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Study of One Protein Implicated in Wegener Disease (DAP12WEGENER)

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ClinicalTrials.gov Identifier: NCT01167491
Recruitment Status : Completed
First Posted : July 22, 2010
Last Update Posted : April 21, 2015
Sponsor:
Information provided by (Responsible Party):

Study Description
Brief Summary:
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).

Condition or disease Intervention/treatment
Wegener's Granulomatosis Biological: Physiopathology Endpoint Classification

Detailed Description:
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.

Study Design

Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 138 participants
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Official Title: Activating Receptors and DAP12 Protein in Wegener's Granulomatosis
Study Start Date : May 2010
Primary Completion Date : November 2013
Study Completion Date : September 2014


Arms and Interventions

Arm Intervention/treatment
Disease group
Physiopathology
Biological: Physiopathology Endpoint Classification
Blood samples will be collected during a routine medical visit.


Outcome Measures

Primary Outcome Measures :
  1. level of mRNA expression of DAP12 [ Time Frame: less than 24 hours ]
    Measure:level of mRNA expression of DAP12 by RT-PCR in CD4+T cells, macrophages and neutrophils


Secondary Outcome Measures :
  1. level of expression of DAP 12 downstream signalling proteins [ Time Frame: Less than 24 hours ]
    Measure: level of expression of DAP 12 downstream signalling proteins in CD4+ T cells, macrophages and neutrophils


Eligibility Criteria

Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years and older   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Criteria

Inclusion Criteria:

  • Group 1: WG, with granulomatous lesions limited to upper airway or lungs and no evidence of generalized vasculitis ,± biopsy, ± anti-PR3 ANCA
  • Group 2: WG with granulomatous lesions plus vasculitis expression (renal, neurological, skin, gut or heart involvement), ± biopsy, ± anti-PR3 ANCA
  • Group 3: Necrotizing vasculitis with no granulomatous lesions, ± PAUCI immune glomerulonephritis, ± anti-MPO ANCA
  • Group 4: clinical presentation compatible with sarcoidosis, ± biopsy, ± ECA elevated ± tuberculin anergy

Exclusion Criteria:

  • <18 years
  • Pregnancy or breastfeeding
  • Absence of signed informed consent
  • No affiliation to insurance
Contacts and Locations

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01167491


Locations
France
Medecine Interne Hôpital Saint Louis
Paris, France, 75010
Sponsors and Collaborators
Assistance Publique - Hôpitaux de Paris
Investigators
Principal Investigator: Mathilde De Menthon,, MD, PhD Assistance Publique - Hôpitaux de Paris
More Information

Responsible Party: Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov Identifier: NCT01167491     History of Changes
Other Study ID Numbers: P081238
First Posted: July 22, 2010    Key Record Dates
Last Update Posted: April 21, 2015
Last Verified: April 2015

Keywords provided by Assistance Publique - Hôpitaux de Paris:
Wegener
CD4+ T cells
DAP12
NKG2D

Additional relevant MeSH terms:
Granulomatosis with Polyangiitis
Lung Diseases, Interstitial
Lung Diseases
Respiratory Tract Diseases
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Systemic Vasculitis
Vasculitis
Vascular Diseases
Cardiovascular Diseases
Autoimmune Diseases
Immune System Diseases