PMID- 37907255
OWN - NLM
STAT- MEDLINE
DCOM- 20240104
LR  - 20240210
IS  - 1468-2060 (Electronic)
IS  - 0003-4967 (Print)
IS  - 0003-4967 (Linking)
VI  - 83
IP  - 1
DP  - 2024 Jan 2
TI  - Data quality and patient characteristics in European ANCA-associated vasculitis 
      registries: data retrieval by federated querying.
PG  - 112-120
LID - 10.1136/ard-2023-224571 [doi]
AB  - 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.
CI  - (c) Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published 
      by BMJ.
FAU - Gisslander, Karl
AU  - Gisslander K
AUID- ORCID: 0000-0003-1915-2656
AD  - Clinical Sciences, Rheumatology, Lund University, Lund, Sweden 
      karl.gisslander@med.lu.se.
FAU - Rutherford, Matthew
AU  - Rutherford M
AUID- ORCID: 0000-0001-5626-2470
AD  - School of Infection and Immunity, University of Glasgow, Glasgow, UK.
FAU - Aslett, Louis
AU  - Aslett L
AUID- ORCID: 0000-0003-2211-233X
AD  - Department of Mathematical Science, University of Durham, Durham, UK.
FAU - Basu, Neil
AU  - Basu N
AUID- ORCID: 0000-0003-4246-3145
AD  - School of Infection and Immunity, University of Glasgow, Glasgow, UK.
FAU - Dradin, Francois
AU  - Dradin F
AUID- ORCID: 0009-0009-3377-0996
AD  - Telemedicine Technologies, Liege, Belgium.
FAU - Hederman, Lucy
AU  - Hederman L
AUID- ORCID: 0000-0001-6073-4063
AD  - ADAPT SFI Centre, School of Computer Science and Statistics, Trinity College 
      Dublin, Dublin, Ireland.
FAU - Hruskova, Zdenka
AU  - Hruskova Z
AUID- ORCID: 0000-0002-2658-365X
AD  - Department of Nephrology, General University Hospital, Prague, Czech Republic.
AD  - First Faculty of Medicine, Charles University, Prague, Czech Republic.
FAU - Kardaoui, Hicham
AU  - Kardaoui H
AUID- ORCID: 0009-0008-8403-0982
AD  - National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, 
      Paris, France.
AD  - Universite Paris Cite, Paris, France.
FAU - Lamprecht, Peter
AU  - Lamprecht P
AUID- ORCID: 0000-0001-5929-868X
AD  - Department of Rheumatology and Clinical Immunology, Universitat zu Lubeck, 
      Lubeck, Germany.
FAU - Licholai, Sabina
AU  - Licholai S
AUID- ORCID: 0000-0001-7008-2492
AD  - Division of Molecular Biology and Clinical Genetics, Jagiellonian University 
      Medical College, Krakow, Poland.
FAU - Musial, Jacek
AU  - Musial J
AUID- ORCID: 0000-0002-0955-1808
AD  - 2nd Department of Internal Medicine, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - O'Sullivan, Declan
AU  - O'Sullivan D
AUID- ORCID: 0000-0003-1090-3548
AD  - ADAPT SFI Centre, School of Computer Science and Statistics, Trinity College 
      Dublin, Dublin, Ireland.
FAU - Puechal, Xavier
AU  - Puechal X
AUID- ORCID: 0000-0003-3573-9203
AD  - National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, 
      Paris, France.
AD  - French Vasculitis Study Group, Paris, France.
FAU - Scott, Jennifer
AU  - Scott J
AUID- ORCID: 0000-0001-8837-5250
AD  - ADAPT SFI Centre, School of Computer Science and Statistics, Trinity College 
      Dublin, Dublin, Ireland.
AD  - Trinity Kidney Centre, Trinity Translational Medicine Institute, Trinity College 
      Dublin, Dublin, Ireland.
FAU - Segelmark, Marten
AU  - Segelmark M
AUID- ORCID: 0000-0002-1221-0772
AD  - Department of Clinical Sciences, Lund University, Lund, Sweden.
AD  - Department of Endocrinology, Nephrology and Rheumatology, Skane University 
      Hospital, Lund, Sweden.
FAU - Straka, Richard
AU  - Straka R
AUID- ORCID: 0009-0007-2572-3941
AD  - General University Hospital in Prague, Praha, Czech Republic.
FAU - Terrier, Benjamin
AU  - Terrier B
AUID- ORCID: 0000-0001-6612-7336
AD  - National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, 
      Paris, France.
AD  - French Vasculitis Study Group, Paris, France.
FAU - Tesar, Vladimir
AU  - Tesar V
AUID- ORCID: 0000-0001-6982-0689
AD  - Department of Nephrology, General University Hospital, Prague, Czech Republic.
AD  - First Faculty of Medicine, Charles University, Prague, Czech Republic.
