National Cohort of Patients With SITRAME Syndrome (CO-SITRAME)

Assistance Publique - Hôpitaux de Paris

Status: Not yet enrolling

Conditions: Auto Inflammatory Diseases

Study type: Observational

Funder types: Other

Identifiers

NCT07168746

APHP250008

Details and patient eligibility

About

SITRAME syndrome is a recently described disease. In the absence of epidemiological data on SITRAME syndrome in France, the National Reference Center for Autoinflammatory Diseases proposes coordinating a database to collect both retrospective and prospective cases of this rare disease.

Currently, there are no available data on the epidemiology or treatment of SITRAME syndrome in France or globally. The importance of this research is to provide an overview of demographic, etiological, and disease progression data for patients with SITRAME syndrome in France. This will allow the establishment of quantitative data on the morbidity and mortality of this rare disease.

Enrollment: 100 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• SITRAME syndrome meeting the diagnostic criteria for the disease

All the following criteria must be met:

1. Systemic inflammation: at least 1 documented episode of CRP >5mg/mL during a skin flare
2. Maculopapular rash on the trunk: fixed, non-itchy maculopapular rash on the trunk appearing within hours with clear borders
3. Recurrence in the same areas: at least 3 different episodes
4. Acute: duration less than 8 days

And at least 1 of the following criteria:

1. Fever during flare-ups
2. Flare-ups triggered by infections, vaccinations, or intense physical exercise
3. Fatigue during and/or after flare-ups
4. Associated papular rash

And all the following exclusion criteria must be met:

• No evidence of a monogenic autoinflammatory disease, evolving neoplasm, or evolving autoimmune disease.
• Adult patients at the time of inclusion
• Patients not opposed to participating in the research

Exclusion criteria

- Persons with special protection cannot be included in the study:

• Adults under guardianship or curatorship
• Hospitalized persons without consent and not legally protected
• Persons deprived of liberty