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

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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.


Peer reviewed: True

Acknowledgements: The authors would like to thank the patient organisations Vasculitis International and Vasculitis Ireland Awareness, and especially Peter Verhoeven and Julie Power for ensuring the consideration of patient priorities and perspectives throughout the project. Thanks to Nathan Lea, Maria Christofidou and Dipak Kalra at the European Institute for Innovation through Health Data for guidance and support in data governance issues. Thanks also to Pintail Ltd and Grace Davey, and Elena Gelain at Meyer Children Hospital Florence for administrative assistance and Professor Cathal Walsh at the School of Medicine, Trinity College Dublin for statistical guidance.

Funder: French Vasculitis Study Group


epidemiology, granulomatosis with polyangiitis, quality indicators, health care, systemic vasculitis, Humans, Granulomatosis with Polyangiitis, Data Accuracy, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Microscopic Polyangiitis, Antibodies, Antineutrophil Cytoplasmic, Registries, Information Storage and Retrieval

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Ann Rheum Dis

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DFG project (441416480)
European Regional Development Fund (ERDF) (13/RC/2106_P2)
Science Foundation Ireland (11/Y/B2093, 13/RC/2106_P2)
Horizon 2020 Framework Programme (EJP RD COFUND-EJP N° 825575, K/NCB/000058)
Crafoordska Stiftelsen (20220623)
Health Research Board (203930/B/16/Z)
German Research Foundation / Deutsche Forschungsgemeinschaft (DFG-LA-1339/5-1)
CSL Vifor (IIS-NA-2021-0497)
Wellcome Trust (203930/B/16/Z)
Vetenskapsrådet (2019-00263)