Study of One Protein Implicated in Wegener Disease (DAP12WEGENER)

April 20, 2015 updated by: Assistance Publique - Hôpitaux de Paris

Activating Receptors and DAP12 Protein in Wegener's Granulomatosis

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).

Study Overview

Status

Completed

Conditions

Intervention / Treatment

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 Type

Interventional

Enrollment (Actual)

138

Phase

  • Not Applicable

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • France
      • Paris, France, 75010
        • Medecine Interne Hôpital Saint Louis

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Allocation: Non-Randomized
  • Interventional Model: Single Group Assignment
  • Masking: None (Open Label)

Arms and Interventions

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

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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
less than 24 hours

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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
Less than 24 hours

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Assistance Publique - Hôpitaux de Paris

Investigators

  • Principal Investigator: Mathilde De Menthon,, MD, PhD, Assistance Publique - Hôpitaux de Paris

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

May 1, 2010

Primary Completion (Actual)

November 1, 2013

Study Completion (Actual)

September 1, 2014

Study Registration Dates

First Submitted

July 12, 2010

First Submitted That Met QC Criteria

July 21, 2010

First Posted (Estimate)

July 22, 2010

Study Record Updates

Last Update Posted (Estimate)

April 21, 2015

Last Update Submitted That Met QC Criteria

April 20, 2015

Last Verified

April 1, 2015

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • P081238

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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