An observational study in Hereditary Autoinflammatory Diseases, Schnitzler Syndrome and Behcet Syndrome, sponsored by University of Siena. Recruiting at 112 sites in 22 countries. Per ClinicalTrials.gov, last updated 2025-07-10.
Sponsored by University of Siena · Observational
Autoinflammatory diseases (AID) are clinical entities characterized by recurrent inflammatory attacks in absence of infection, neoplasm or deregulation of the adaptive immune system. Among them, hereditary periodic syndromes, also known as monogenic AID, represent the prototype of this disease group, caused by mutations in genes involved in the regulation of innate immunity, inflammation and cell death. Based on recent experimental acquisitions in the field of monogenic AID, several immunologic disorders have been reclassified as polygenic/multifactorial AID, sharing pathogenetic and clinical features with hereditary periodic fevers. This has paved the way to new treatment targets for patients suffering from rare diseases of unknown origin, including Behçet's disease, Still disease, Schnitzler's disease, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome, chronic recurrent multifocal osteomyelitis (CRMO), non-infectious uveitis and scleritis. Gathering information on such rare conditions is made difficult by the small number of patients, along with the difficulty of obtaining an accurate diagnosis in non-specialized clinical settings.
In this context, the AIDA project promotes international collaboration among clinical centres to develop a permanent registry aimed at collecting demographic, genetic, clinical and therapeutic data of patients affected by monogenic and polygenic AID, in order to expand the current knowledge of these rare conditions.
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:
The registry will pursue the following aims within the first 36 months from the start of the enrollment:
Additional aims of the AIDA project, to be reached over the next 10 years, are the following:
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).
136 studies on the registry are indexed under Behcet Syndrome; 42 are open to participants now.
This study's planned enrollment of 3,500 is above the median of 90 across 63 observational studies indexed under Behcet Syndrome.
Browse Behcet Syndrome studies →University of Siena is the lead sponsor of 65 studies on the registry; 15 are open to participants now.
Compared within Behcet Syndrome, this study's primary condition as ClinicalTrials.gg classifies it; the registry's first condition term is Hereditary Autoinflammatory Diseases. The counts are of registry records indexed under each term, refreshed daily. How we classify →
Patients consecutively diagnosed and treated at the hospitals/ research centres affiliated to the AIDA Network (https://aidanetwork.org/en/)
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
- /assent not provided by the patient and/or his/her legal representative.
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Other: No intervention is foreseen by the protocol.
Patients will be observed for 10 years at least. Demographic, genetic, clinical, clinimetric, laboratory, radiologic and therapeutic data will be collected both retrospectively and prospectively during routine follow-up visits.
Change of the number of enrolled subjects
This is an observational registry. The primary outcome is the increase of the number of subjects enrolled within each nosologic group.
Time frame: 0-36-60-120 months
Change in the disease activity score
Change in the disease activity score specific for the nosologic entity (BDCAF score, Pouchot score, modified Pouchot score, JADAS27/JADAS27(CRP), DAS28) or according to composite indexes that will be defined in each study based on the registry.
Time frame: 0-36-60-120 months
Change in the % of patients with new organ involvement
Time frame: 0-36-60-120 months
Incidence of death or adverse events
Time frame: 0-36-60-120 months
Change in the inflammatory markers values (ESR)
ESR measured in mm/h
Time frame: 0-36-60-120 months
Change in the inflammatory markers values (CRP and SAA)
CRP and SAA measured in mg/dl
Time frame: 0-36-60-120
Change in visual acuity expressed as Best Corrected Visual Acuity (BCVA)
Time frame: 0-36-60-120 months
Change in the overall function score
Change in the overall function score according to HAQ (adults) or CHAQ (children)
Time frame: 0-36-60-120 months
Change in the overall damage score
Change in the overall damage score specific for the nosologic entity (ADDI score, BODI score, JADI score)
Time frame: 0-36-60-120 months
Change in articular pain measured as Visual Analogue Scale for articular Pain (VAS Pain)
Time frame: 0-36-60-120 months
Changes in height and weight percentile values for age and sex
Time frame: 0-36-60-120 months
Change in the % of patients experiencing fatigue
Time frame: 0-36-60-120 months
Change in the % of patients with fertility reduction or pregnancy complications
Time frame: 0-36-60-120
Change in the socioeconomic indicators
Number of days lost at work/school during the last 3 months, Number of visits/ phone calls to the general practitioner during the last 3 months, Number of accesses to the Emergency Room during the last 6 months, Days of hospitalization during the last 3 months, Overall number of days lost at work by relatives during the last 3 months
Time frame: 0-36-60-120 months
Showing the first 100 of 112 sites across 22 countries. Choose a country and city above to see the rest.
Plan to share: No
Started Aug 2020 (actual). Primary completion is estimated for Aug 2026, 72 months after the start.
Of 21 observational studies under Behcet Syndrome, half reached primary completion within 9 months of starting (the median), counting those still running; a quarter within 5 and three quarters within 81. This study's planned 72 months is above that.
How these figures are worked out, and which studies are left out of them.
Eligibility is decided by the study team. Share this record with your doctor or contact the team directly.
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