Data quality and patient characteristics in European ANCA-associated vasculitis registries: data retrieval by federated querying.
Data quality and patient characteristics in European ANCA-associated vasculitis registries: data retrieval by federated querying.
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DOI：
10.1136/ard-2023-224571
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发表时间：
2024-01-02
影响因子：
27.4
通讯作者：
中科院分区：
文献类型：
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作者：
本研究旨在描述数据结构和协调过程，探索数据质量并定义六个联合抗中性粒细胞胞浆抗体相关性血管炎 (AAV) 登记处患者的特征、治疗和结果。通过创建血管炎特定的可查找、可访问、可互操作、可重用的 VASCulitis 本体，我们协调了注册并实现了语义互操作性。我们评估了数据质量的唯一性、一致性、完整性和正确性。使用语义查询语言 SPARQL 协议和资源描述框架查询语言 (SPARQL) 检索聚合数据，并通过随机效应荟萃分析评估结果率。总共发现了 5282 例 AAV 病例。所有评估变量的唯一性和数据类型一致性均为 100%。完整性和正确性分别从 49%–100% 到 60%–100% 不等。肉芽肿性多血管炎 (GPA) 病例 2754 例 (52.1%)，显微镜下多血管炎 1580 例 (29.9%)，嗜酸性粒细胞性 GPA 937 例 (17.7%)。受累器官类型包括：肺3281例（65.1%）、耳鼻喉2860例（56.7%）、肾2534例（50.2%）。 982 例 (50.7%) 患者使用静脉注射环磷酰胺作为缓解诱导治疗，505 例 (17.7%) 患者使用利妥昔单抗，脉冲静脉注射糖皮质激素的使用差异很大 (11%–91%)。终末期肾病的总死亡率和发病率分别为每 1000 名患者年 28.8 例（95% CI 19.7 至 42.2）和 24.8 例（95% CI 19.7 至 31.1）。在报道的最大规模的 AAV 队列研究中，我们使用语义网络技术联合患者登记，并强调了对数据质量的担忧。不同的招募环境阻碍了患者特征、治疗和结果的比较。
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. 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. 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. 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.
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影响因子：
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作者：
MASI, AT;HUNDER, GG;ZVAIFLER, NJ
通讯作者：
ZVAIFLER, NJ
影响因子：
2.2
作者：
Iking-Konert C;Wallmeier P;Arnold S;Adler S;de Groot K;Hellmich B;Hoyer BF;Holl-Ulrich K;Ihorst G;Kaufmann M;Kötter I;Müller-Ladner U;Magnus T;Rech J;Schubach F;Schulze-Koops H;Venhoff N;Wiech T;Villiger P;Lamprecht P
通讯作者：
Lamprecht P
影响因子：
4.9
作者：
Rastegar-Mojarad M;Sohn S;Wang L;Shen F;Bleeker TC;Cliby WA;Liu H
通讯作者：
Liu H
影响因子：
--
作者：
Jennette, J. C.;Falk, R. J.;Watts, R. A.
通讯作者：
Watts, R. A.
影响因子：
2.3
作者：
Chanouzas, Dimitrios;McGregor, Julie Anne G.;Harper, Lorraine
通讯作者：
Harper, Lorraine