Идёт набор NCT04265144

Cohort of Patients With Systemic Sclerosis Within the Framework of the RESO Reference Centre

Без фазы С лечением Scleroderma Systemic Sclerosis

Ориентир для пациента и семьи

Простыми словами

Автоматическая сводка по структурированным данным реестра. Она помогает сориентироваться, но не заменяет официальный протокол или оценку врача.

Что изучают: В протоколе указаны: Blood samples, Biopsy, Bronchoalveolar samples, Biopsy.
Кому может быть актуально: Состояния в реестре: Scleroderma, Systemic Sclerosis. Базовые параметры: от 18 лет · Все.
Что важно проверить: Возраст, диагноз и пол — только базовые ориентиры. Предыдущее лечение, анализы и другие обязательные условия указаны ниже в критериях участия.
Где проводится: Франция
Следующий шаг: Сохраните исследование, покажите его лечащему врачу и уточните актуальный статус у исследовательского центра. Расходы, документы и поездка →

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Официальное название

Cohort of Patients With Systemic Sclerosis and Associated Biological Collection Within the Framework of the RESO Reference Centre for Rare Systemic Autoimmune Diseases

Обзор

Systemic sclerosis (SSc) is a rare form of connective tissue disease characterized by vascular involvement and the intensity of fibrosis. The lack of available treatment is largely due to the very fragmented understanding of the pathophysiology of SSc. However, one of the keys to conducting quality research on this disease remains the development of well-documented patient cohorts with reliable biological samples. The main objective of this cohort is to study the natural progression of SSc in a cohort of patients followed over 5 years.

Подробное описание

Systemic sclerosis (SSc) is a rare form of connective tissue disease characterized by vascular involvement and the intensity of fibrosis. Its prevalence and incidence are difficult to assess, however, in France, a population survey conducted in Seine-St-Denis calculated a prevalence of 161 cases per million inhabitants.

The pathophysiology of SSc, the exact etiology of which remains unknown, involves an interaction between genetic and environmental factors. Its evolution can impact the aesthetic, functional and even vital prognosis of the affected patient.Within the analysis of SSc pathophysiology, a " very early systemic sclerosis " form of disease has been defined according to the presence of Raynaud's phenomenon and auto-antibodies in blood sample (ACAN positivity (≥1/160) with anti-Scl70, anti-centromere or anti-ARNPolIII specificity).

At present, no treatment to control this disease is available. The lack of available treatment is largely due to the very fragmented understanding of the pathophysiology of SSc. However, one of the keys to research remains the development of well-documented patient cohorts with quality biological samples. The investigators had the opportunity to start a major work on this plan with the VISS study (Vasculopathy and Inflammation in Systemic Scleroderma study) in 2012 as part of a project promoted by the University Hospital of Bordeaux (NCT02562079). This project has paved the way for many local, national and international collaborations. It has made it possible to structure and federate various partners of the Bordeaux University Hospital around translational research on SSc.

The investigators wish to continue our research and collaborations by further strengthening our expertise in the collection of rare and valuable biological samples for this disease.

Вмешательства

Биопрепарат Blood samples
62 ml whole blood for Peripheral blood mononuclear cell (PBMC) and monocytes isolation
Другое Biopsy
Skin biopsies only for volunteers among patients
Другое Bronchoalveolar samples
50 ml of bronchoalveolar samples if pulmonary flare requires this type of exploration only for volunteers among patients
Другое Biopsy
Digestives biopsies if requires this type of exploration in the standard of care only for volunteers among patients

Первичные конечные точки

Change of the main clinical characteristics of scleroderma patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]

Вторичные конечные точки (7)

Proportion of pulmonary arterial hypertension diagnosis in SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Proportion of interstitial lung disease diagnosis in SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Proportion of renal crisis diagnosis in SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Mean of Rodnan score for the evaluation of disease activity for SSc patients, with higher values mean higher disease activity. [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Mean of Diffusing capacity (DLCO) for the evaluation of disease activity for SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Mean of Forced vital capacity (FVC) for the evaluation of disease activity for SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]
Proportion of therapeutic strategies set up for SSc patients [Срок оценки: At baseline (Day 0) and 60 months after baseline]

Критерии участия

Критерии включения

Patient over 18 years old
Patient with systemic scleroderma according to the ACR/EULAR 2013 criteria, or with a " very early systemic sclerosis " defined by the presence of Raynaud's phenomenon and auto-antibodies in blood sample (ACAN positivity (≥1/160) with anti-Scl70, anti-centromere or anti-ARNPolIII specificity).
Person affiliated or benefiting from a social security scheme.
Free, informed and written consent signed by the participant and the investigator (no later than the day of inclusion and prior to any review required by the research)