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NCT Number: NCT01167491

Study of One Protein Implicated in Wegener Disease

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).

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Key information

Conditions

Wegener's Granulomatosis Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Autoimmune Diseases Cardiovascular Diseases Granulomatosis with Polyangiitis Immune System Diseases Lung Diseases Lung Diseases, Interstitial Respiratory Tract Diseases Skin Diseases Skin Diseases, Vascular Skin and Connective Tissue Diseases Systemic Vasculitis Vascular Diseases Vasculitis

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Medecine Interne Hôpital Saint Louis

Paris, 75010, France

About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Physiopathology Endpoint Classification

Biological

Blood samples will be collected during a routine medical visit.

Primary outcomes

  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 outcomes

  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

Sponsors and collaborators

Lead sponsor

Assistance Publique - Hôpitaux de Paris

Other

Collaborators

  • URC-CIC Paris Descartes Necker Cochin

Registry information

Official study title

Activating Receptors and DAP12 Protein in Wegener's Granulomatosis

Acronym: DAP12WEGENER

Important dates

Study start
2010
Primary completion
2013
Study completion
2014
First posted
Jul 22, 2010
Registry last updated
Mar 31, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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