Sota Jurgen, Vitale Antonio, Więsik-Szewczyk Ewa, Frassi Micol, Lopalco Giuseppe, Emmi Giacomo, Govoni Marcello, de Paulis Amato, Marino Achille, Gidaro Antonio, Monti Sara, Opris-Belinski Daniela, Pereira Rosa Maria R, Jahnz-Rózyk Karina, Gaggiano Carla, Crisafulli Francesca, Iannone Florenzo, Mattioli Irene, Ruffilli Francesca, Mormile Ilaria, Rybak Katarzyna, Caggiano Valeria, Airò Paolo, Tufan Abdurrahman, Gentileschi Stefano, Ragab Gaafar, Almaghlouth Ibrahim A, Aboul-Fotouh Khalil Adham, Cattalini Marco, La Torre Francesco, Tarsia Maria, Giardini Henrique A Mayrink, Ali Saad Moustafa, Bocchia Monica, Caroni Federico, Giani Teresa, Cinotti Elisa, Ruscitti Piero, Rubegni Pietro, Dagostin Marília A, Frediani Bruno, Guler Aslihan Avanoglu, Della Casa Francesca, Maggio Maria Cristina, Recke Andreas, von Bubnoff Dagmar, Krause Karoline, Balistreri Alberto, Fabiani Claudia, Rigante Donato, Cantarini Luca
Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
Department of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, Warsaw, Poland.
Front Med (Lausanne). 2022 Jul 18;9:931189. doi: 10.3389/fmed.2022.931189. eCollection 2022.
The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.
This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries.
Since its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access.
This International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder.
本文描述了专门针对施尼茨勒综合征患者的自身炎症性疾病联盟（AIDA）国际注册库的设计、开发和实施情况。
这是一个由临床医生推动、基于人群和电子的注册库，用于回顾性和前瞻性收集施尼茨勒综合征患者的真实生活数据；该注册库基于研究电子数据采集（REDCap）工具，该工具旨在收集临床研究的标准化信息，并已实现根据未来的科学成果随时间变化，并可能与其他现有或未来类似的注册库进行通信。
自启动以来，来自四大洲23个国家的113个中心参与其中。其中57个已获得当地伦理委员会的批准。截至2022年4月28日，该平台有324名用户（114名主要研究者、205名现场研究者、2名首席研究者和3名数据管理人员）。该注册库使用3924个字段（分为25份文件）收集基线和随访数据，包括患者的人口统计学信息、病史、临床表现和症状、触发/风险因素、实验室检查、仪器检查、治疗方法、社会经济信息以及医疗保健获取情况。
这个针对施尼茨勒综合征患者的国际注册库有助于标准化数据收集，通过数据共享和知识传播实现国际合作项目；反过来，它将揭示这种复杂的自身炎症性疾病的许多盲点。
