifor: IIS-NA-2021-0497 (NB and MR). Fundings from the French Vasculitis Study Group who implemented and coordinated this French registry (XP and BT). DFG-project 441416480 (PL and DJ). The ADAPT SFI Centre for Digital Content Technology is funded by Science Foundation Ireland through the SFI Research Centres Programme and is cofunded under the European Regional Development Fund (ERDF) through Grant # 13/RC/2106_P2. JS is a Wellcome-HRB Irish Clinical Academic Training (ICAT) Fellow, and this work was performed within the Irish Clinical Academic Training (ICAT) Programme, supported by the Wellcome Trust and the Health Research Board (Grant Number 203930/B/16/Z), the Health Service Executive, National Doctors Training and Planning and the Health and Social Care, Research and Development Division, Northern Ireland. German Research Foundation/ Deutsche Forschungsgemeinschaft DFG-LA-1339/5-1 (PL).
• Status: Early Online Publication
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Basu, Professor Neil and Rutherford, Dr Matthew
• Authors: Gisslander, K., Rutherford, M., Aslett, L., Basu, N., Dradin, F., Hederman, L., Hrušková, Z., Kardaoui, H., Lamprecht, P., Lichołai, S., Musiał, J., O’Sullivan, D., Puéchal, X., Scott, J., Segelmark, M., Straka, R., Terrier, B., Tesař, V., Tesi, M., Vaglio, A., Wandrei, D., White, A., Wójcik, K., Yaman, B., Little, M. A., and Mohammad, A. J.
• College/School: College of Medical Veterinary and Life Sciences > School of Infection & Immunity
• Journal Name: Annals of the Rheumatic Diseases
• Publisher: BMJ Publishing Group
• ISSN: 0003-4967
• ISSN (Online): 1468-2060
• Published Online: 31 October 2023
• Copyright Holders: Copyright: © Author(s) (or their employer(s)) 2023
• First Published: First published in Annals of the Rheumatic Diseases 2023
• Publisher Policy: Reproduced under a Creative Commons licence