AutoInflammatory Disease Alliance Registry (AIDA)

The AIDA registry service is based on REDCap (Research Electronic Data Capture, project-redcap.org), a secure web application for building and managing online surveys and databases, designed to support data capture for research studies. The platform is directly accessible through the AIDA website, after inserting a personal username and password. Currently, 11 registries are available, each one dedicated to the collection of data about:

• monogenic AID

• PFAPA syndrome

• undifferentiated systemic AID (USAID)

• Behçet's disease

• Schnitzler's disease

• VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome

• Still disease

• noninfectious uveitis

• noninfectious scleritis

• spondyloarthritis

• Castleman disease

The registry will pursue the following aims within the first 36 months from the start of the enrollment:

1. to identify any association between clinical manifestations and gender, disease duration, body mass index and tabagism;

2. to detect differences in the clinical phenotype between pediatric-onset and adult-onset patients;

3. to identify the impact of different treatment approaches on clinical and laboratory disease manifestations.

Additional aims of the AIDA project, to be reached over the next 10 years, are the following:

1. to overcome fragmentation in the clinical experience on these rare conditions by sharing the knowledge at the international level;

2. to improve knowledge about the clinical presentation, genotype-phenotype correlations, response to treatment, long-term complications and social impact when monogenic and polygenic AID manifest during either childhood or adulthood;

3. to identify the long-term clinical course of patients diagnosed with monogenic or polygenic AID;

4. to promote awareness among physicians and enhance early recognition of these diseases;

5. to describe the impact of AID on quality of life;

6. to identify any impact of monogenic and polygenic AID on fertility;

7. to study the course of AID during pregnancy;

8. to assess the socioeconomic impact of AID;

9. to promote future multicentric studies.

Data collection is both retrospective and prospective. It includes demographic, clinical, diagnostic, genetic, clinimetric, laboratory, radiologic, therapeutic and socio-economic data. Instruments are designed to be filled out during routine clinical visits, usually scheduled every 3-6 months. Separation of personally identifiable information and medical data by using only pseudonyms for storing medical data ensures compliance with data protection regulations. The description of symptoms, diseases, procedures and injuries is based on the International Statistical Classification of Diseases and Related Health Problems (ICD)-10 coding system. Data management is both central and decentral. Data are extracted and statistically analyzed on a regular basis according to individual study protocols. A policy for authorship and dissemination of research findings is in place among the AIDA partners contributing to the registry.

The AIDA registry will support data collection for the conduction of clinical trials, observational studies, comparative effectiveness research, and other research on patients with monogenic and polygenic AID.

Several healthcare providers contributing to the AIDA Network are members of the European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA).