Clinical Sciences, Rheumatology, Lund University, Lund, Sweden
School of Infection and Immunity, University of Glasgow, Glasgow, UK.
Ann Rheum Dis. 2024 Jan 2;83(1):112-120. doi: 10.1136/ard-2023-224571.
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.
本研究旨在描述数据结构和协调过程，探索数据质量，并定义六个联邦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、治疗和结局。
通过创建专门针对血管炎的可查找、可访问、互操作、可重复使用的 VASCulitis 本体，我们协调了登记处并实现了语义互操作性。我们评估了各个域的独特性、一致性、完整性和正确性。使用语义查询语言 SPARQL Protocol and Resource Description Framework Query Language (SPARQL) 检索聚合数据，并通过随机效应荟萃分析评估结局发生率。
共确定了 5282 例 AAV 病例。所有评估变量的独特性和数据类型一致性均为 100%。完整性和正确性分别为 49%-100%至 60%-100%。2754 例（52.1%）被归类为肉芽肿性多血管炎（GPA），1580 例（29.9%）为显微镜下多血管炎，937 例（17.7%）为嗜酸性粒细胞性 GPA。器官受累的模式包括：3281 例（65.1%）肺部受累，2860 例（56.7%）耳部-鼻部-咽喉受累，2534 例（50.2%）肾脏受累。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，静脉注射糖皮质激素的使用情况差异很大（11%-91%）。总体死亡率和终末期肾病的发生率分别为每 1000 患者年 28.8（95%CI 19.7 至 42.2）和 24.8（95%CI 19.7 至 31.1）。
在报告的最大 AAV 队列研究中，我们使用语义网技术联合了患者登记处，并强调了对数据质量的关注。由于招募环境的异质性，患者特征、治疗和结局的比较受到阻碍。
