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| Name | Class |
|---|---|
| URC-CIC Paris Descartes Necker Cochin | OTHER |
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The investigators recently showed an abnormal expansion of NK-like CD4+ T cells in Wegener's granulomatosis (WG), mainly in the diffuse vasculitis presentation. These cells expressed an assortment of activating NK cell receptors and their signaling partners, in particular DAP12. The investigators hypothesize that DAP12, or a downstream signaling target of DAP12, is the missing link between the different cell components involved in WG (neutrophils, macrophages, CD4 T cells).
The investigators will test our hypothesis of "DAP-12 gain-of-function" by quantitative (mRNA and protein) and qualitative (DNA, signaling and cellular activation) analysis of the DAP12 signaling pathway in WG patients with localized (group 1; n=30) or diffuse WG (group 2; n=30) by comparison with patients with micro polyangitis (group 3; n=30), or with sarcoidosis ( group 4; n=30), and healthy blood donors (group 5; n=30). Blood samples will be collected during a routine medical visit. These results may help to design future therapeutic strategies based on modulation of specific intra-cellular pathways involved in the disease.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Disease group | Other | Physiopathology |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Physiopathology Endpoint Classification | Biological | Blood samples will be collected during a routine medical visit. |
|
| Measure | Description | Time Frame |
|---|---|---|
| level of mRNA expression of DAP12 | Measure:level of mRNA expression of DAP12 by RT-PCR in CD4+T cells, macrophages and neutrophils | less than 24 hours |
| Measure | Description | Time Frame |
|---|---|---|
| level of expression of DAP 12 downstream signalling proteins | Measure: level of expression of DAP 12 downstream signalling proteins in CD4+ T cells, macrophages and neutrophils | Less than 24 hours |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Mathilde De Menthon,, MD, PhD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Medecine Interne Hôpital Saint Louis | Paris | 75010 | France |
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| ID | Term |
|---|---|
| D014890 | Granulomatosis with Polyangiitis |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D056648 | Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis |
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| D056647 | Systemic Vasculitis |
| D014657 | Vasculitis |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D017445 | Skin Diseases, Vascular |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |