National Cohort of Patients With SITRAME Syndrome
Trial Snapshot
- Phase
- Not Applicable
- Status
- Not yet recruiting
- Enrollment
- 100
- Primary Endpoint
- Description of the sexe at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Study Overview
Brief Summary
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.
Study Design
- Study Type
- Observational
- Observational Model
- Cohort
- Time Perspective
- Prospective
Eligibility Criteria
- Ages
- 18 Years to — (Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- No
Inclusion Criteria
- •SITRAME syndrome meeting the diagnostic criteria for the disease
- •All the following criteria must be met:
- •Systemic inflammation: at least 1 documented episode of CRP >5mg/mL during a skin flare
- •Maculopapular rash on the trunk: fixed, non-itchy maculopapular rash on the trunk appearing within hours with clear borders
- •Recurrence in the same areas: at least 3 different episodes
- •Acute: duration less than 8 days
- •And at least 1 of the following criteria:
- •Fever during flare-ups
- •Flare-ups triggered by infections, vaccinations, or intense physical exercise
- •Fatigue during and/or after flare-ups
- •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
Outcomes
Primary Outcomes
Description of the sexe at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Time Frame: Day 0- Baseline
Description of the age at the diagnosis of the disease
Time Frame: Day 0- Baseline
Description of the age at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Description of cardiovascular co-morbidity
Time Frame: Day 0- Baseline
Description of cardiovascular co-morbidity at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Description of presence of an allergical context
Time Frame: Day 0- Baseline
Description of presence of an allergical context in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Description of the different therapeutic lines
Time Frame: Year 15
Description of the different therapeutic lines in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Description of the age at death
Time Frame: Day 0- Baseline
Description of the age at death in order to describe the clinical characteristics of patients followed for SITRAME syndrome
Secondary Outcomes
- Description of the rare biological manifestations (outside of the well-established disease criteria)(Year 15)
- Measure of the prevalence of this disease annually using the register of newly included patients(Every year up to 15 years)
- Measure of the incidence of this disease(Every year up to 15 years)
- Description of the rare clinical manifestations (outside of the well-established disease criteria)(Year 15)
- Measure of the number of patients eligible for various clinical research studies on SITRAME syndrome(Every year up to 15 years)
- Measure of the proportion of patients using targeted therapies throughout the study(Year 15)
- Measure of the number of deaths in patients followed for SITRAME syndrome(every 5 years up to 15 years)
- Description of the death causes in patients followed for SITRAME syndrome(every 5 years up to 15 years)
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (pulmonary manifestations)(every 5 years up to 15 years)
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (cardiac manifestations)(Every 5 years up to 15 years)
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (neurological manifestations)(every 5 years up to 15 years)
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (gastrointestinal manifestations)(every 5 years up to 15 years)
- C-reactive protein (CRP) rate in the population of dead patients followed for SITRAME syndrome(every 5 years up to 15 years)
- Frequency of inflammatory flares throughout the disease course in the population of dead patients followed for SITRAME syndrome(every 5 years up to 15 years)
- Ferritin rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Liver enzymes rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Creatinine rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Red blood cells rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Platelets rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Hemoglobin rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Hematocrit rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Mean corpuscular volume (MCV) rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- White blood cells rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Neutrophils rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Lymphocytes rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Monocytes rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Eosinophils rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)
- Basophils rate of the dead patient followed for SITRAME syndrome(every 5 years up to 15 years)