NCT07168746 - National Cohort of Patients With SITRAME Syndrome | Crick | Crick

National Cohort of Patients With SITRAME Syndrome

N/ANot Yet RecruitingNCT07168746

Assistance Publique - Hôpitaux de Paris100 enrolled

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.

Study Type

OBSERVATIONAL

Enrollment

100

Conditions

Auto Inflammatory Diseases

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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

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

Description of the age 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 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: Day 0- Baseline

Secondary Outcomes

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

Measure of the incidence of this disease annually using the register of newly included patients Time frame: annually

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)

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

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)

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

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)

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

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)

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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: 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

Time frame: every 5 years up to 15 years