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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.
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| Measure | Description | Time Frame |
|---|---|---|
| Description of the sexe at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Day 0- Baseline | |
| Description of the age at the diagnosis of the disease | Description of the age at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Day 0- Baseline |
| Description of cardiovascular co-morbidity | Description of cardiovascular co-morbidity at the diagnosis of the disease in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Day 0- Baseline |
| Description of presence of an allergical context | Description of presence of an allergical context in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Day 0- Baseline |
| Description of the different therapeutic lines | Description of the different therapeutic lines in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Year 15 |
| Description of the age at death | Description of the age at death in order to describe the clinical characteristics of patients followed for SITRAME syndrome | Day 0- Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| 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 | Measure of the incidence of this disease annually using the register of newly included patients Time frame: annually |
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Inclusion Criteria:
• SITRAME syndrome meeting the diagnostic criteria for the disease
All the following criteria must be met:
And at least 1 of the following criteria:
And all the following exclusion criteria must be met:
Exclusion Criteria:
- Persons with special protection cannot be included in the study:
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Patients will be recruited in France via the national reference center for autoinflammatory diseases at Tenon Hospital (Internal Medicine, Dermatology and Allergology Department at Tenon Hospital).
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Angele SORIA, PU-PH, MD, PhD | Contact | 00 33 1 56 01 72 20 | angele.soria@aphp.fr | |
| Sophie GEORGIN-LAVIALLE, PUPH, MD, PhD | Contact | 00 33 1 56 01 74 31 | sophie.georgin-lavialle@aphp.fr |
| Name | Affiliation | Role |
|---|---|---|
| Angele SORIA, PUPH, MD, PhD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
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| Every year up to 15 years |
| Description of the rare clinical manifestations (outside of the well-established disease criteria) | Year 15 |
| Description of the rare biological 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) | Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (pulmonary manifestations) in order to describe the clinical characteristics of this population | every 5 years up to 15 years |
| Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (cardiac manifestations) | Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (cardiac manifestations) in order to describe the clinical characteristics of this population | Every 5 years up to 15 years |
| Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (neurological manifestations) | Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (neurological manifestations) in order to describe the clinical characteristics of this population | every 5 years up to 15 years |
| Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (gastrointestinal manifestations) | Number of dead patients followed for SITRAME syndrome with presence and pattern of extracutaneous involvement (gastrointestinal manifestations) in order to describe the clinical characteristics of this population | every 5 years up to 15 years |
| C-reactive protein (CRP) rate in the population of dead patients followed for SITRAME syndrome | C-reactive protein (CRP) rate in the population of dead patients followed for SITRAME syndrome in order to describe the biological characteristics of this population | 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 | Frequency of inflammatory flares throughout the disease course in the population of dead patients followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Ferritin rate of the dead patient followed for SITRAME syndrome | Ferritin rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Liver enzymes rate of the dead patient followed for SITRAME syndrome | Liver enzymes rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Creatinine rate of the dead patient followed for SITRAME syndrome | Creatinine rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Red blood cells rate of the dead patient followed for SITRAME syndrome | Red blood cells rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Platelets rate of the dead patient followed for SITRAME syndrome | Platelets rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Hemoglobin rate of the dead patient followed for SITRAME syndrome | Hemoglobin rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Hematocrit rate of the dead patient followed for SITRAME syndrome | Hematocrit rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Mean corpuscular volume (MCV) rate of the dead patient followed for SITRAME syndrome | Mean corpuscular volume (MCV) rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| White blood cells rate of the dead patient followed for SITRAME syndrome | White blood cells rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Neutrophils rate of the dead patient followed for SITRAME syndrome | Neutrophils rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Lymphocytes rate of the dead patient followed for SITRAME syndrome | Lymphocytes rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Monocytes rate of the dead patient followed for SITRAME syndrome | Monocytes rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Eosinophils rate of the dead patient followed for SITRAME syndrome | Eosinophils rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |
| Basophils rate of the dead patient followed for SITRAME syndrome | Basophils rate of the dead patient followed for SITRAME syndrome in order to describe the biological characteristics of this population | every 5 years up to 15 years |