FAU - Tesi, Michelangelo
AU  - Tesi M
AUID- ORCID: 0000-0001-5500-9991
AD  - Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Firenze, Italy.
FAU - Vaglio, Augusto
AU  - Vaglio A
AUID- ORCID: 0000-0002-3814-9172
AD  - Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Firenze, Italy.
FAU - Wandrei, Dagmar
AU  - Wandrei D
AUID- ORCID: 0000-0002-8144-162X
AD  - Clinical Trials Unit, Medical Center, University of Freiburg Faculty of Medicine, 
      Freiburg, Germany.
FAU - White, Arthur
AU  - White A
AUID- ORCID: 0000-0002-7268-5163
AD  - School of Computer Science and Statistics, Trinity College Dublin, Dublin, 
      Ireland.
FAU - Wojcik, Krzysztof
AU  - Wojcik K
AUID- ORCID: 0000-0001-9786-2499
AD  - 2nd Department of Internal Medicine, Jagiellonian University Medical College, 
      Krakow, Poland.
FAU - Yaman, Beyza
AU  - Yaman B
AUID- ORCID: 0000-0003-2130-0312
AD  - ADAPT SFI Centre, School of Computer Science and Statistics, Trinity College 
      Dublin, Dublin, Ireland.
FAU - Little, Mark A
AU  - Little MA
AUID- ORCID: 0000-0001-6003-397X
AD  - ADAPT SFI Centre, School of Computer Science and Statistics, Trinity College 
      Dublin, Dublin, Ireland.
AD  - Trinity Kidney Centre, Trinity Translational Medicine Institute, Trinity College 
      Dublin, Dublin, Ireland.
FAU - Mohammad, Aladdin J
AU  - Mohammad AJ
AUID- ORCID: 0000-0002-7169-6936
AD  - Clinical Sciences, Rheumatology, Lund University, Lund, Sweden.
AD  - Department of Medicine, University of Cambridge, Cambridge, UK.
CN  - FAIRVASC consortium
LA  - eng
GR  - WT_/Wellcome Trust/United Kingdom
PT  - Journal Article
PT  - Meta-Analysis
DEP - 20240102
PL  - England
TA  - Ann Rheum Dis
JT  - Annals of the rheumatic diseases
JID - 0372355
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
SB  - IM
MH  - Humans
MH  - *Granulomatosis with Polyangiitis/drug therapy/epidemiology/complications
MH  - Data Accuracy
MH  - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug 
      therapy/epidemiology/complications
MH  - *Microscopic Polyangiitis/drug therapy/epidemiology
MH  - Antibodies, Antineutrophil Cytoplasmic
MH  - Registries
MH  - Information Storage and Retrieval
PMC - PMC10804071
OTO - NOTNLM
OT  - epidemiology
OT  - granulomatosis with polyangiitis
OT  - quality indicators, health care
OT  - systemic vasculitis
COIS- Competing interests: None declared.
FIR - Tassoni, Adrian
IR  - Tassoni A
FIR - Tais, Arlette
IR  - Tais A
FIR - Walsh, Cathal
IR  - Walsh C
FIR - Armstrong, Cecil
IR  - Armstrong C
FIR - Kalra, Dipak
IR  - Kalra D
FIR - Gelain, Elena
IR  - Gelain E
FIR - Schubach, Fabian
IR  - Schubach F
FIR - Ihorst, Gabriele
IR  - Ihorst G
FIR - Aerts, Hannelore
IR  - Aerts H
FIR - Sengers, Iris
IR  - Sengers I
FIR - Mills, John
IR  - Mills J
FIR - Power, Julie
IR  - Power J
FIR - Wawrzycka-Adamczyk, Katarzyna
IR  - Wawrzycka-Adamczyk K
FIR - McGlinn, Kris
IR  - McGlinn K
FIR - Dunne, Margaret
IR  - Dunne M
FIR - Christofidou, Maria
IR  - Christofidou M
FIR - Lea, Nathan
IR  - Lea N
FIR - Verhoeven, Peter
IR  - Verhoeven P
FIR - Boer, Raissa de
IR  - Boer R
FIR - Arnold, Sabrina
IR  - Arnold S
EDAT- 2023/11/01 00:42
MHDA- 2024/01/04 11:44
PMCR- 2024/01/23
CRDT- 2023/10/31 20:53
PHST- 2023/06/13 00:00 [received]
PHST- 2023/09/16 00:00 [accepted]
PHST- 2024/01/04 11:44 [medline]
PHST- 2023/11/01 00:42 [pubmed]
PHST- 2023/10/31 20:53 [entrez]
PHST- 2024/01/23 00:00 [pmc-release]
AID - ard-2023-224571 [pii]
AID - 10.1136/ard-2023-224571 [doi]
PST - epublish
SO  - Ann Rheum Dis. 2024 Jan 2;83(1):112-120. doi: 10.1136/ard-2023-224571.