National Cohort of Patients With SITRAME Syndrome
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- This is an observational study: the protocol does not assign a study treatment.
- Who it may be relevant to
- Registry conditions: Auto Inflammatory Diseases. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Center list to be confirmed — check the primary protocol.
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Overview
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.
Primary outcome measures
- 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 cardiovascular co-morbidity [Time frame: Day 0- Baseline]
- Description of presence of an allergical context [Time frame: Day 0- Baseline]
- Description of the different therapeutic lines [Time frame: Year 15]
- Description of the age at death [Time frame: Day 0- Baseline]
Secondary outcome measures (12)
- Measure of the prevalence of this disease annually using the register of newly included patients [Time frame: Every year up to 15 years]
- Measure of the incidence of this disease [Time frame: Every year up to 15 years]
- Description of the rare clinical manifestations (outside of the well-established disease criteria) [Time frame: Year 15]
- Description of the rare biological manifestations (outside of the well-established disease criteria) [Time frame: Year 15]
- Measure of the number of patients eligible for various clinical research studies on SITRAME syndrome [Time frame: Every year up to 15 years]
- Measure of the proportion of patients using targeted therapies throughout the study [Time frame: Year 15]
- Measure of the number of deaths in patients followed for SITRAME syndrome [Time frame: every 5 years up to 15 years]
- Description of the death causes in patients followed for SITRAME syndrome [Time frame: every 5 years up to 15 years]
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (pulmonary manifestations) [Time frame: every 5 years up to 15 years]
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (cardiac manifestations) [Time frame: Every 5 years up to 15 years]
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (neurological manifestations) [Time frame: every 5 years up to 15 years]
- Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (gastrointestinal manifestations) [Time frame: every 5 years up to 15 years]
Eligibility criteria
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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
Center list to be confirmed — check the primary protocol.
Identifiers
NCT: NCT07168746 · APHP250008